INSTRUCTIONS TO AUTHORS
A. General Information
The Greek Paediatric Society is the owner of “Paediatriki”, its official scientific journal, which is distributed to its members. Its objectives are the publication of paediatric scientific work and the continuing education of paediatricians. For this purpose, it publishes a variety of articles, and in particular:
1.Editorials (upon invitation by the Editorial Board).
2.Review articles.
3.Award-winning articles.
4.Original articles.
5.Clinical Quiz.
6.Round tables.
7.Current issues.
8.Issues of healthcare management and education.
9.Case reports.
10.News.
11.Brief reports.
12.Letters to the editor.
13.Abstracts.
14.Future congresses and events.
15.Book reviews.
The Editorial Board reserves the right to publish articles of special scientific interest and articles on current issues without observing submission order. In addition it publishes upon decision original papers presented at the Annual Paediatric Conference, presentations of special interest - in whole or in part, and letters - in whole or in part - referring to scientific articles published in the journal. Regarding papers on current issues, the author’s request for immediate publication should be quoted on the first page. The Editorial Board reserves the right to accept such papers for immediate publication.
All manuscripts should not have been published previously, in whole or in part, and not be under consideration by another publication. Manuscripts should acknowledge any funding, sponsorship or other financial support. All clinical research should have been conducted following informed consent of participants or of their legal representatives according to the Declarations of Helsinki and Tokyo. In addition, the US National Institute of Health guide for the care and use of laboratory animals (DHEW Publication, NIH, 80-23) should have been observed. Clinical trials should have been approved by the Ethics Committee of the Hospital.
Authors’ opinions and conclusions expressed in the published papers do not necessarily reflect those of the journal. The Greek Paediatric Society, the Editorial Board and the Publisher of the journal do not necessarily approve the content of the advertisements appearing in the journal.
The copyright of all published papers is held by “Paediatriki” and their reproduction in whole or in part is authorized only following written consent of the journal.
B. Manuscript Preparation
“Paediatriki” suggests compliance with the “Uniform Requirements for Manuscripts Submitted to Biomedical Journals”, recently modified and published on the websites: http://www.icmje.org and http://www.icmje.org/icmje.pdf
The entire paper (including figure legends and tables)
should be typed on one side of blank paper format A4 (21x29.7 cm), double line spacing and minimum indent 2.5 cm on both sides.
The paper should have the following structure: title page, short title, abstract in Greek and English, list of abbreviations, text, acknowledgements and quoting of grants, sponsorships or other financial support sources, references, tables, figures, figure legends. Each of these sections should be started on a new page. Pages should be numbered consecutively, beginning with the title page.
Text length shall be:
ñ review articles 2000-3000 words;
ñ original articles 1500-2500 words and case reports 1000-1500 words;
ñ brief reports 1000-1500 words; ñletters 250-500 words.
The title page should include:
ñ the title (<14 words) and the short title (<5 words) of the article. No abbreviations are permitted in the title;
ñ the name and surname of all authors;
ñ the centre (institution, clinic, laboratory) of origin of the paper. If there is no affiliation with a specific centre, the status of the author(s) should be cited (e.g., private paediatrician) and home address;
ñ the complete address, e-mail and telephone number of the author to whom correspondence should be addressed.
Abstracts
The abstract should summarize the objectives, methodology, main results and conclusions of the study.
ñ It should contain at least 200 words, and not exceed 250 words.
ñ It should consist of the following paragraphs: background, methods, results and conclusions.
The English abstract should cite at the beginning the title of the paper and the authors’ names in English. The content of the text should consist of the following paragraphs: background, methods, results and conclusions. The abstract in English should not differ in content from the corresponding Greek abstract.
Beneath the Greek and English abstracts, three to five key words in the respective language should be supplied, to be used in the thematic index.
Text
Original articles include: introduction, methods, results and discussion. The introduction includes the latest research data on the subject and the main references and the objectives of the paper. The description of the methods should be precise and detailed so as to enable reproduction by other researchers. In addition, the statistical methods of analysis and evaluation of the results should be described. Results should be presented clearly, together with the appropriate statistical analysis. Discussion should cover the results ensuing from the research, their significance and possible associations with the observations of other researchers.
Case reports comprise a short introduction, case description and brief discussion, with emphasis on differential diagnosis.
The structure of all other articles is free, according to the judgment of the authors.
Thanks or acknowledgements (reference to grants, sponsorships or other sources of financial support) should be quoted at the end of the text, before references.
Units of measures of laboratory analyses
Laboratory analyses should be expressed in the Système International (SI) units and in the metric (Conventional) system in parentheses. See conversion tables on the websites: http://www.icmje.org and http://www.icmje.org/icmje.pdf
Abbreviations
All issues of the journal contain internationally established abbreviations. Complex or long terms often repeated in the text may be replaced by abbreviations explained by the authors in a list submitted with the paper. Abbreviations are reported in parentheses only in abstracts.
References
The reference section contains all references numbered in the order in which they appear in the text. In the text, references are to be indicated by Arabic numerals in parentheses. References should be no more than:
ñ 70 in review articles;
ñ 30 in original articles;
ñ 12 in current issues and case reports;
ñ5 in brief reports and letters.
In listing references follow the recently modified standards of the International Committee of Medical Journal Editors/Uniform Requirements for Manuscripts Submitted to Biomedical Journals, (http://www.icmje.org and http:// www.icmje.org/icmje.pdf). Abbreviated names of journals should conform to the Cumulated Index Medicus [List of Journals Indexed in Index Medicus (http://www.nlm.nih.gov/bsd/uniform requirements.html)].
Examples of reference style
I. JOURNALS
All authors are cited if they are six or less; if they are 7 or more, the first six are cited, followed by “et al”.
Regular edition:
Proesmans W. Bartter syndrome and its neonatal variant. Eur J Pediatr 1997;156:669-679.
Supplement issue:
Flyvbjerg A. Role of growth hormone, insulin-like growth factors (IGFs) and IGF-binding proteins in the renal complications of diabetes. Kidney Int 1997;52 (60 Suppl):S12-S19.
No author:
National Institutes of Health Consensus Development Conference. Neurofibromatosis conference statement. Arch Neurol 1988;45:575-578.
Article type specification:
Schreiner GF, Lange L. Ethanol modulation of macrophage influx in glomerulonephritis [Abstract]. J Am Soc Nephrol 1991;2:562.
Should antileukotriene therapies be used instead of inhaled corticosteroids in asthma? [Editorial]. Am J Respir Crit Care Med 1998;158:1697-1701.
Laux-End R, Inaebnit D, Gerber HA, Bianchetti MG. Vasculitis associated with levamisole and circulating autoantibodies [Letter]. Arch Dis Child 1996;75:355-356.
II. BOOKS
Chapter in book:
Clark AG, Barratt TM. Steroid-responsive nephrotic syndrome. In: Barratt TM, Arner ED, Harmon WE, editors. Pediatric Nephrology. 4th ed. Baltimore: Lippincott William Wilkins; 1999. p. 742.
Book or monograph:
Gorlin RJ, Cohen MM, Levin LS. Syndromes of the head and neck. 3rd ed. New York: Oxford University Press; 1990.
Publication in a volume of proceedings:
Bauer AW. The two definitions of bacterial resistance. In: Smith AJ, Rogers CA, eds. Proceedings of the Third International Congress of Chemotherapy; 1962 May 29-31; New York: International Society of Chemotherapy; 1963. p. 484-500.
Doctoral dissertation:
Kaplan SJ. Post hospital home health care: the elderly’s access and utilization [dissertation]. St. Louis (Mo): Washington Univ.; 1995.
πππ. CD-ROM
Anderson SC, Poulsen KB. Anderson’s electronic atlas of hematology [CD-ROM]. Philadelphia: Lippincott Williams & Wilkins; 2002.
IV. ON THE INTERNET
Article in journal
Abood S. Quality improvement initiative in nursing homes: the ANA acts in an advisory role. Am J Nurs [Internet]. 2002 Jun: Webpage: http://www.nursingworld.org/ AJN/2002/june/Wawatch.htm
Monograph
Foley KM, Gelband H, editors. Improving palliative care for cancer [Monograph, Internet]. Washington: National Academy Press; 2001. Webpage: http://www.nap.edu/books/0309074029/html
Websites
Cancer-Pain.org [Webpage, Internet]. New York: Association of Cancer Online Resources, Inc.; 2002: http://www.cancer-pain.org/
Tables and Figures
Three copies should be submitted (original plus 2 copies). Their width should either be equal to the width of one column (7.5 cm) or to the width of the page (15.5 cm). Their maximum length, titles included, should not exceed 22 cm.
Tables are numbered with Arabic numerals in the order in which they appear in the text. They should have a short title and abbreviations should be listed at the bottom. Vertical lines in tables should be avoided.
All illustration material is considered as figures (graphs, pictures, etc.). They should be of excellent quality. Also, at the back of every picture, the number of the picture and the name of the first author should be noted in pencil, with an arrow showing the top of the picture. The identity of patients should not be recognizable from their pictures nor should their names be stated.
C. Manuscript Submission and Publication
All manuscripts should be accompanied by a floppy disk or CD, as well as by a letter, signed by all the authors, in which it is stated that the paper has not been published in part or in whole, or is not under consideration by another journal and that the authors accept its publication in “Paediatriki”. Any funding, sponsorship or other financial support should be acknowledged.
Once the manuscript has been accepted, the corrected version, rewritten according to the reviewers’ recommendations should be submitted to the Editorial Board accompanied by a floppy disk or CD, containing the paper in Word format, along with a covering letter specifying in detail the modifications or objections to the reviewers’ suggestions.
Delay in submission of the modified paper exceeding 30 days entails new submission.
Authors will be charged film and reprint expenses, paid upon dispatch of the first proof directly to the printer.
Manuscripts of papers which have not been approved for publication are not returned to the author. The accompanying figures and photographs can be returned upon request within six months.
Manuscripts submitted for review and publication in “Paediatriki” should be sent in three copies to the following address: Editorial Board, Greek Paediatric Society 92, Michalakopoulou Street, 115 28 Athens, Greece
Before submitting your paper, make sure it contains:
1.3 copies of the text of the paper, printed according to instructions.
2.A floppy disk or CD with the entire material of the paper (text, tables, pictures).
3.A covering letter and a statement that the paper has not been previously published.
4.The title page (on a separate page), which includes: a.the title and short title of the paper; b.the name and surname (full name)of the author(s); c.the academic centre(s) of origin; d.the corresponding authors’ name, address and telephone number;
5.English and Greek abstracts, with the following structure: background, methods, results and conclusions (double space, separate page) and keywords.
6.List of abbreviations (double space, separate page).
7.Text (double space, separate page).
8.Acknowledgements and reference to funding, sponsorships or other financial sources.
9.References (double space, separate page).
10.Tables (one per page) in three copies.
11.Figures with an arrow at the back showing the top, numbered, in two copies.
12.Figure titles (double space - on separate pages) in three copies.
π·ÙÚÈ΋ ‚·ÛÈṲ̂ÓË ÛÙȘ ÂӉ›ÍÂȘ -
∆È
·ÏÏ¿˙ÂÈ ÛÙËÓ È·ÙÚÈ΋ Ú¿ÍË;
™. ∏Ï›·, µ. ∞ÁÁÂÏ¿ÎÔ˘
¶ÂÚ›ÏË„Ë: ∏ π·ÙÚÈ΋ µ·ÛÈṲ̂ÓË ÛÙȘ ∂Ӊ›ÍÂȘ (πµ∂) Â›Ó·È Ë “Â˘Û˘Ó›‰ËÙË, Û·Ê‹˜ Î·È Û˘ÓÂÙ‹
¯Ú‹ÛË ÙˆÓ ÈÔ ÚfiÛÊ·ÙˆÓ Î·È ÈÔ ·ÍÈfiÈÛÙˆÓ Â˘ÚËÌ¿ÙˆÓ Ù˘ ÙÚ¤¯Ô˘Û·˜ È·ÙÚÈ΋˜ ¤Ú¢ӷ˜ ÛÙË
Ï‹„Ë ·ÔÊ¿ÛÂˆÓ ÁÈ· ÙËÓ ÂÚ›ı·Ï„Ë ÙÔ˘ οı ·ÛıÂÓÔ‡˜ ˆ˜ ͯˆÚÈÛÙ‹˜ ÔÓÙfiÙËÙ·˜”, fiˆ˜ ÂÚÈÁÚ¿ÊËΠ·fi ÙÔÓ Sackett Î·È Û˘Ó. ∏ ·Ó·ÁηÈfiÙËÙ· ÙˆÓ ÛËÌÂÚÈÓÒÓ ÎÏÈÓÈÎÒÓ ÁÈ·ÙÚÒÓ Ó· ˘ÔÛÙËÚ›-
˙Ô˘Ó Ï¤ÔÓ ÙȘ ·ÔÊ¿ÛÂȘ ÙÔ˘˜ Ì ÂÈÛÙËÌÔÓÈο ·Î¤Ú·È˜ ÂӉ›ÍÂȘ, ÏfiÁˆ ÙˆÓ Û˘Ó¯Ҙ ·˘Í·Ófi-
ÌÂÓˆÓ È·ÙÚÈÎÒÓ ÏËÚÔÊÔÚÈÒÓ, ¤‰ˆÛ Á¤ÓÂÛË ÛÙËÓ È‰¤· ·˘Ù‹. ∏ ‰È·‰Èηۛ· πµ∂ ¤¯ÂÈ ÙË ‚¿ÛË Ù˘
Û ¤Ó· ÎÏÈÓÈÎfi Úfi‚ÏËÌ· Ô˘ ·ÓÙÈÌÂÙˆ›˙ÂÈ ¤Ó·˜ È·ÙÚfi˜, Ì·˙› Ì ÙÔÓ ·ÛıÂÓ‹ ÙÔ˘, Î·È ·ÎÔÏÔ˘ı› Ù· ÂÍ‹˜ ‚‹Ì·Ù·: ·Ú¯Èο ÌÂÙ·ÙÚ¤ÂÙ·È ÙÔ ÎÏÈÓÈÎfi Úfi‚ÏËÌ· ÛÂ
Û·ÊÒ˜ ‰È·Ù˘ˆÌ¤ÓË ÂÚÒÙËÛË, Ë ÔÔ›· ¯ÚËÛÈÌÔÔÈÂ›Ù·È ˆ˜ ‚¿ÛË ÁÈ· ÙË ‚È‚ÏÈÔÁÚ·ÊÈ΋ ·Ó·˙‹ÙËÛË. ∞ÎÔÏÔ˘ı› Ë ¤Ú¢ӷ ÙˆÓ ÈÔ
·ÍÈfiÈÛÙˆÓ ËÁÒÓ ÏËÚÔÊÔÚÈÒÓ ÛÙÔ ¢È·‰›ÎÙ˘Ô ÁÈ· ÙËÓ ·Ó‡ÚÂÛË ÙˆÓ Î·Ï‡ÙÂÚˆÓ ÂӉ›ÍˆÓ. ™ÙË
Û˘Ó¤¯ÂÈ· ·ÍÈÔÏÔÁÔ‡ÓÙ·È ÔÈ ÂӉ›ÍÂȘ ·˘Ù¤˜, fiÛÔÓ ·ÊÔÚ¿ ÙËÓ ÂÁ΢ÚfiÙËÙ· Î·È ÙËÓ ·ÍÈÔÈÛÙ›· ÙÔ˘˜.
∆ÂÏÂ˘Ù·›Ô ‚‹Ì· ÛÙËÓ ‰È·‰Èηۛ· πµ∂ Â›Ó·È Ë ÂÊ·ÚÌÔÁ‹ ÙˆÓ Î·Ï‡ÙÂÚˆÓ ÂӉ›ÍÂˆÓ ÛÙȘ ȉȷ›ÙÂÚ˜
∆.∫. 714 09 ∏Ú¿ÎÏÂÈÔ, ∫Ú‹ÙË ∆.£. 44 E-mail: stayrinah@hotmail.com
∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 05-07-2005
∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 10-02-2006
Evidence based medicine - What changes in clinical practice?
S. Ilia, V. Agelakou
Abstract: Evidence based medicine (EBM) is founded on the idea that decisions about the care of individual patients should encompass the "conscientious, explicit and judicious use of current best evidence", as outlined by Sackett and colleagues. The concept of EBM emerged as clinicians were obliged to integrate an ever-increasing amount of medical information into the clinical decision-making process. The EBM process concerns the clinical problem faced by a clinician and a patient and consists of specific steps. Initially, the practitioner should convert the clinical problem into a well-formulated answerable question, which can be used to run a literature search. According to the clinical query, the best evidence should be found by searching the most reputable sources of information on the Internet. The next step is to critically appraise the evidence and decide about its validity and reliability. The final step is to integrate the best evidence with clinical expertise and to apply it, taking into consideration the relevant values, preferences and expectations of the individual patient. This review describes the elements of EBM and demonstrates how they can be incorporated into clinical problems in a way which improves healthcare delivery and bridges the gap between medical practice and research.
Key words: Evidence based medicine, Internet, research, systematic review, critical appraisal.
Ist Pediatric Clinic, General Hospital “Venizeleio”, Herakleion, Crete
Correspondence: Stavroula Ilia
1st Pediatric Clinic Knossos Av, 714 09 Herakleion, Crete P.o. Box 44 E-mail: stayrinah@hotmail.com
Date of submission: 05-07-2005
Date of approval: 10-02-2006
EÈÛ·ÁˆÁ‹
∏ ¤ÓÓÔÈ· “π·ÙÚÈ΋ µ·ÛÈṲ̂ÓË ÛÙȘ ∂Ӊ›-
ÍÂȘ” (πµ∂) (Evidence Based Medicine) ÔÚ›ÛÙËΠÚÒÙË ÊÔÚ¿ ·fi ÌÈ· ÔÌ¿‰· ÎÏÈÓÈÎÒÓ ÂȉË-
ÌÈÔÏfiÁˆÓ ÙÔ 1990, (D. Sackett Î·È Û˘Ó., ¶·ÓÂ-
ÈÛÙ‹ÌÈÔ McMaster, ∫·Ó·‰¿˜), ÔÈ ÔÔ›ÔÈ ‰È·›-
ÛÙˆÛ·Ó ÙËÓ ·Ó·ÁηÈfiÙËÙ·, Û ڷÎÙÈÎfi ›Â-
‰Ô È·, Ó· ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ·ÍÈfiÈÛÙ˜ ηÈ
ÙÂÎÌËÚȈ̤Ó˜ ÏËÚÔÊÔڛ˜ Ù˘ È·ÙÚÈ΋˜ ‚È-
‚ÏÈÔÁÚ·Ê›·˜ Î·È ¤Ú¢ӷ˜ ‰›Ï· ÛÙÔ ÎÚ‚¿ÙÈ
ÙÔ˘ ·ÛıÂÓÔ‡˜ (1). ∏ ‰È·‰Èηۛ· πµ∂ ÔÚ›ÛÙËÎÂ
ˆ˜ Ë “Â˘Û˘Ó›‰ËÙË, Û·Ê‹˜ Î·È Û˘ÓÂÙ‹ ¯Ú‹ÛË
ÙˆÓ ÈÔ ÚfiÛÊ·ÙˆÓ Î·È ÈÔ ·ÍÈfiÈÛÙˆÓ Â˘ÚË-
Ì¿ÙˆÓ Ù˘ ÙÚ¤¯Ô˘Û·˜ È·ÙÚÈ΋˜ ¤Ú¢ӷ˜ ÛÙË
Ï‹„Ë ·ÔÊ¿ÛÂˆÓ ÁÈ· ÙËÓ ÂÚ›ı·Ï„Ë ÙÔ˘ οı ·ÛıÂÓÔ‡˜ ˆ˜ ȉȷ›ÙÂÚË
1.
ÂÛÙÈ·Ṳ̂ÓÔ ÎÏÈÓÈÎfi ÂÚÒÙËÌ·.
2. ∞Ó¿Ù˘ÍË ÛÙÚ·ÙËÁÈÎÒÓ ÁÈ· ·ÔÙÂÏÂÛÌ·-
ÙÈ΋ ·Ó·˙‹ÙËÛË ÙˆÓ Î·Ï‡ÙÂÚ· ÙÂÎÌËÚȈ̤ӈÓ
ÂÈÛÙËÌÔÓÈÎÒÓ ‰Â‰ÔÌ¤ÓˆÓ (best evidence)
ÛÙËÓ È·ÙÚÈ΋ ‚È‚ÏÈÔÁÚ·Ê›·, Ô˘ ÂӉ¯Ô̤ӈ˜
ı· ‰ÒÛÔ˘Ó ·¿ÓÙËÛË ÛÙÔ ÎÏÈÓÈÎfi ÂÚÒÙËÌ·.
3. ∫ÚÈÙÈ΋ ·ÍÈÔÏfiÁËÛË ÙˆÓ ‚ÈÔ˚·ÙÚÈÎÒÓ ‰Ë-
ÌÔÛÈÂ˘Ì¿ÙˆÓ Ô˘ ·Ó¤‰ÂÈÍÂ Ë ¤Ú¢ӷ, fiÛÔÓ
·ÊÔÚ¿ ÙËÓ ÂÁ΢ÚfiÙËÙ· Î·È ÙËÓ ÏËÚfiÙËÙ· ÙˆÓ
ÏËÚÔÊÔÚÈÒÓ Ô˘ ÚÔÛʤÚÔ˘Ó Î·ıÒ˜ Î·È ÙËÓ
·ÓÙÈÛÙÔȯ›· ÙÔ˘˜ Ì ÙÔ Û˘ÁÎÂÎÚÈ̤ÓÔ ÎÏÈÓÈÎfi
ÂÚÒÙËÌ·.
4. ∂Ê·ÚÌÔÁ‹ ÙˆÓ Î·Ï‡ÙÂÚˆÓ ÂӉ›ÍÂˆÓ ÛÙËÓ
ÎÏÈÓÈ΋ Ú¿ÍË, ÛÂ Û˘Ó‰˘·ÛÌfi ¿ÓÙÔÙÂ, Ì ٷ ȉȷ›ÙÂÚ· ¯·Ú·ÎÙËÚÈÛÙÈο ÙÔ˘ οı ·ÛıÂÓÔ‡˜.
5. ∞ÍÈÔÏfiÁËÛË ÙÔ˘ ·ÔÙÂϤÛÌ·ÙÔ˜ ÌÂÙ¿ ·fi ·˘Ù‹ ÙËÓ ÔÚıÔÏÔÁÈο ‰ÔÌË̤ÓË Î·È Û˘-
ÛÙËÌ·ÙÈ΋ ÚÔÛ¤ÁÁÈÛË ÙˆÓ ÎÏÈÓÈÎÒÓ ÚÔ‚ÏËÌ¿ÙˆÓ Î·È Ï‹„Ë ÎÏÈÓÈÎÒÓ ·ÔÊ¿ÛÂˆÓ ÌÂÙ¿
Ï‹„Ë ÎÏÈÓÈÎÒÓ ·ÔÊ¿ÛˆÓ
Û ȷÙÚÈο ‚È‚Ï›·, οÔÈ· ·fi ·˘Ù¿ ›Ûˆ˜ Ó· ¯ÚÔÓÔÏÔÁÔ‡ÓÙ·Ó Î·È ·fi ‰ÂηÂÙÈÒÓ (!), ‰ÈÂıÓ‹ È·ÙÚÈο ÂÚÈÔ‰Èο, Ô˘ fï˜ Ë ÚfiÛ‚·ÛË ÛÙ· ÙÂÏÂ˘Ù·›· ‹Ù·Ó ÂÚÈÔÚÈṲ̂ÓË ·fi ÙÔ˘˜ ÂÚÈÛÛfiÙÂÚÔ˘˜ ÁÈ·ÙÚÔ‡˜, fiˆ˜ ›Û˘ Î·È ÛÙËÓ ÚÔÛˆÈ΋ ›ڷ ÙÔ˘ οı ÎÏÈÓÈÎÔ‡. ™‹ÌÂÚ·, Ô Ó¤Ô˜ È·ÙÚfi˜, ÏfiÁˆ ÂÎÚËÎÙÈ΋˜ ·Ó¿Ù˘Í˘ ÙÔ˘ ¢È·‰ÈÎÙ‡Ô˘, ¤¯ÂÈ ÚfiÛ‚·ÛË Û ÌÂÁ¿ÏÔ fiÁÎÔ Ù˘ È·ÙÚÈ΋˜ ‚È‚ÏÈÔÁÚ·Ê›·˜, ÂÎÛ˘Á¯ÚÔÓ›˙ÂÈ Û˘Ó¯Ҙ ÙȘ ÁÓÒÛÂȘ ÙÔ˘, ÂÍÔÈÎÂÈÒÓÂÙ·È Ì ÌÂıfi‰Ô˘˜ Ù˘ ÎÏÈÓÈ΋˜ ¤Ú¢ӷ˜, ÙËÚ› ÎÚÈÙÈ΋ ÛÙ¿ÛË ·¤Ó·ÓÙÈ ÛÙ· ÂÚ¢ÓËÙÈο ·ÔÙÂϤÛÌ·Ù· Î·È Â›Ó·È Û›ÁÔ˘ÚÔ˜ ÁÈ· ÙË ÏÔÁÈ΋ ÂÍ‹ÁËÛË Î·È ÔÚıfiÙËÙ· ÙˆÓ ·ÔÊ¿ÛÂÒÓ ÙÔ˘ (4). ŒÙÛÈ ·ÎÔÏÔ˘ıÂ›Ù·È ÌÈ· ÈÔ ·‰ÔÁÌ¿ÙÈÛÙË, ÈÔ Û˘ÛÙËÌ·ÙÈ΋, ÈÔ ·Ó·Ï˘ÙÈ΋ Î·È ÈÔ ·˘ÛÙËÚ‹ ÌÂıÔ‰ÔÏÔÁ›· Ï‹„˘ ÎÏÈÓÈÎÒÓ ·ÔÊ¿ÛˆÓ. ™˘ÌÂÚ·ÛÌ·ÙÈο, Ë ‰È·‰Èηۛ· πµ∂ ¤ÁÎÂÈÙ·È ÛÙË Û˘ÛÙËÌ·ÙÈ΋ ·Ó‡ÚÂÛË, ·ÍÈÔÏfiÁËÛË Î·È ÂÊ·ÚÌÔÁ‹ ÙˆÓ ·ÓÙ›ÛÙÔȯˆÓ
ÙÚ¤¯Ô˘Û·˜ ¤Ú¢ӷ˜ ÛÙÔ ÂοÛÙÔÙ ÎÏÈÓÈÎfi Úfi‚ÏËÌ·.
∆Ô ÎÏÈÓÈÎfi ÂÚÒÙËÌ·
∆Ô ÈÔ ÛËÌ·ÓÙÈÎfi ‚‹Ì· ÛÙË ‰È·‰Èηۛ· πµ∂ Â›Ó·È Ë ÌÂÙ·ÙÚÔ‹ ÙÔ˘ ÎÏÈÓÈÎÔ‡ ÚÔ‚Ï‹Ì·ÙÔ˜ Û ۈÛÙ¿ ‰ÔÌË̤ÓÔ ÂÚÒÙËÌ· (frame question). ªÂÙ¿ ÙË Ï‹„Ë ÈÛÙÔÚÈÎÔ‡ Î·È ÙËÓ ÎÏÈÓÈ΋ ÂͤٷÛË ·Ó·‰‡ÔÓÙ·È ÔÏÏ¿ ÂÚˆÙ‹Ì·Ù· Ô˘ Û¯ÂÙ›˙ÔÓÙ·È Ì ÙË ‰È¿ÁÓˆÛË, ÙË ıÂڷ›·, ÙËÓ ÚfiÁÓˆÛË Î·È ÙËÓ ÚfiÏË„Ë Ù˘ Èı·Ó‹˜ ÓfiÛÔ˘. ∏ ‰È·Ù‡ˆÛË ÙˆÓ ÂÚˆÙËÌ¿ÙˆÓ ·˘ÙÒÓ Á›ÓÂÙ·È ÌÂ Û˘ÁÎÂÎÚÈ̤ÓÔ ÙÚfiÔ Î·È ·ÔÙÂÏÂ›Ù·È ·fi Ù· ÂÍ‹˜ Ù¤ÛÛÂÚ· ̤ÚË:
1. √Ì¿‰· ÛÙfi¯Ô˜ (population): ÏËı˘ÛÌfi˜ ÌÂ Û˘ÁÎÂÎÚÈ̤ӷ ¯·Ú·ÎÙËÚÈÛÙÈο fiÌÔÈ· Ì ÙÔ˘ ·ÛıÂÓÔ‡˜, fiˆ˜ .¯. ʇÏÔ, ËÏÈΛ·, Û˘ÁÎÂÎÚÈ̤ÓË ·Ûı¤ÓÂÈ· Î.¿.
2. ¶·Ú¤Ì‚·ÛË/ ŒÎıÂÛË (intervention/ exposure): ηıÔÚ›˙ÂÙ·È ˆ˜ ·ÈÙÈÔÏÔÁ›·, ‰È¿ÁÓˆÛË, ıÂڷ›·, ÚfiÁÓˆÛË, ÚfiÏË„Ë Î.¿.
3. √Ì¿‰· Û‡ÁÎÚÈÛ˘ (control group): fiÔ˘ Â›Ó·È ÂÊ·ÚÌfiÛÈÌÔ .¯. ıÂڷ›· placebo, ÂÓ·ÏÏ·ÎÙÈÎfi ‰È·ÁÓˆÛÙÈÎfi test Î.Ï. 4. ∫ÏÈÓÈ΋ ¤Î‚·ÛË (outcome): Â›Ó·È ÙÔ ˙ËÙÔ‡ÌÂÓÔ ÌÂÙ¿ ÙËÓ ·Ú¤Ì‚·ÛË/¤ÎıÂÛË Ô˘ ı· ¤ÏıÂÈ Î·È ı· ÂËÚ¿ÛÂÈ ÙËÓ ÎÏÈÓÈ΋
5.
·ÔÊ˘Á‹ ÂÈÏÔÎÒÓ, ÈηÓfiÙËÙ· ÂÓfi˜ ̤ÛÔ˘ Ó·
ÂȂ‚·ÈÒÓÂÈ ÌÈ· ·Ûı¤ÓÂÈ· Î.¿. (5). °È· Ó· Á›ÓÂÈ ÈÔ Î·Ù·ÓÔËÙ‹ Ë ‰È·‰Èηۛ· πµ∂,
·Ú·ı¤ÙÔ˘Ì ٷ ·Ú·Î¿Ùˆ ·Ú·‰Â›ÁÌ·Ù·, Ù·
ÔÔ›· Ê·›ÓÔÓÙ·È Û˘ÓÔÙÈο ÛÙÔÓ ¶›Ó·Î· 2:
¶·Ú¿‰ÂÈÁÌ· 1: ∞ÁfiÚÈ 3 ÂÙÒÓ, Ì ÂχıÂÚÔ
·ÙÔÌÈÎfi ·Ó·ÌÓËÛÙÈÎfi, ÚÔÛ¤Ú¯ÂÙ·È ÛÙÔ Â͈ÙÂ-
ÚÈÎfi ·È‰È·ÙÚÈÎfi È·ÙÚÂ›Ô ÂÂÈÁfiÓÙˆÓ ÂÚÈÛÙ·-
ÙÈÎÒÓ Ì ˘ÚÂÙfi ¤ˆ˜ 38ÆC, ÚÈÓ›Ùȉ·, ‚‹¯· ηÈ
¤ÓÙÔÓË ˆÙ·ÏÁ›·. ∏ ÎÏÈÓÈ΋ ÂͤٷÛË ·Ôηχ-
ÙÂÈ ÔÍ›· ̤ÛË ˆÙ›Ùȉ· (√ªø). ∆Ô ÂÚÒÙËÌ·
Ô˘ ·Ó·‰‡ÂÙ·È ‰È·Ù˘ÒÓÂÙ·È, Ì ‚¿ÛË Ù· ·-
Ú·¿Óˆ, ˆ˜ ÂÍ‹˜:
ñ (ÏËı˘ÛÌfi˜) ·È‰È¿ ÚÔÛ¯ÔÏÈ΋˜ ËÏÈΛ·˜
Ì √ªø
ñ (·Ú¤Ì‚·ÛË) ¯ÔÚ‹ÁËÛË ·ÓÙÈ‚ÈÔÙÈ΋˜
·ÁˆÁ‹˜
ñ (ÔÌ¿‰· Û‡ÁÎÚÈÛ˘) ÌË ¯ÔÚ‹ÁËÛË ·ÓÙÈ‚ÈÔ-
ÙÈ΋˜ ·ÁˆÁ‹˜
ñ (ÎÏÈÓÈ΋ ¤Î‚·ÛË) ‡ÊÂÛË ˆÙ·ÏÁ›·˜ - ·ÔÊ˘Á‹ ÂÈÏÔÎÒÓ √ªø - ·ÓÂÈı‡ÌËÙ˜ ÂÓ¤Ú-
ÁÂȘ ·ÓÙÈ‚ÈÔÙÈÎÒÓ Î.Ï.
ñ ‰È·Ù‡ˆÛË, ÏÔÈfiÓ, ÙÔ˘ ÂÚˆÙ‹Ì·ÙÔ˜: “ÃÚ‹˙ÂÈ ·ÓÙÈ‚›ˆÛ˘ ¤Ó· ·È‰› Ì √ªø;” ÌÂÙ·ÙÚ¤ÂÙ·È Û‡Ìʈӷ Ì ٷ ·Ú·¿Óˆ: “™Â ·È‰È¿ ÚÔÛ¯ÔÏÈ΋˜ ËÏÈΛ·˜ Ì √ªø (ÏËı˘ÛÌfi˜), Â›Ó·È ‰˘Ó·ÙfiÓ Ë ¯ÔÚ‹ÁËÛË ·ÓÙÈ‚ÈÔÙÈÎÔ‡
(·Ú¤Ì‚·ÛË) -Û ۯ¤ÛË Ì ÙË ÌË ¯ÔÚ‹ÁËÛË
·ÓÙÈ‚ÈÔÙÈÎÔ‡ (ÔÌ¿‰· Û‡ÁÎÚÈÛ˘)- Ó· ·Ì‚χÓÂÈ
Ù· Û˘ÌÙÒÌ·Ù· Ù˘ ˆÙ·ÏÁ›·˜, ÙÔ˘ ˘ÚÂÙÔ‡ Î·È Ù˘ ÚÈÓ›Ùȉ·˜; Ó· ÌÂÈÒÛÂÈ ÙÔ ÔÛÔÛÙfi ÙˆÓ ÂÈÏÔÎÒÓ √ªø, fiˆ˜ ÎÒʈÛË, ÌËÓÈÁÁ›Ùȉ· ‹
Ì·ÛÙÔÂȉ›Ùȉ·; Ó· ÂÈ‚·Ú‡ÓÂÈ ÙÔÓ ·ÛıÂÓ‹ ÏfiÁˆ
ÙˆÓ ·ÓÂÈı‡ÌËÙˆÓ ÂÓÂÚÁÂÈÒÓ ÙˆÓ ·ÓÙÈ‚ÈÔÙÈÎÒÓ (ÎÏÈÓÈ΋ ¤Î‚·ÛË);” (6).
¶·Ú¿‰ÂÈÁÌ· 2: ∆Ô ÎÏÈÓÈÎfi ÂÚÒÙËÌ· “¯ÚÂÈ¿˙ÂÙ·È Ó· ÓÔÛËχԢÌ ·È‰È¿ Ì ÂÂÈÛfi‰ÈÔ ·ÏÒÓ ˘ÚÂÙÈÎÒÓ Û·ÛÌÒÓ;” ‰È·Ù˘ÒÓÂÙ·È, Û‡Ìʈӷ Ì ٷ
ˆ˜ ÂÍ‹˜: “™Â ·È‰È¿
0-6 ÂÙÒÓ Ì ·ÏÔ‡˜ ˘ÚÂÙÈÎÔ‡˜ Û·ÛÌÔ‡˜ (ÏËı˘ÛÌfi˜), Ë 24ˆÚË ÓÔÛËÏ›· (·Ú¤Ì‚·ÛË)
Û ۇÁÎÚÈÛË Ì ÙËÓ 6ˆÚË ·Ú·ÎÔÏÔ‡ıËÛË (ÔÌ¿‰· Û‡ÁÎÚÈÛ˘), ÌÂÈÒÓÂÈ ÙËÓ Èı·ÓfiÙËÙ· ˘ÔÙÚÔ‹˜ ÙˆÓ ˘ÚÂÙÈÎÒÓ Û·ÛÌÒÓ (·ÔÙ¤ÏÂÛÌ·);”
¶·Ú¿‰ÂÈÁÌ· 3: ∆Ô ÎÏÈÓÈÎfi ÂÚÒÙËÌ· “¶Ú¤-
ÂÈ Ó· ÂÌ‚ÔÏÈ¿˙ÔÓÙ·È Ù· ‚Ú¤ÊË Ì ÙÔ ÂÌ‚fiÏÈÔ
ÙÔ˘ ÎÔηÙË;” ÌÂÙ·ÙÚ¤ÂÙ·È ÛÙÔ ÂÍ‹˜: “™Â
‚Ú¤ÊË 2 ÌËÓÒÓ (ÏËı˘ÛÌfi˜), Ô ÂÌ‚ÔÏÈ·ÛÌfi˜
Ì ÙÔ ÂÌ‚fiÏÈÔ ÙÔ˘ ÎÔηÙË (·Ú¤Ì‚·ÛË), ÛÂ
Û‡ÁÎÚÈÛË Ì ηıfiÏÔ˘ ÂÌ‚ÔÏÈ·ÛÌfi (ÔÌ¿‰· Û‡ÁÎÚÈÛ˘), ÌÂÈÒÓÂÈ ÙË ÓÔÛËÚfiÙËÙ· Ù˘ ÓfiÛÔ˘ ÙÔ˘ ÎÔηÙË; ·˘Í¿ÓÂÈ ÙË ÓÔÛËÚfiÙËÙ· Ô˘ Û¯ÂÙ›˙ÂÙ·È Ì ÙÔ Û˘ÁÎÂÎÚÈ̤ÓÔ ÂÌ‚fiÏÈÔ; (·ÔÙ¤ÏÂÛÌ· Ô˘ ·Ó·˙ËÙÔ‡ÌÂ)”.
∞Ó·˙‹ÙËÛË
ηٿÏÏËψÓ
ÂӉ›ÍˆÓ
‚È‚ÏÈÔÁÚ·Ê›· ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È ÌÂϤ-
Ù˜ Û¯ÂÙÈο Ì ÙË ‰È¿ÁÓˆÛË, ÙËÓ ÚfiÁÓˆÛË, ÙËÓ ·ÈÙÈÔÏÔÁ›· Î·È ÙË ıÂڷ›·, ı¤Ì·Ù· ‰ËÏ·‰‹ Ô˘ ηıËÌÂÚÈÓ¿ ··Û¯ÔÏÔ‡Ó ÙÔ˘˜ ÎÏÈÓÈÎÔ‡˜ È·ÙÚÔ‡˜. ∞Ó¿ÏÔÁ· Ì ÙÔÓ Ù‡Ô ÙÔ˘ ÎÏÈÓÈÎÔ‡ ÂÚˆÙ‹Ì·ÙÔ˜ ·Ó·˙ËÙÔ‡ÌÂ Î·È ÙËÓ ·ÓÙ›ÛÙÔȯ· ۯ‰ȷṲ̂ÓË ÌÂϤÙË (¶›Ó·Î·˜ 3). ∂¿Ó, ÁÈ· ·Ú¿‰ÂÈÁÌ·, ÙÔ ÎÏÈÓÈÎfi ÂÚÒÙËÌ· ·ÊÔÚ¿ ıÂڷ›· ÌÈ·˜ ÓfiÛÔ˘, ÙfiÙ ·Ó·˙ËÙÔ‡ÌÂ Ù˘¯·ÈÔÔÈË̤Ó˜ ÂÏÂÁ¯fiÌÂÓ˜ ÌÂϤÙ˜ (Randomized Control Trials - RCTs) Ô˘ ·ÔÙÂÏÔ‡Ó ÚfiÙ˘Ô
¶›Ó·Î·˜ 2. ¶·Ú·‰Â›ÁÌ·Ù· ‰È·Ù‡ˆÛ˘ ÎÏÈÓÈÎÒÓ ÚÔ‚ÏËÌ¿ÙˆÓ πµE
OÌ¿‰· ÛÙfi¯Ô˜/ ¶·Ú¤Ì‚·ÛË/ √Ì¿‰· Û‡ÁÎÚÈÛ˘ŒÎ‚·ÛË ¶ÏËı˘ÛÌfi˜¤ÎıÂÛË
ËÏÈΛ·˜ Ì √ªø·ÓÙÈ‚ÈÔÙÈÎÒÓ·ÓÙÈ‚ÈÔÙÈÎÒÓÛ˘ÌÙˆÌ¿ÙˆÓ √ªø (˘ÚÂÙfi˜, ˆÙ·ÏÁ›· Î.Ï.)
ñ ∞ÔÊ˘Á‹ ÂÈÏÔÎÒÓ √ªø (Ì·ÛÙÔÂȉ›Ùȉ·, ÎÒʈÛË Î.Ï.)
¶·È‰È¿ 0-6 ÂÙÒÓ 24ˆÚË ÓÔÛËÏ›·6ˆÚË ·Ú·ÎÔÏÔ‡ıËÛË ªÂ›ˆÛË
Ì ·ÏÔ‡˜ ˘ÚÂÙÈÎÔ‡˜
›‰Ô˜ ¤Ú¢ӷ˜ ÁÈ· ÙÔÓ ¤ÏÂÁ¯Ô Ù˘ ·ÔÙÂÏÂÛÌ·-
ÙÈÎfiÙËÙ·˜ ıÂڷ¢ÙÈÎÒÓ Ì¤ÛˆÓ. ¶Ôχ ÂÚÈÛ-
ÛfiÙÂÚÔ ·Ó·˙ËÙÔ‡ÌÂ Û˘ÛÙËÌ·ÙÈΤ˜ ·Ó·ÛÎÔ‹-
ÛÂȘ ·˘ÙÒÓ ÙˆÓ ÌÂÏÂÙÒÓ Ì Èı·Ó‹ ÌÂÙ·-·Ó¿-
Ï˘ÛË ÙˆÓ ·ÔÙÂÏÂÛÌ¿ÙˆÓ (Systematic Review (SR) of RCTs - Meta-analyses of SR of RCTs)
Ô˘ Â›Ó·È ·ÎfiÌ· ¤Ó· ‚‹Ì· ÈÔ ¤Ú·, ·ÊÔ‡ Ù·
·ÔÙÂϤÛÌ·Ù· ÔÏÏ·ÏÒÓ Ù˘¯·ÈÔÔÈË̤ӈÓ
ÌÂÏÂÙÒÓ Û˘Ó‰˘¿˙ÔÓÙ·È ÌÂٷ͇ ÙÔ˘˜ Î·È ·ÔÚ-
Ú¤Ô˘Ó Û˘ÁÎÂÓÙÚˆÙÈο ·ÔÙÂϤÛÌ·Ù·. °È· ÙÔ˘˜
˘fiÏÔÈÔ˘˜ Ù‡Ô˘˜ ÎÏÈÓÈÎÒÓ ÂÚˆÙËÌ¿ÙˆÓ ·-
Ú·¤ÌÔ˘Ì ÛÙÔÓ ¶›Ó·Î· 3. ∂›Ó·È, ÏÔÈfiÓ, Ô-
χ ÛËÌ·ÓÙÈÎfi Ó· ÁÓˆÚ›˙Ô˘Ì ÂÍ·Ú¯‹˜ ÙÔ Â›‰Ô˜
Ù˘ ÌÂϤÙ˘ Ô˘ ·ÓÙÈÛÙÔȯ› ÛÙÔÓ Ù‡Ô ÙÔ˘
ÂÚˆÙ‹Ì·Ùfi˜ Ì·˜ (‰È¿ÁÓˆÛË - ·ÈÙÈÔÏÔÁ›· - ıÂڷ›· - ÚfiÁÓˆÛË), ·ÊÔ‡ ¤ÙÛÈ ÂÍ·ÛÊ·Ï›˙Ô˘ÌÂ
ηٿ ÌÂÁ¿ÏÔ Ì¤ÚÔ˜ ÙËÓ ÂÁ΢ÚfiÙËÙ· Î·È ÙËÓ ·ÍÈÔÈÛÙ›· ÙˆÓ ÏËÚÔÊÔÚÈÒÓ
Pediatrics ÁÈ· ·È‰È·ÙÚÈο ı¤Ì·Ù·, ÙÔ Red Book ÁÈ· ÏÔÈÌÒ‰Ë ÓÔÛ‹Ì·Ù·, ÙÔ ∂ıÓÈÎfi ™˘ÓÙ·ÁÔÏfiÁÈÔ ÙÔ˘ ∂√º ÁÈ· Ê¿Ú̷η ÛÙË ¯ÒÚ· Ì·˜ Î.Ô.Î., ηıÒ˜ Î·È ÂÚÈÔ‰Èο ÂÈÛÙËÌÔÓÈÎÔ‡ ÂÚȯÔ̤ÓÔ˘, fiˆ˜, .¯. The Lancet. ∂›Û˘ ·ÚÎÂÙ¿ ·fi Ù· ÂÁ¯ÂÈÚ›‰È· Ô˘ ÚԷӷʤÚıËηÓ, ηıÒ˜ Î·È ¿ÏÏ·, ˘¿Ú¯Ô˘Ó Î·È Û ËÏÂÎÙÚÔÓÈΤ˜ ÂΉfiÛÂȘ ÛÙÔ ‰È·‰›ÎÙ˘Ô. ∂Ó‰ÂÈÎÙÈο ·Ó·Ê¤ÚÔÓÙ·È Nelson on-line, Harrison on-line, e-Medicine, Archives of Disease in Childhood, Pediatrics eedition Î.Ï., Ô˘ Û˘Ó¯Ҙ ‰›‰Ô˘Ó ÏËÚÔÊÔڛ˜ Î·È Ì¿ÏÈÛÙ· ·Ó·ÓÂÒÓÔ˘Ó ÙÔ ˘ÏÈÎfi ÙÔ˘˜ Ôχ ÈÔ ¿ÌÂÛ· ·fi ÙȘ ÁÚ·Ù¤˜ ÂΉfiÛÂȘ.
ÙˆÓ
ηχÙÂÚˆÓ ÙÚÂ¯Ô˘ÛÒÓ ÂӉ›ÍÂˆÓ (best current evidence) ÁÈ· ÙË Ï‹„Ë ÙˆÓ Î·Ù·ÏÏËÏfiÙÂÚˆÓ
ÎÏÈÓÈÎÒÓ ·ÔÊ¿ÛÂˆÓ Ô˘ ·ÊÔÚÔ‡Ó ÙÔ˘˜ ·ÛıÂÓ›˜ Ì·˜ ÙË ‰Â‰Ô̤ÓË ¯ÚÔÓÈ΋ ÛÙÈÁÌ‹.
¶ËÁ¤˜ ÂӉ›ÍÂˆÓ ™Ù· Ï·›ÛÈ· ·Ó·˙‹ÙËÛ˘ ··ÓÙ‹ÛÂˆÓ ÛÙȘ
ηıËÌÂÚÈÓ¤˜ ÎÏÈÓÈΤ˜ ·Ôڛ˜ ¯ÚËÛÈÌÔÔÈÔ‡-
ÓÙ·È ·Ó·ÁÓˆÚÈṲ̂ӷ È·ÙÚÈο ÂÁ¯ÂÈÚ›‰È· Î·È ‚È-
‚Ï›·, Ô˘ Û˘Ó‹ıˆ˜ ‚Ú›ÛÎÔÓÙ·È ÛÙËÓ ÚÔÛˆÈ΋
‚È‚ÏÈÔı‹ÎË ÙÔ˘ οı ȷÙÚÔ‡. ÷ڷÎÙËÚÈÛÙÈο ·Ú·‰Â›ÁÌ·Ù· ›ӷÈ: ÙÔ Nelson-Textbook of
΋˜ ÌÂϤÙ˘
£Âڷ›·
ñ ∆˘¯·ÈÔÔÈË̤Ó˜ ÂÏÂÁ¯fiÌÂÓ˜ ÌÂϤÙ˜ (∆∂ª) (Randomised Control Trials (RCTs))
ñ ™˘ÛÙËÌ·ÙÈ΋ ·Ó·ÛÎfiËÛË ∆∂ª
ñ ªÂÙ·-·Ó¿Ï˘ÛË Û˘ÛÙËÌ·ÙÈÎÒÓ ·Ó·ÛÎÔ‹ÛÂˆÓ ∆∂ª
¢È¿ÁÓˆÛË (screening)
ñ ™˘Á¯ÚÔÓÈΤ˜ ÌÂϤÙ˜ (cross-sectional studies)
ñ ™˘ÛÙËÌ·ÙÈ΋ ·Ó·ÛÎfiËÛË Û˘Á¯ÚÔÓÈÎÒÓ ÌÂÏÂÙÒÓ
∞ÈÙÈÔÏÔÁ›· ñ ªÂϤÙ˜ ·ÛıÂÓÒÓ-Ì·ÚÙ‡ÚˆÓ (case-control studies)
ñ ¢È·¯ÚÔÓÈΤ˜ ÌÂϤÙ˜ (cohort studies)
¶ÚfiÁÓˆÛË
ñ ¢È·¯ÚÔÓÈΤ˜ ÌÂϤÙ˜ (cohort studies)
ª›· ¿ÏÏË ËÁ‹ ÏËÚÔÊÔÚÈÒÓ Â›Ó·È ÔÈ ËÏÂÎÙÚÔÓÈΤ˜ ‚¿ÛÂȘ ‰Â‰ÔÌ¤ÓˆÓ (electronic databases), ÔÈ Ôԛ˜ ‰È·ÎÚ›ÓÔÓÙ·È Û ‰‡Ô ηÙËÁÔڛ˜: ·) ‚¿ÛÂȘ ‰Â‰ÔÌ¤ÓˆÓ ¯ˆÚ›˜ Ê›ÏÙÚÔ. ÷ڷÎÙËÚÈÛÙÈÎfi ·Ú¿‰ÂÈÁÌ· ·˘Ù‹˜ Ù˘ ηÙËÁÔÚ›·˜ Â›Ó·È ÙÔ Û‡ÛÙËÌ· MEDLINE, ÙÔ ÔÔ›Ô Û˘ÓÙ¿ÛÛÂÙ·È ·fi ÙËÓ National Library of Medicine ÙˆÓ ∏ÓˆÌ¤ÓˆÓ ¶ÔÏÈÙÂÈÒÓ. ªÔÚԇ̠ӷ Ê·ÓÙ·ÛÙԇ̠ÌÈ· ÙÂÚ¿ÛÙÈ· ‚È‚ÏÈÔı‹ÎË Ì ·Ì¤ÙÚËÙ· Ú¿ÊÈ· fiÔ˘ ˘¿Ú¯Ô˘Ó ¿ÚıÚ· È·ÙÚÈÎÔ‡ ÂÚȯÔ̤ÓÔ˘ ÂÚÈÛÛfiÙÂÚ· ·fi 2.000.000 Û ·ÚÈıÌfi Î·È ÚÔ¤Ú¯ÔÓÙ·È ·fi ¿Óˆ ·fi 70 ¯ÒÚ˜, fiÔ˘ Û˘Ó¯Ҙ ·Ú¯ÂÈÔıÂÙÔ‡ÓÙ·È Ó¤· (ÂÚ›Ô˘ 1.000) ¿ÚıÚ· ËÌÂÚËÛ›ˆ˜. ∞˘Ù‹ Ë ËÏÂÎÙÚÔÓÈ΋ ‚È‚ÏÈÔı‹ÎË Â›Ó·È Â‡ÎÔÏ· Î·È ‰ˆÚÂ¿Ó ÚÔÛ‚¿ÛÈÌË Ì¤Ûˆ ÙÔ˘ ¢È·‰ÈÎÙ‡Ô˘ (http://www.ncbi.nlm.nih. gov/PubMed/). TÔ Û‡ÛÙËÌ· Medline Â›Ó·È Ì›· ·fi ÙȘ ÂÚ›Ô˘ 40 Ù¤ÙÔȘ ËÏÂÎÙÚÔÓÈΤ˜ ‚¿ÛÂȘ ‰Â‰ÔÌ¤ÓˆÓ fiÔ˘ ηٷ¯ˆÚÂ›Ù·È -¯ˆÚ›˜ fï˜ Ó· ·ÍÈÔÏÔÁÂ›Ù·È ÙÔ ÂÚȯfiÌÂÓfi ÙÔ˘˜ (¯ˆÚ›˜ Ê›ÏÙÚÔ)- ¤Ó·˜ ÁÈÁ·ÓÙÈ·›Ô˜ ·ÚÈıÌfi˜ ÂÈÛÙËÌÔÓÈÎÒÓ ¿ÚıÚˆÓ, fiÔ˘ Ô ·Ó·ÁÓÒÛÙ˘ ηÏÂ›Ù·È Ó· ·ÔÙÈÌ‹ÛÂÈ ÙËÓ ÔÈfiÙËÙ· ÙˆÓ ÏËÚÔÊÔÚÈÒÓ Ô˘ Ï·Ì‚¿ÓÂÈ (7). ‚) ‚¿ÛÂȘ ‰Â‰ÔÌ¤ÓˆÓ Ì ʛÏÙÚÔ. ™ÙËÓ Î·ÙËÁÔÚ›· ·˘Ù‹ ÔÈ ÌÂϤÙ˜ Ô˘ ÚÔÛʤÚÔÓÙ·È ÛÙÔÓ ¯Ú‹ÛÙË ¤¯Ô˘Ó ‹‰Ë ˘ÔÛÙ› ·˘ÛÙËÚ‹ ·ÍÈÔÏfiÁËÛË. ∂ÈÙÚÔ¤˜ ·fi ÂÍÂȉÈÎÂ˘Ì¤ÓÔ˘˜ ÂÈÛÙ‹ÌÔÓ˜ ¿ӈ ÛÙÔÓ Û¯Â‰È·ÛÌfi ÂÚ¢ÓÒÓ ÂÍ·ÛÊ·Ï›˙Ô˘Ó ÙËÓ ·ÚÔ¯‹ ÏËÚÔÊÔÚÈÒÓ ˘„ËÏ‹˜ ÔÈfiÙËÙ·˜, Ì ÌÂȈ̤ӷ Ù· ÂÚÈıÒÚÈ· ÌÂÚÔÏË„›·˜, ‚·ÛÈ˙fiÌÂÓ˜ ÛÙ· ηχÙÂÚ· ‚È‚ÏÈÔÁÚ·ÊÈο ‰Â‰Ô̤ӷ Ô˘ ¤¯ÂÈ Ó· ·Ó·‰Â›ÍÂÈ Ë ÙÚ¤¯Ô˘Û· ¤Ú¢ӷ. £· ÙÔÏÌÔ‡Û·Ì ӷ ԇ̠ˆ˜ ·ÔÙÂÏ› ÙÔ ›‰ÈÔ ÙÔ ‚‹Ì· 2 Ù˘ π·ÙÚÈ΋˜ µ·ÛÈṲ̂Ó˘ ÛÙȘ ∂Ӊ›ÍÂȘ (Evidence Based Medicine) (‚Ϥ ¶›Ó·Î· 1), fiÔ˘ οÔÈÔÈ ÙÚ›ÙÔÈ ¤¯Ô˘Ó οÓÂÈ ÙË ‰Ô˘ÏÂÈ¿ ÁÈ· Ì·˜ ÛÙË ‰È·‰Èηۛ· ·Ó·˙‹ÙËÛ˘ Î·È Û˘ÏÏÔÁ‹˜ ÙˆÓ Î·Ï‡ÙÂÚˆÓ
Ù¤ÙÔÈˆÓ ËÁÒÓ ÂӉ›ÍÂˆÓ ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È ÛÙÔÓ
¶›Ó·Î· 4 (8).
∂›Û˘, Û ÔÚÈṲ̂ӷ ·ÓÂÈÛÙ‹ÌÈ·, ÙÌ‹Ì·Ù· È·ÙÚÈÎÒÓ Û¯ÔÏÒÓ ¤¯Ô˘Ó ·Û¯ÔÏËı› ÌÂ Û˘Ó‹ıË ÚÔ‚Ï‹Ì·Ù· Ô˘ ··ÓÙÒÓÙ·È ÛÙËÓ Î·ıËÌÂÚÈÓ‹ ÎÏÈÓÈ΋ Ú¿ÍË Î·È, ·ÎÔÏÔ˘ıÒÓÙ·˜ fiÏË ÙË
‰È·‰Èηۛ· πµ∂, ·Ô‰›‰Ô˘Ó ÌÈÎÚ¤˜, ηٷÓÔËÙ¤˜, ‡ÎÔÏ· ÂÊ·ÚÌfiÛÈ̘ ÂÚÈÏ‹„ÂȘ ÙˆÓ Î·Ï‡ÙÂÚˆÓ ÙÚÂ¯Ô˘ÛÒÓ ÂӉ›ÍˆÓ. ∆· ı¤Ì·Ù· ·˘Ù¿, Ô˘ ¤¯Ô˘Ó ‹‰Ë ÎÚÈÙÈο ÚÔ-·ÍÈÔÏÔÁËı›
(Critically Appraised Topics - CATs), ·Ó¢ڛÛÎÔÓÙ·È ÛÙËÓ Â›ÛËÌË ÈÛÙÔÛÂÏ›‰· ÙÔ˘˜ (‚Ϥ ¶›Ó·Î· 4).
ŒÂÈÙ· ·fi ÙËÓ ·ÍÈÔÏfiÁËÛË ÙˆÓ ‰Â‰Ô̤ӈÓ, ÔÈ ËÁ¤˜ Ì ʛÏÙÚÔ ·Ó·Ê¤ÚÔ˘Ó ÛÙÔ Ù¤ÏÔ˜
οÔȘ ˘Ô‰Â›ÍÂȘ (recommendations), ÔÈ
Ôԛ˜ Û˘Óԉ‡ÔÓÙ·È ·fi ·Ú·ÙËÚ‹ÛÂȘ ÁÈ· ÙÔ
Â›Â‰Ô ·ÍÈÔÈÛÙ›·˜ Î·È ÔÈfiÙËÙ·˜ ÙˆÓ ‰Â‰ÔÌ¤ÓˆÓ Ô˘ Û˘Óı¤ÙÔ˘Ó ÙȘ ˘Ô‰Â›ÍÂȘ ·˘Ù¤˜. ∆·
›‰· ·˘Ù¿ ·Ó·Ê¤ÚÔÓÙ·È ˆ˜ ∞, µ, C Î·È D (Ï·ÙÈÓÈο ÁÚ¿ÌÌ·Ù·). °È· ÂÚÈÛÛfiÙÂÚ˜ ÏÂÙÔ̤ÚÂȘ ·Ú·¤ÌÔ˘Ì ÛÙÔÓ ¶›Ó·Î· 5 (9).
∞ÊÔ‡ Û˘ÓÙ¿ÍÔ˘Ì ÙËÓ ÂÚÒÙËÛ‹ Ì·˜, ÔÚ›˙Ô˘Ì ϤÍÂȘ ÎÏÂȉȿ Ô˘ ·ÊÔÚÔ‡Ó ¿ÌÂÛ· ÙÔ
ÎÏÈÓÈÎfi Ì·˜ Úfi‚ÏËÌ·. ∏ οı ËÁ‹ ÏËÚÔÊÔÚÈÒÓ ÛÙÔ ¢È·‰›ÎÙ˘Ô ·Ú¤¯ÂÈ Â›Û˘, ¤Ó· ¢ÚÂÙ‹ÚÈÔ Ï¤ÍÂˆÓ ÁÈ· ÙË ‰È¢ÎfiÏ˘ÓÛË ÙÔ˘ ¯Ú‹ÛÙË, Ô˘ Û˘ÁÎÂÎÚÈ̤ӷ ÛÙÔ MEDLINE ÔÓÔÌ¿˙ÂÙ·È
Medical Subject Headings (MeSH). °È· ·Ú¿‰ÂÈÁÌ· ·Ó ¯ÚËÛÈÌÔÔÈ‹ÛÔ˘Ì ÙËÓ ·ÁÁÏÈ΋ ϤÍË chickenpox (·ÓÂÌ¢ÏÔÁÈ¿), ÛÙÔ MeSH ·Ó·Ê¤ÚÂÙ·È Î·È ˆ˜ Varicella Zoster. ŒÙÛÈ, ¯ÚËÛÈÌÔÔÈÒÓÙ·˜ ÙȘ ‰ÈΤ˜ Ì·˜ ϤÍÂȘ ·Ó·˙‹ÙËÛ˘, ηıÒ˜ Î·È ÙȘ ϤÍÂȘ ÙÔ˘ ¢ÚÂÙËÚ›Ô˘ (MeSH), ‚ÂÏÙÈÒÓÔ˘Ì ÙËÓ Â˘·ÈÛıËÛ›· Ù˘ ¤ÚÂ˘Ó¿˜ Ì·˜ Î·È ÂÍ·ÛÊ·Ï›˙Ô˘Ì ÙËÓ ·Ó¿ÎÙËÛË fiÛÔ ÙÔ ‰˘Ó·ÙfiÓ
ÂÚÈÛÛfiÙÂÚˆÓ Û¯ÂÙÈÎÒÓ ¿ÚıÚˆÓ. ŒÂÈÙ· ı¤ÙÔ˘Ì οÔÈÔ˘˜ ÂÚÈÔÚÈÛÌÔ‡˜ (limits) ÛÙËÓ ¤ÚÂ˘Ó¿ Ì·˜, fiˆ˜ .¯. Ù· ¿ÚıÚ· Ô˘ ı· ·ÓÂ-
¶›Ó·Î·˜ 4. µ¿ÛÂȘ ‰Â‰ÔÌ¤ÓˆÓ (databases)
‚ÚÂıÔ‡Ó Ó· ·ÊÔÚÔ‡Ó Û ·ÓıÚÒÔ˘˜ Î·È fi¯È ˙Ò·, ËÏÈΛ· ÏËı˘ÛÌÔ‡ .¯. ·È‰È·ÙÚÈÎfi˜ ÏËı˘ÛÌfi˜, ÁÏÒÛÛ· ¿ÚıÚÔ˘ .¯. Ó· Â›Ó·È ÌfiÓÔ
∞ÁÁÏÈο, ›‰Ô˜ ¤Ú¢ӷ˜, Ì ‚¿ÛË ·Ó ÙÔ ÎÏÈÓÈÎfi
Ì·˜ ı¤Ì· ·ÊÔÚ¿ ıÂڷ›·, ÚfiÁÓˆÛË Î.Ï., .¯. Meta-analyses of Systematic Reviews, Cohort studies ·ÓÙ›ÛÙÔȯ·, Î.Ô.Î. (10).
ŒÓ·˜ ÔÏ˘¿Û¯ÔÏÔ˜ ÎÏÈÓÈÎfi˜ È·ÙÚfi˜ ‰ÂÓ Â›Ó·È ¿ÓÙÔÙ Û ı¤ÛË Ó· ·ÍÈÔÏÔÁ‹ÛÂÈ ÙËÓ ÂÁ΢ÚfiÙËÙ· ÙˆÓ ÏËÚÔÊÔÚÈÒÓ Ô˘ ·Ó¢ڛÛÎÂÈ, ›ÙÂ
ÏfiÁˆ ¤ÏÏÂȄ˘ ¯ÚfiÓÔ˘ ›Ù ÏfiÁˆ ¤ÏÏÂȄ˘
ÁÓÒÛÂˆÓ ÛÙ·ÙÈÛÙÈ΋˜ ‹ ·ÎfiÌ·, ÏfiÁˆ ÂÚÈÔÚÈṲ̂Ó˘ ÚfiÛ‚·Û˘ ÛÙo ¢È·‰›ÎÙ˘Ô. ∫·Ï‡ÙÂÚÔ, ÏÔÈfiÓ, Â›Ó·È Ó· ÍÂÎÈÓ¿ ÙËÓ ¤Ú¢ӷ ·fi ÙȘ ËÁ¤˜ ÂΛӘ Ô˘ ÚÔÛʤÚÔ˘Ó ˘„ËÏ‹˜ ÔÈfiÙË-
ÏËÚÔÊÔڛ˜, ÙȘ ÚÔ·Ó·ÊÂÚı›Û˜ ‚¿ÛÂȘ ‰Â‰ÔÌ¤ÓˆÓ Ì ʛÏÙÚÔ, fiˆ˜ Cochrane Library, Clinical Evidence, UpToDate, CATs Î.Ï. ∂¿Ó
ÔÈ ËÁ¤˜ ‰ÂÓ ·Ô‰ÒÛÔ˘Ó Î¿ÔȘ ¯Ú‹ÛÈ-
¯ˆÚ›˜ Ê›ÏÙÚÔ, fiˆ˜ .¯. ÙË MEDLINE.
∞ÍÈÔÏfiÁËÛË ‰Â‰ÔÌ¤ÓˆÓ ªÂÙ¿ ÙË Û˘ÏÏÔÁ‹ fiÏˆÓ ÙˆÓ ÂӉ›ÍÂˆÓ Ì¤Ûˆ ÙˆÓ ËÁÒÓ Ô˘ ÚԷӷʤÚıËηÓ, ÙÔ ÂfiÌÂÓÔ ‚‹Ì· Â›Ó·È Ë ÎÚÈÙÈ΋ ·ÍÈÔÏfiÁËÛË (critical appraisal) Ù˘ ·ÍÈÔÈÛÙ›·˜ ÙˆÓ ÏËÚÔÊÔÚÈÒÓ ·˘ÙÒÓ, ÒÛÙ ӷ ·Ô‰Âȯı› Ë ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Î·È Ë Â¿ÚÎÂÈ¿ ÙÔ˘˜ ÛÙËÓ ·Ó·˙‹ÙËÛË ÙÚfiˆÓ ‚ÂÏÙ›ˆÛ˘ Ù˘ ÊÚÔÓÙ›‰·˜ ÙÔ˘ ·ÛıÂÓÔ‡˜.
™ËÌ·ÓÙÈÎfi Â›Ó·È ÛÙÔ Â›Â‰Ô ·˘Ùfi Ó· Á›ÓÂÈ Î·Ù·ÓÔËÙ‹ Ë ‰È·ÊÔÚ¿ ÌÂٷ͇ ÎÚÈÙÈ΋˜ ·ÍÈÔÏfiÁË-
Û˘ Ì›·˜ ÎÏÈÓÈ΋˜ ÌÂϤÙ˘ Î·È ·Ï¿ ·Ó¿ÁÓˆÛ‹˜
Ù˘, ·ÊÔ‡ ÔÔÈÔÛ‰‹ÔÙ ÌÔÚ› Ó· ‰ËÌÔÛȇÛÂÈ ÔÙȉ‹ÔÙ ÛÙÔ ¢È·‰›ÎÙ˘Ô. ŒÙÛÈ ·ÍÈÔÏÔÁÔ‡ÌÂ:
µ¿ÛÂȘ ‰Â‰Ô̤ӈÓπÛÙÔÛÂÏ›‰·
The Cochrane Collaborationhttp://www.thecochranelibrary.com
Database of Abstracts of Reviews of Effects http://www.york.ac.uk/inst/crd/ Clinical Evidencehttp://www.clinicalevidence.org
TRIP (Turning Research into Practice)http://www.tripdatabase.com
UpToDatehttp://www.uptodate.com
ACP Journal Clubhttp://acpjc.org
Evidence-based Medicine Reviews (OVID)http://www.ovid.com
MEDLINE (PubMed)http://www.ncbi.nlm.nih.gov/PubMed/
CATs - University of Michigan, USAhttp://www.med.umich.edu/pediatrics/ebm/
CATs - University of North Carolina, USAhttp://www.med.unc.edu/medicine/edusrc/
CATs - University of Manchester, UKhttp://www.bestbets.org
¶›Ó·Î·˜ 5. ∂›Â‰Ô ÂӉ›ÍÂˆÓ (levels of evidence) ηÈ
(recommendation grades)
∂›Â‰Ô (level) ∂Ӊ›ÍÂȘ∂ÚÌËÓ›· Û˘ÛÙ¿ÛˆÓ
ÌÂÙ·-·Ó¿Ï˘ÛË (ª∞)À„ËÏ‹˜ ÔÈfiÙËÙ·˜ ÂӉ›ÍÂȘ
∆∂ª ‹ ¿ÓÙ· ·Ô‰ÂÎÙ¤˜ Û˘ÛÙ¿ÛÂȘ, Ù˘¯·ÈÔÔÈË̤ÓË ÂÏÂÁ¯fiÌÂÓË
(∆∂ª) ÌÂ
·Ô‰Â‰ÂÈÁ̤ӷ ·ÛÊ·Ï›˜, ÌÈÎÚfi ΛӉ˘ÓÔ ÛÊ¿ÏÌ·ÙÔ˜ Ì ·ÔÙÂϤÛÌ·Ù· ¿ÌÂÛ· ·Ó·ÌÊ›‚ÔÏ· ¯Ú‹ÛÈ̘
ÂÊ·ÚÌfiÛÈÌ· ÛÙÔÓ ÏËı˘ÛÌfi-ÛÙfi¯Ô Î·È ·Ú·Ï‹ÛÈ·
ÌÂٷ͇ ÙˆÓ ÎÏÈÓÈÎÒÓ ÌÂÏÂÙÒÓ.
B À„ËÏ‹˜ ÔÈfiÙËÙ·˜ ÌÂϤÙ˜ ·ÛıÂÓÒÓ-Ì·ÚÙ‡ÚˆÓŸ¯È ˘„ËÏ‹˜ ÔÈfiÙËÙ·˜ ÂӉ›ÍÂȘ(case-control studies) ‹ ‰È·¯ÚÔÓÈΤ˜ (cohort studies) ‹ ·Ô‰ÂÎÙ¤˜ Û˘ÛÙ¿ÛÂȘ, ·ÛÊ·Ï›˜, ™∞ ·˘ÙÒÓ, Ì ÌÈÎÚfi ΛӉ˘ÓÔ ‡·Ú͢ Û˘Á¯˘ÙÈÎÒÓ ¯Ú‹ÛÈ̘ (¯Ú‹ÛË ÙˆÓ Ì¤¯ÚÈ ÙÒÚ· ·Ú·ÁfiÓÙˆÓ, Ù˘¯·›Ô˘ ‹ ÛÊ¿ÏÌ·ÙÔ˜, Ì ÌÂÁ¿ÏË Û˘ÛÙ¿ÛˆÓ/Ô‰ËÁÈÒÓ ‹ ¯Ú‹ÛË ÙˆÓ
Èı·ÓfiÙËÙ·
‰È·¯ÚÔÓÈÎÒÓ ÌÂÏÂÙÒÓ ‹ ˘„ËÏ‹˜ ÔÈfiÙËÙ·˜ ÌÂÏÂÙÒÓ
‹ ‰È·¯ÚÔÓÈÎÒÓ ÌÂÏÂÙÒÓ Ì ÌÈÎÚfi
ΛӉ˘ÓÔ ‡·Ú͢ Û˘Á¯˘ÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ, Ù˘¯·›Ô˘ ‹
ÛÊ¿ÏÌ·ÙÔ˜ Î·È Ì ÌÂÁ¿ÏË Èı·ÓfiÙËÙ· Ë Û¯¤ÛË Ô˘
ÌÂÏÂÙ¿Ù·È Ó· Â›Ó·È ·ÈÙÈÔÏÔÁÈ΋.
D ªË-·Ó·Ï˘ÙÈΤ˜ ÌÂϤÙ˜ .¯. ·ÚÔ˘ÛÈ¿ÛÂȘ ÂÚÈÛÙ·ÙÈÎÒÓ ªË ·Ô‰ÂÎÙ¤˜ Û˘ÛÙ¿ÛÂȘ, ÌË (case reports), ÛÂÈÚ¿ ÂÚÈÛÙ·ÙÈÎÒÓ (case series) ‹ ÁÓÒ̘ ÙÂÎÌËÚȈ̤ÓÔ fiÊÂÏÔ˜, Èı·ÓfiÓ Î·È
ÂȉÈÎÒÓ ‹ Û˘ÁÎÂÓÙÚˆÙÈο ÛÙÔȯ›· ηϿ ۯ‰ȷÛÌ¤ÓˆÓ ‚Ï·‚ÂÚ¤˜ Û˘Ó¤ÂȘ.
ÌÂÏÂÙÒÓ ·ÛıÂÓÒÓ-Ì·ÚÙ‡ÚˆÓ ‹ ‰È·¯ÚÔÓÈÎÒÓ ÌÂÏÂÙÒÓ ÌÂ
ÌÈÎÚfi ΛӉ˘ÓÔ ‡·Ú͢ ·Ú·ÁfiÓÙˆÓ Û‡Á¯˘Û˘, Ù˘¯·›Ô˘ ‹
ÛÊ¿ÏÌ·ÙÔ˜ Î·È Ì ̤ÙÚÈ· Èı·ÓfiÙËÙ· Ë Û¯¤ÛË Ô˘
ÌÂÏÂÙ¿Ù·È Ó· Â›Ó·È ·ÈÙÈÔÏÔÁÈ΋.
ÌÂϤÙ˘
Ô˘ Ú·ÁÌ·Ù‡ÂÙ·È ÙË ıÂڷ›· ÌÈ·˜ ÓfiÛÔ˘ ‰›-
‰ÂÙ·È ÛÙÔÓ ¶›Ó·Î· 6 (11).
ii) ÙË ¯ÚËÛÈÌfiÙËÙ¿ ÙÔ˘˜ (usefulness), ηٿ
fiÛÔÓ ‰ËÏ·‰‹, Ù· Â˘Ú‹Ì·Ù· Ù˘ ¤Ú¢ӷ˜ ›ӷÈ
∂Ê·ÚÌÔÁ‹ ÂӉ›ÍˆÓ
ÂÊ·ÚÌÔÁ‹ ÙˆÓ ÂӉ›ÍˆÓ, Ô˘ ¤¯Ô˘ÌÂ Û˘ÏϤÍÂÈ ÌÂÙ¿ ·fi ÌÂıÔ‰È΋ ¤Ú¢ӷ Î·È ·ÓÙÈÎÂÈÌÂÓÈ΋ ·ÍÈÔÏfiÁËÛË, ·ÔÙÂÏ› ÙËÓ ÎÂÓÙÚÈ΋
Based Medicine - apply conclusions),
ÛËÌ·ÓÙÈο (important), ¯Ú‹ÛÈÌ· (useful), ÂÊ·ÚÌfiÛÈÌ· (applicable) ÛÙË ÎÏÈÓÈ΋ Ú¿ÍË Î·È Û¯ÂÙÈο (relevant) Ì ÙÔ ·Ú¯ÈÎfi ÎÏÈÓÈÎfi ÂÚÒÙËÌ· (‚Ϥ ¶›Ó·Î· 6).
1) ∂›Ó·È ¤Á΢ڷ Ù· ·ÔÙÂϤÛÌ·Ù· Ù˘ ÌÂϤÙ˘; ➤ •ÂΛÓËÛ·Ó ÔÈ ÔÌ¿‰Â˜ ÌÂϤÙ˘ Î·È Û‡ÁÎÚÈÛ˘ Ì ÙȘ ›‰È˜ Èı·ÓfiÙËÙ˜ ÚfiÁÓˆÛ˘; ñ ◊Ù·Ó ÔÈ ·ÛıÂÓ›˜ Ù˘¯·ÈÔÔÈË̤ÓÔÈ;
ñ ◊Ù·Ó Ë Ù˘¯·ÈÔÔ›ËÛË Ù˘ÊÏ‹;
ñ ªÂÏÂÙ‹ıËÎ·Ó ÔÈ ·ÛıÂÓ›˜ ÛÙȘ ·Ú¯ÈΤ˜ ÔÌ¿‰Â˜ (ÌÂϤÙ˘ ‹ Û‡ÁÎÚÈÛ˘) Ô˘ ›¯·Ó Ù˘¯·ÈÔÔÈËı›;
(ÌÂÙ·‹‰ËÛË ·ÛıÂÓÒÓ)
ñ ◊Ù·Ó Ù· ¯·Ú·ÎÙËÚÈÛÙÈο ÙˆÓ ·ÛıÂÓÒÓ ÛÙȘ ‰‡Ô ÔÌ¿‰Â˜ ·ÚÂÌÊÂÚ‹ fiÛÔÓ ·ÊÔÚ¿ ÁÓˆÛÙÔ‡˜
ÚÔÁÓˆÛÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜; ➤
¢È·Ù‹ÚËÛ·Ó ÔÈ ÔÌ¿‰Â˜ ÌÂϤÙ˘ Î·È ÂϤÁ¯Ô˘ ·ÚfiÌÔÈ· ÚfiÁÓˆÛË ÌÂÙ¿ ÙËÓ ¤Ó·ÚÍË Ù˘ ÎÏÈÓÈ΋˜ ÌÂϤÙ˘; ñ °ÓÒÚÈ˙·Ó ÔÈ ·ÛıÂÓ›˜ Û ÔÈ· ÔÌ¿‰· ·Ó‹ÎÔ˘Ó; ñ °ÓÒÚÈ˙·Ó ÔÈ ÎÏÈÓÈÎÔ› ÂÚ¢ÓËÙ¤˜ ÙËÓ Î·Ù·ÓÔÌ‹ ÙˆÓ ·ÛıÂÓÒÓ;
ñ °ÓÒÚÈ˙·Ó ÔÈ ÎÏÈÓÈÎÔ› ·ÔÙÈÌËÙ¤˜ ÙÔ˘ ·ÔÙÂϤÛÌ·ÙÔ˜ Ù˘ ¤Ú¢ӷ˜ ÙËÓ Î·Ù·ÓÔÌ‹ ÙˆÓ ·ÛıÂÓÒÓ; ñ √ÏÔÎÏËÚÒıËÎÂ Ë ·Ú·ÎÔÏÔ‡ıËÛË (follow-up) ÙˆÓ ·ÛıÂÓÒÓ;
2) ¶ÔÈ· ‹Ù·Ó Ù· ·ÔÙÂϤÛÌ·Ù·; ➤ ¶fiÛÔ ÌÂÁ¿ÏÔ ‹Ù·Ó ÙÔ ıÂڷ¢ÙÈÎfi ·ÔÙ¤ÏÂÛÌ·; (treatment effect) ➤ ¶fiÛÔ ·ÎÚÈ‚‹˜ ‹Ù·Ó Ë ÂÎÙ›ÌËÛË ÙÔ˘ ıÂڷ¢ÙÈÎÔ‡ ·ÔÙÂϤÛÌ·ÙÔ˜; (confidence intervals)
3) ¶Ò˜ ÌÔÚÒ Ó· ÂÊ·ÚÌfiÛˆ Ù· ·ÔÙÂϤÛÌ·Ù· ÛÙÔÓ ‰ÈÎfi ÌÔ˘ ·ÛıÂÓ‹; ➤
Ô˘ ‚·Û›˙ÔÓÙ·È ÛÙ· Â˘Ú‹Ì·Ù· Ù˘ ÙÚ¤¯Ô˘Û·˜ ¤Ú¢ӷ˜. ∆· ÂÚˆÙ‹Ì·Ù· Ô˘ ·Ó·‰‡ÔÓÙ·È ÛÙÔ
ÛËÌÂ›Ô ·˘Ùfi ÁÈ· ÙÔÓ ÎÏÈÓÈÎfi È·ÙÚfi Â›Ó·È ÔÏÏ¿:
·fi fiÏ· ·˘Ù¿ ÌÔÚ› Ó· ÂÊ·ÚÌÔÛÙ› ÛÙËÓ
Ú¿ÍË; ¶fiÛÔ ·ÓÙÈÎÚÔ‡ÔÓÙ·È Ì ٷ fiÛ· ÁÓÒÚÈ˙Â
Î·È ¯ÚËÛÈÌÔÔÈÔ‡Û ̤¯ÚÈ Û‹ÌÂÚ·; ∫·Ù¿ fiÛÔÓ
Ù· ·ÔÙÂϤÛÌ·Ù· ÙˆÓ ÂÚ¢ÓÒÓ Ô˘ ·ÊÔÚÔ‡Ó ÙË
̤ÛË ¤Î‚·ÛË ÌÈ·˜ ÔÌ¿‰·˜ ·ÙfiÌˆÓ ÌÔÚÔ‡Ó Ó·
ÂÓۈ̷وıÔ‡Ó ÛÙȘ ·Ó¿ÁΘ, ÛÙȘ ÚÔÛ‰Ô˘
Î·È ÛÙȘ ÚÔÙÈÌ‹ÛÂȘ ÂÓfi˜ ·ÛıÂÓÔ‡˜; ¢ÂÓ Â›Ó·È Û¿ÓÈÔ, ¤Ó·˜ ÎÏÈÓÈÎfi˜ È·ÙÚfi˜, ÌÂÙ¿ ·fi ÌÈ· ÂÍ·ÓÙÏËÙÈ΋ ¤Ú¢ӷ Ù˘ ‚È‚ÏÈÔÁÚ·Ê›·˜, Ó· ηٷϋÍÂÈ ÛÂ Û˘ÌÂÚ¿ÛÌ·Ù· Î·È ·ÔÊ¿ÛÂȘ Ô˘, ‰˘ÓËÙÈο, ı· ÌÔÚÔ‡Û ӷ Ï¿‚ÂÈ Ì ÙȘ ¤ˆ˜ ÂΛÓË ÙË ¯ÚÔÓÈ΋ ÛÙÈÁÌ‹ ÁÓÒÛÂȘ
ÙÔ˘. ∏ Ú·ÎÙÈ΋ πµ∂ ÙÔ˘ ‰›ÓÂÈ ÙËÓ Â˘Î·ÈÚ›· Ó· ‰È·ÛÙ·˘ÚÒÛÂÈ ÙȘ ÁÓÒÛÂȘ, ÙȘ ÂÌÂÈڛ˜ -ηÈ
ÁÈ·Ù› fi¯È- ÙËÓ ÚˆÙ·Ú¯È΋ ‰È·›ÛıËÛ‹ ÙÔ˘ ÁÈ· ÙË
Ï‹„Ë Ù˘ ÛˆÛÙfiÙÂÚ˘ ·fiÊ·Û˘. ∂ÈϤÔÓ, Ë
ÙȘ ÁÓÒÛÂȘ ÂÓfi˜ ÂȉÈÎÔ‡ ÎÏÈÓÈÎÔ‡ È·ÙÚÔ‡. √ ¤ÌÂÈÚÔ˜ ÎÏÈÓÈÎfi˜ Â›Ó·È ·˘Ùfi˜ Ô˘ ı· ·ÔÊ·-
Ò˜ ı· ¯ÚËÛÈÌÔÔÈ‹ÛÂÈ ·˘Ù¤˜ ÙȘ Ӥ˜
ÁÓÒÛÂȘ Î·È Ò˜ ı· ÙȘ “·ÓÙÚ¤„ÂÈ” Ì ÙȘ
·Í›Â˜ Î·È È‰È·ÈÙÂÚfiÙËÙ˜ ÙÔ˘ οı ·ÛıÂÓÔ‡˜ Î·È ÛÂ Û˘ÓÂÚÁ·Û›· Ì·˙› ÙÔ˘ Ó· ÏËÊı› Ë Î·Ï‡-
ÙÂÚË ÎÏÈÓÈ΋ ·fiÊ·ÛË (2).
™˘ÌÂÚ¿ÛÌ·Ù·
∏ (¶·È‰)π·ÙÚÈ΋ µ·ÛÈṲ̂ÓË ÛÙȘ ∂Ӊ›ÍÂȘ (Evidence Based Medicine/ Evidence Based Pediatrics) ‰ÂÓ ‰È‰¿ÛÎÂÈ Î·ÓfiÓ˜ ‰Ú¿Û˘ Û οÔÈÔ ÎÏÈÓÈÎfi Úfi‚ÏËÌ·, ·ÏÏ¿ ·ÔÙÂÏ› ÂÚÁ·ÏÂ›Ô ÎÚÈÙÈ΋˜ Î·È ‰ÔÌË̤Ó˘ ÛΤ„˘, ·ÊÔ‡ Ë
Û˘ÏÏÔÁ‹ ‰Â‰ÔÌ¤ÓˆÓ ·fi ÙËÓ ÙÚ¤¯Ô˘Û· ‚È‚ÏÈÔ-
Ó¤ˆÓ Î·È ÂÎÛ˘Á¯ÚÔÓÈÛÌ¤ÓˆÓ ÁÓÒÛˆÓ, ·ÏÏ¿ Ë
ÔÚıÔÏÔÁÈ΋ ¯ÚËÛÈÌÔÔ›ËÛ‹ ÙÔ˘˜ ÒÛÙ ӷ ÚÔ-
ÛʤÚÂÈ Â˘ÓÔïÎfiÙÂÚ˜ ÚÔ¸Ôı¤ÛÂȘ ÁÈ· ÙËÓ Î·-
χÙÂÚË ÔÚ›· ÙˆÓ ·ÛıÂÓÒÓ ÙÔ˘ (12).
µÈ‚ÏÈÔÁÚ·Ê›·
1.ªÔ˘ÓÙÔηϿ΢ £.¢. π·ÙÚÈ΋ ‚·ÛÈ˙fiÌÂÓË Û ÂӉ›ÍÂȘ ÌÈ· ÚÒÙË Ì·ÙÈ¿. Evidence Based Medicine. π·ÙÚÈ΋ ™¯ÔÏ‹ ¶·ÓÂÈÛÙËÌ›Ô˘ ∫Ú‹Ù˘- °·ÛÙÚÂÓÙÂÚÔÏÔÁÈ΋ ∫ÏÈÓÈ΋. 2001.
2.Sackett DL, Rosenberg WM, Gray JA, Haynes RB, Richardson WS. Evidence Based Medicine: what it is and what it isn’t. BMJ 1996;312:71-72.
3.Elphick HE, Smyth RL. Research: the principles of evidence-based medicine. Current Paediatrics 2004; 14:525-531.
4.Onady G, Raslich MA. Evidence-based medicine for the pediatrician. Pediatr Rev 2002;23:318-322.
5. Focusing clinical questions. [Internet]. Oxford: Centre for Evidence Based Medicine. 2002. Webpage:
http://cebm.jr2.ox.ac.uk/docs/focusquest.html
6.Glasziou PP, Del Mar CB, Sanders SL, Hayem M. Antibiotics for acute otitis media in children [Cochrane Review]. The Cochrane Library, Issue 4. Oxford: Update software, 2001.
7.Greenhalgh T. How to read a paper: The Medline database. BMJ 1997;315:180-183.
8.Onady GM, Raslich MA. Evidence-based medicine: searching literature and databases for clinical evidence (search tools). Pediatrics in Review 2004; 25:358-363.
9.Jones LV, Smyth RL. How to perform a literature search. Current Paediatrics 2004;14:482-488.
10. Baumer JH. Comparison of two otitis media guidelines. Archives of Disease in Childhood Education and Practice Edition 2004;89:76-78.
11.Guyatt G, Cook D, Devereaux PJ, Meade M, Straus S. Therapy. In: Guyatt G, Rennie D. Users’ Guides to the medical literature.1st ed. USA: American Medical Association; 2002.
12. Bauchner H. Evidence-based Medicine: A new science or an epidemiologic fad? Pediatrics 1999; 103:1029-1031.
°ÔÓ›‰È· Î·È Ì˯·ÓÈÛÌÔ› ÛÙËÓ ·Ó¿Ù˘ÍË
ÙˆÓ ÓÂÊÚÒÓ
¶.¶. ¶¤ÙÚÔ˘
¶ÂÚ›ÏË„Ë: √ ·Ó·Ù˘ÛÛfiÌÂÓÔ˜ ÓÂÊÚfi˜ ¯ÚËÛÈÌÔÔÈÂ›Ù·È Â˘Ú¤ˆ˜ ˆ˜ ÂÈÚ·Ì·ÙÈÎfi Û‡ÛÙËÌ· ÁÈ· ÙË
‰ÈÂÚ‡ÓËÛË ‚·ÛÈÎÒÓ Ì˯·ÓÈÛÌÒÓ ÔÚÁ·ÓÔÁ¤ÓÂÛ˘. ∏ ÌÔÚÊÔÁ¤ÓÂÛË ÙÔ˘ ÌÂÙ¿ÓÂÊÚÔ˘, ÙÔ˘ ÌfiÓÈÌÔ˘ ÓÂÊÚÔ‡ ÛÙÔÓ ÂÓ‹ÏÈη, ÂÚÈÏ·Ì‚¿ÓÂÈ ‰È·‰Èηۛ˜ fiˆ˜ ÂÈıËÏȷΤ˜-ÌÂÛÂÁ¯˘Ì·ÙÈΤ˜ ·ÏÏËÏÂȉڿÛÂȘ, ‰È·ÎÏ¿‰ˆÛË ÂÈıËÏ›ˆÓ, ÂÈıËÏÈÔÔ›ËÛË ÌÂÛÂÁ¯˘Ì·ÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ Î·È Î˘ÙÙ·ÚÈ΋ ‰È·ÊÔÚÔÔ›ËÛË. √È ·Ú·¿Óˆ ‰ÈÂÚÁ·Û›Â˜ Û˘ÓÙÔÓ›˙ÔÓÙ·È ·fi ÌÈ· ÛÂÈÚ¿ ·ÌÊ›‰ÚÔÌˆÓ ÛËÌ·ÙÔ‰ÔÙ‹ÛÂˆÓ ÌÂٷ͇ ‰‡Ô ÂÌ‚Ú˘˚ÎÒÓ ÈÛÙÒÓ Ô˘ ÚÔ·ÙÔ˘Ó ·fi ÙÔ ‰È¿ÌÂÛÔ ÌÂÛfi‰ÂÚÌ·, Ù˘ Ô˘ÚËÙËÚÈ΋˜ ηٷ-
‚ÔÏ‹˜ Î·È ÙÔ˘ ÌÂÙ·ÓÂÊÚÈÎÔ‡ ÌÂÛÂÁ¯‡Ì·ÙÔ˜ ηıÒ˜ ›Û˘ Î·È ÙˆÓ ·Ú·ÁÒÁˆÓ ÙÔ˘˜. ∆· ÙÂÏÂ˘Ù·›·
¯ÚfiÓÈ· Ë ·ÍÈÔÔ›ËÛË ÙÔ˘ ÔÓÙÈÎÔ‡ ˆ˜ ÂÈÚ·Ì·ÙÈÎfi ÌÔÓÙ¤ÏÔ Â›Ù ÁÈ· ÙËÓ ·ÂÓÂÚÁÔÔ›ËÛË ÁÔÓȉ›ˆÓ, ›Ù ÁÈ· ÙË ‰ËÌÈÔ˘ÚÁ›· ‰È·ÁÔÓȉȷÎÒÓ ˙ÒˆÓ, ¤¯ÂÈ
ÌÔÚÈ·ÎÒÓ Ì˯·ÓÈÛÌÒÓ Î·È ÛÙËÓ Ù·˘ÙÔÔ›ËÛË ÁÔÓȉȷÎÒÓ ÚÔ˚fiÓÙˆÓ Ô˘ ÂÌϤÎÔÓÙ·È ÛÙËÓ ·Ó¿-
Ù˘ÍË ÙˆÓ ÓÂÊÚÒÓ. ¶·ÚfiÏË ÙËÓ ¤ÓÙÔÓË ÂÚ¢ÓËÙÈ΋ ‰Ú·ÛÙËÚÈfiÙËÙ·
ηıÒ˜
ÛÙËÓ ¤ÎÙ·ÛË ÙÔ˘ ÎÂÈ̤ÓÔ˘, ·Ó·Ê¤ÚÔÓÙ·È ÌfiÓÔ ÔÈ Î˘ÚÈfiÙÂÚÔÈ
Genes and mechanisms in kidney development
P.P. Petrou
ÂÓÒ
¶·ÓÂÈÛÙ‹ÌÈÔ
∫Ú‹Ù˘,
µÈÔÏÔÁ›·˜ 71 409 ∏Ú¿ÎÏÂÈÔ, ∫Ú‹ÙË E-mail: petrou@imbb.forth.gr
∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 20-09-2005
∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 02-12-2005
Abstract: The developing kidney is widely used as an experimental system for the study of fundamental mechanisms of organogenesis. Morphogenesis of the metanephros, the permanent kidney in the adult, includes processes such as epithelial-mesenchymal interactions, epithelial branching, mesenchymal-to-epithelial transition and cell differentiation. These processes are coordinated by a series of reciprocal signaling events between two embryonic tissues derived from the intermediate mesoderm, the ureteric bud and the metanephric mesenchyme, and their descendants. The use of the mouse as an experimental model, either for the inactivation of genes or for the generation of transgenic animals, has greatly contributed in the characterization of molecular mechanisms and the identification of gene products involved in kidney development. However, despite the intensive experimental activity in the field of kidney morphogenesis many questions remain to be answered. Recently developed powerful techniques of genetic manipulation in the mouse are expected to provide insight into some of the so far unknown aspects of kidney development. This review reports on the main stages of kidney development and the most important genes involved in each stage.
Key words: Kidney, genes, mouse, ureteric bud, metanehric mesenchyme.
University of Crete, Department of Biology
Correspondence: P.P. Petrou University of Crete, Department of Biology 71 409, Herakleion, Crete E-mail: petrou@imbb.forth.gr
Date of submission: 20-09-2005
Date of approval: 02-12-2005
·Ú¿ÁˆÁ· ÙÔ˘ ‰È¿ÌÂÛÔ˘ ÌÂÛÔ‰¤ÚÌ·ÙÔ˜ ηÈ
‰È·‰¤¯ÔÓÙ·È ÙÔ ¤Ó· ÙÔ ¿ÏÏÔ. ∫ÂÊ·ÏÈÎfiÙÂÚ·
Û¯ËÌ·Ù›˙ÔÓÙ·È ÔÈ ÚfiÓÂÊÚÔÈ, ÔÈ ÔÔ›ÔÈ Â›Ó·È ÌË
ÏÂÈÙÔ˘ÚÁÈÎÔ› Î·È ·ÔÙÂÏÔ‡Ó ÙÔÓ ÈÔ ÚˆÙfiÁÔ-
ÓÔ Ù‡Ô ÓÂÊÚÒÓ ÙˆÓ ÛÔÓ‰˘ÏˆÙÒÓ (∂ÈÎfiÓ·
1∞). ∞˘ÙÔ› ÂÌÊ·Ó›˙ÔÓÙ·È Î·Ù¿ ÙËÓ 3Ë Â‚‰ÔÌ¿-
‰· Ù˘ ·ËÛ˘ Î·È ÂÎÊ˘Ï›˙ÔÓÙ·È Û‡ÓÙÔÌ· ÁÈ·
Ó· ÙÔ˘˜ ‰È·‰Â¯ıÔ‡Ó ÔÈ ÌÂÛfiÓÂÊÚÔÈ, ÔÈ ÔÔ›ÔÈ
·Ó·Ù‡ÛÛÔÓÙ·È Ô˘Ú·ÈfiÙÂÚ· ÛÙÔ Â›Â‰Ô ÙÔ˘
3Ô˘ ÔÛÊ˘˚ÎÔ‡ ÛÔÓ‰‡ÏÔ˘ Î·È Ë ·Ó¿Ù˘ÍË ÙÔ˘˜
ÔÏÔÎÏËÚÒÓÂÙ·È Î·Ù¿ ÙËÓ 8Ë Â‚‰ÔÌ¿‰· Ù˘
·ËÛ˘ (∂ÈÎfiÓ· 1µ). √È ÌÂÛfiÓÂÊÚÔÈ ·ÔÙÂÏÔ‡Ó
Ù· ·ÚÈ· ·ÂÎÎÚÈÙÈο fiÚÁ·Ó· ηٿ ÙËÓ ÂÌ‚Ú˘-
˚΋ ·Ó¿Ù˘ÍË. Ÿˆ˜ Î·È ÔÈ ÚfiÓÂÊÚÔÈ, ÔÈ ÌÂ-
ÛfiÓÂÊÚÔÈ ÂÍ·Ê·Ó›˙ÔÓÙ·È ÛÙ·‰È·Î¿ Î·È ‰ÂÓ ˘¿Ú¯Ô˘Ó ÛÙÔÓ ÂÓ‹ÏÈη, Ì ÂÍ·›ÚÂÛË Û˘ÁÎÂÎÚÈ̤ӷ ÌfiÓÔ ÙÌ‹Ì·Ù· ÙˆÓ ÌÂÛÔÓÂÊÚÈÎÒÓ ÛˆÏËÓ·Ú›ˆÓ, Ù· ÔÔ›· ÂÓۈ̷ÙÒÓÔÓÙ·È ÛÙÔ ·Ó··Ú·ÁˆÁÈÎfi Û‡ÛÙËÌ· ÛÙÔ ¿ÚÚÂÓ. ∆¤ÏÔ˜, ÔÈ ÌÂÙ¿ÓÂÊÚÔÈ ·Ú¯›˙Ô˘Ó Ó· ·Ó·Ù‡ÛÛÔÓÙ·È
›‰Ë ÓÂÊÚÒÓ Î·È ˆÚÈÌ¿˙Ô˘Ó
ÏÂÈÙÔ˘ÚÁÈο ÚÔ˜ ÙÔ Ù¤ÏÔ˜ Ù˘ ÂÌ‚Ú˘˚΋˜ ·Ó¿-
Ù˘Í˘, ηıÒ˜ ÔÈ ÌÂÛfiÓÂÊÚÔÈ ÂÎÊ˘Ï›˙ÔÓÙ·È. √È
ÌÂÙ¿ÓÂÊÚÔÈ, ÛÙÔ˘˜ ÔÔ›Ô˘˜ ı· ·Ó·ÊÂÚıÔ‡ÌÂ
ÂÎÙÂÓ¤ÛÙÂÚ· ·Ú·Î¿Ùˆ, Â›Ó·È ÔÈ ÌfiÓÈÌÔÈ ÏÂÈ-
ÙÔ˘ÚÁÈÎÔ› ÓÂÊÚÔ› ÛÙÔÓ ÂÓ‹ÏÈη.
ªÔÚÊÔÁ¤ÓÂÛË ÙˆÓ ÌÂÙ¿ÓÂÊÚˆÓ
∏ ·Ó¿Ù˘ÍË ÙˆÓ ÌÂÙ¿ÓÂÊÚˆÓ ·Ú¯›˙ÂÈ ÌÂ
ÙËÓ ÂÌÊ¿ÓÈÛË Ù˘ Ô˘ÚËÙËÚÈ΋˜ ηٷ‚ÔÏ‹˜ (O∫), ÂÓfi˜ ÂÎÎÔÏÒÌ·ÙÔ˜ ÛÙÔ ÂÈı‹ÏÈÔ ÙÔ˘ ÌÂÛÔÓÂÊÚÈÎÔ‡ fiÚÔ˘ (1). ∞˘Ùfi ÂÈÛ‚¿ÏÏÂÈ ÛÂ
ÌÈ· ÂÍÂȉÈÎÂ˘Ì¤ÓË ÂÚÈÔ¯‹ ÙÔ˘ ‰È¿ÌÂÛÔ˘ ÌÂÛÔ‰¤ÚÌ·ÙÔ˜, ÙÔ ÔÔ›Ô ÔÓÔÌ¿˙ÂÙ·È ÌÂÙ·ÓÂÊÚÈÎfi
ÌÂÛ¤Á¯˘Ì· (∂ÈÎfiÓ˜ 1°, 2∞). ™˘ÓÂÒ˜, Ë ·Ó¿-
Ù˘ÍË ÙˆÓ ÌÂÙ¿ÓÂÊÚˆÓ ÂÍ·ÚÙ¿Ù·È ·fi ÙËÓ ·ÏÏËÏ›‰Ú·ÛË Î·È ÙË Û˘Ì‚ÔÏ‹ ‰‡Ô ÂÌ‚Ú˘˚ÎÒÓ
ÈÛÙÒÓ, Ù˘ √∫ ·fi ÙËÓ ÔÔ›· ÚÔ·ÙÂÈ Ô Ô˘ÚËÙ‹Ú·˜, Ë ÓÂÊÚÈ΋ ‡ÂÏÔ˜ Î·È Ù· ·ıÚÔÈÛÙÈο
¶ÚfiÓÂÊÚÔ˜
¡ÂÊÚÈÎfi˜ ¶fiÚÔ˜
¢È¿ÌÂÛÔ ªÂÛfi‰ÂÌ·
∞Ì¿Ú·
¶ÚfiÓÂÊÚÔ˜
°ÔÓ¿‰·
ªÂÛfiÓÂÊÚÔ˜
√˘ÚËÙËÚÈ΋ ∫·Ù·‚ÔÏ‹
ªÂÙ·ÓÂÊÚÈÎfi ªÂÛ¤Á¯˘Ì·
ªÂÛÔÓÂÊÚÈÎfi˜ ¶fiÚÔ˜
∂ÈÎfiÓ· 1. ™¯ËÌ·ÙÈ΋ ·Ó··Ú¿ÛÙ·ÛË ÙˆÓ ÙÚÈÒÓ Û˘ÛÙËÌ¿ÙˆÓ ÓÂÊÚÒÓ ÛÂ
ÓÂÛ˘. ∞) ™¯ËÌ·ÙÈÛÌfi˜ ÙˆÓ ÚÔÓÂÊÚÈÎÒÓ ÛˆÏËÓ·Ú›ˆÓ ηıÒ˜ Ô ÚÒÈÌÔ˜ ÓÂÊÚÈÎfi˜ fiÚÔ˜ ÂÂÎÙ›ÓÂÙ·È ÚÔ˜ ÙËÓ ·Ì¿Ú·. µ) √ ÌÂÛfiÓÂÊÚÔ˜ ‰ËÌÈÔ˘ÚÁÂ›Ù·È Î·ıÒ˜ Ô ÚfiÓÂÊÚÔ˜ ÂÎÊ˘Ï›˙ÂÙ·È. °) ∏ ·Ó¿Ù˘ÍË ÙÔ˘ ÌÂÙ¿ÓÂÊÚÔ˘ Û˘ÓÙÔÓ›˙ÂÙ·È ·fi ÙËÓ ·ÏÏËÏ›‰Ú·ÛË ‰‡Ô ÂÌ‚Ú˘˚ÎÒÓ ÈÛÙÒÓ, Ù˘ Ô˘ÚËÙËÚÈ΋˜ ηٷ‚ÔÏ‹˜, Ë ÔÔ›· ÂÎʇÂÙ·È ·fi ÙÔ ÂÈı‹ÏÈÔ ÙÔ˘ ÌÂÛÔÓÂÊÚÈÎÔ‡ fiÚÔ˘ Î·È ÙÔ˘ ÌÂÙ·ÓÂÊÚÈÎÔ‡ ÌÂÛÂÁ¯‡Ì·ÙÔ˜. √ ÌÂÛfiÓÂÊÚÔ˜ ÛÙ·‰È·Î¿ ÂÍ·Ê·Ó›˙ÂÙ·È. ∞µ°
ÁfiÙÂÚ· ı· ‰È·ÊÔÚÔÔÈËıÔ‡Ó ÛÙ· ÒÚÈÌ· ·ıÚÔÈÛÙÈο ÛˆÏËÓ¿ÚÈ· ÙÔ˘ ÓÂÊÚÔ‡. ∞fi ÙËÓ ¿ÏÏË, Ù· ·ÙÙ·Ú· ÙÔ˘ ÌÂÛÂÁ¯‡Ì·ÙÔ˜ ·Ú¯›˙Ô˘Ó Ó· ˘ÎÓÒÓÔ˘Ó ÛÙȘ ÎÔÚ˘Ê¤˜ ÙÔ˘ ‰È·ÎÏ·‰È˙fiÌÂÓÔ˘ ÂÈıËÏ›Ô˘ (∂ÈÎfiÓ· 2µ). ™˘Ì·Á›˜ ÔÌ¿‰Â˜ Ù¤ÙÔÈˆÓ ÌÂÛÂÁ¯˘Ì·ÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ ÌÂÙ·ÙÚ¤ÔÓÙ·È ·ÎÔÏÔ‡ıˆ˜ Û ÂÈıËÏȷο, Ù· ÔÔ›· Û¯ËÌ·Ù›˙Ô˘Ó ÌÈÎÚ¿ ΢ÛÙ›‰È· (∂ÈÎfiÓ· 2°). ∞˘Ù¿, ·ÎÔÏÔ˘ıÔ‡Ó ÌÈ· ÛÙÂÚÂfiÙ˘Ë ÛÂÈÚ¿ ÌÔÚÊÔÁÂÓÂÙÈÎÒÓ ·ÏÏ·ÁÒÓ Î·È ‰È·ÊÔÚÔÔÈÔ‡ÓÙ·È Û ÛÈÁÌÔÂȉ‹ ÛˆÏËÓ¿ÚÈ·, ÙˆÓ ÔÔ›ˆÓ ÙÔ ¤Ó· ¿ÎÚÔ Û˘Ó‰¤ÂÙ·È Ì ÙÔ ·Ú·Î›ÌÂÓÔ ·ıÚÔÈÛÙÈÎfi ÛˆÏËÓ¿ÚÈÔ, ÂÓÒ ÛÙÔ ¿ÏÏÔ ‰ËÌÈÔ˘ÚÁÔ‡ÓÙ·È Ù· ÓÂÊÚÈο ÛÂÈÚ¿Ì·Ù· Ì ÙËÓ ÂÈÛ¯ÒÚËÛË ÙÚȯÔÂȉÒÓ ·ÈÌÔÊfiÚˆÓ ·ÁÁ›ˆÓ (∂ÈÎfiÓ˜ 2¢-∂). √È ÚÒÈ̘ ·˘Ù¤˜ ‰Ô̤˜ ÙÔ˘ ÌÂÙ¿ÓÂÊÚÔ˘ ˘Ô‚¿ÏÏÔÓÙ·È ÛÙË Û˘Ó¤¯ÂÈ· ÛÙË ‰È·‰Èηۛ· Ù˘ ˆÚ›Ì·ÓÛ˘, Ì ·ÔÙ¤ÏÂÛÌ· ÙË ‰ËÌÈÔ˘ÚÁ›· ·Ó·ÙÔÌÈο Î·È Ê˘ÛÈÔÏÔÁÈο ͯˆÚÈÛÙÒÓ ‰ÔÌÒÓ, fiˆ˜ ÙÔ ÂÁÁ‡˜ ÂÛÂÈڷ̤ÓÔ ÛˆÏËÓ¿ÚÈÔ, Ë ·Á·ÏË ÙÔ˘ Henle Î·È ÙÔ ¿ˆ ÂÛÂÈڷ̤ÓÔ ÛˆÏËÓ¿ÚÈÔ (∂ÈÎfiÓ· 2∂). ∏ ·Ó¿Ù˘ÍË ÙˆÓ ÓÂÊÚÒÓ ÂÚÈÏ·Ì‚¿ÓÂÈ ‰È·‰Èηۛ˜ fiˆ˜ ÂÈıËÏȷΤ˜-ÌÂÛÂÁ¯˘Ì·ÙÈΤ˜
∂ÈÎfiÓ· 2. ¢ËÌÈÔ˘ÚÁ›· ÙˆÓ ÓÂÊÚÒÓˆÓ. ∞) Ë Ô˘ÚËÙËÚÈ΋ ηٷ‚ÔÏ‹ (√∫) ÌÂÙ¿ ÙËÓ Â·ÁˆÁ‹ Ù˘ ÂÈÛ‚¿ÏÏÂÈ ÛÙÔ Î·-
ıÔÚÈṲ̂ÓÔ ÌÂÙ·ÓÂÊÚÈÎfi ÌÂÛ¤Á¯˘Ì· (ªª). µ) ™ËÌ·ÙÔ‰ÔÙÈο ÌfiÚÈ· ·fi ÙÔ ªª ÚÔηÏÔ‡Ó ÙË Û˘Ì‡ÎÓˆÛË ÙˆÓ
ÌÂÛÂÁ¯˘Ì·ÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ ÛÙȘ ÎÔÚ˘Ê¤˜ Ù˘ √∫. °) ∆· Û˘Ì˘Îӈ̤ӷ ÌÂÛÂÁ¯˘Ì·ÙÈο ·ÙÙ·Ú· (™ª) ÂÈıËÏÈÔ-
ÔÈÔ‡ÓÙ·È Î·È Û¯ËÌ·Ù›˙Ô˘Ó Î˘ÛÙ›‰È· (¡∫) Ù· ÔÔ›· ·ÎÔÏÔ‡ıˆ˜ ‰È·ÊÔÚÔÔÈÔ‡ÓÙ·È (¢) Û ÛÈÁÌÔÂȉ‹ ÛˆÏËÓ¿ÚÈ· (S). ∂) ∫·Ù¿ ÙË ‰È·‰Èηۛ· Ù˘ ˆÚ›Ì·ÓÛ˘ ÙÔ ¤Ó· ¿ÎÚÔ ÙˆÓ ÓÂÊÚÈÎÒÓ ÛˆÏËÓ·Ú›ˆÓ
Ì ÙÔ ·ıÚÔÈÛÙÈÎfi ÛˆÏËÓ¿ÚÈÔ (∞ı™), ÂÓÒ ÛÙÔ ¿ÏÏÔ ‰ËÌÈÔ˘ÚÁÂ›Ù·È ÙÔ ÓÂÊÚÈÎfi Û›ڷ̷ (¡™) Ì ÙËÓ ÂÈÛ¯ÒÚËÛË ÙÚȯÔÂȉÒÓ ·ÈÌÔÊfiÚˆÓ ·ÁÁ›ˆÓ. ∆Ì‹Ì·Ù· ÙˆÓ ÓÂÊÚÈÎÒÓ ÛˆÏËÓ·Ú›ˆÓ ‰È·ÊÔÚÔÔÈÔ‡ÓÙ·È Û ·Ó·ÙÔÌÈο Î·È ÏÂÈÙÔ˘ÚÁÈο ͯˆÚÈÛÙ¤˜ ‰Ô̤˜, fiˆ˜ ÙÔ ÂÁÁ‡˜ (∂™) Î·È ¿ˆ (∞™) ÛˆÏËÓ¿ÚÈÔ Î·È Ë ·Á·ÏË ÙÔ˘ Henle (H). B: ο„· ÙÔ˘ Bowman.
·ÏÏËÏÂȉڿÛÂȘ, ‰È·ÎÏ¿‰ˆÛË ÂÈıËÏ›ˆÓ, ΢Ù-
Ù·ÚÈ΋ ‰È·ÊÔÚÔÔ›ËÛË, ÌÂÙ·Û¯ËÌ·ÙÈÛÌfi ÌÂ-
ÛÂÁ¯˘Ì·ÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ Û ÂÈıËÏȷο ηÈ
ÌÔÚÊÔÁ¤ÓÂÛË, ÔÈ Ôԛ˜ ÌÔÚÔ‡Ó Ó· ÌÂÏÂÙËıÔ‡Ó ÛÂ Û˘ÛÙ‹Ì·Ù· in vitro ÈÛÙÔηÏÏȤÚÁÂÈ·˜.
°È· ÙÔ˘˜ ·Ú·¿Óˆ ÏfiÁÔ˘˜, Ë ·Ó¿Ù˘ÍË ÙˆÓ
ÓÂÊÚÒÓ ·ÔÙÂÏ› Â‰Ò Î·È ¿Óˆ ·fi 40 ¯ÚfiÓÈ· ¤Ó· ȉ·ÓÈÎfi Û‡ÛÙËÌ· ÁÈ· ÙË ÌÂϤÙË ‚·ÛÈÎÒÓ ·Ú¯ÒÓ Ù˘ ·Ó·Ù˘Íȷ΋˜ ‚ÈÔÏÔÁ›·˜ Î·È ÔÚÁ·ÓÔÁ¤ÓÂÛ˘. ™ÙËÓ Ù·˘ÙÔÔ›ËÛË ÙÔ˘ ÚfiÏÔ˘ ÂÓfi˜ ÌÂÁ¿ÏÔ˘ ·ÚÈıÌÔ‡ ÁÔÓȉȷÎÒÓ ÚÔ˚fiÓÙˆÓ Ô˘ ÂÌϤÎÔÓÙ·È ÛÙËÓ ·Ó¿Ù˘ÍË ÙˆÓ ÓÂÊÚÒÓ ¤¯ÂÈ Û˘Ì‚¿ÏÂÈ ÛËÌ·ÓÙÈο Ë ¯Ú‹ÛË ˙ˆÈÎÒÓ ÌÔÓ٤ψÓ, ÌÂٷ͇ ·˘ÙÒÓ Î·È ÙÔ˘ ÔÓÙÈÎÔ‡ (‚Ï. ¶›Ó·Î· 1). ¶·Ú·Î¿Ùˆ ·Ó·Ê¤ÚÔÓÙ·È Ù· ΢ÚÈfiÙÂÚ· ÛÙ¿‰È· Ù˘
ÓÂÊÚÈ΋˜ ·Ó¿Ù˘Í˘ Î·È ÔÈ ÛËÌ·ÓÙÈÎfiÙÂÚÔÈ ·Ú¿ÁÔÓÙ˜ Ô˘ ÂÌϤÎÔÓÙ·È ÛÙÔ Î·ı¤Ó·.
∫·ıÔÚÈÛÌfi˜ (specification) ÙÔ˘ ÌÂÙ·ÓÂÊÚÈÎÔ‡ ÌÂÛÂÁ¯‡Ì·ÙÔ˜ ∞·Ú·›ÙËÙË ÚÔ¸fiıÂÛË ÁÈ· ÙËÓ ·Ó¿Ù˘ÍË ÙˆÓ ÌÂÙ¿ÓÂÊÚˆÓ Â›Ó·È Ô Î·ıÔÚÈÛÌfi˜ ÙÔ˘ Ô˘Ú·›Ô˘ ¿ÎÚÔ˘ ÙÔ˘ ‰È¿ÌÂÛÔ˘ ÌÂÛÔ‰¤ÚÌ·ÙÔ˜ Î·È Ë
ÔÈ Ôԛ˜ Â›Ó·È Î˘Ú›ˆ˜ ÌÂÙ·ÁÚ·ÊÈÎÔ› ·Ú¿ÁÔÓÙ˜.
∏ ÚˆÙ½ÓË Eya1*, ÔÌfiÏÔÁË ÙÔ˘ ÌÂÙ·ÁÚ·ÊÈÎÔ‡ ·Ú¿ÁÔÓÙ· eyes absent Ù˘ ‰ÚÔÛfiÊÈÏ·˜, Ê·›ÓÂÙ·È Ó· Â›Ó·È Ô ÚÒÙÔ˜ ·Ú¿ÁÔÓÙ·˜ Ô˘ ··ÈÙÂ›Ù·È ÁÈ· ÙÔÓ Î·ıÔÚÈÛÌfi ÙÔ˘ ÌÂÙ·ÓÂÊÚÈ-
ÎÔ‡ ÌÂÛÂÁ¯‡Ì·ÙÔ˜. ø˜ Û˘Ó¤ÂÈ· Ù˘ ·ÂÓÂÚÁÔÔ›ËÛ˘ ÙÔ˘ ÁÔÓȉ›Ô˘ Eya1 * ·Ú·ÙËÚ›ٷÈ
·Ô˘Û›· ÙˆÓ ÌÂÙ¿ÓÂÊÚˆÓ, ÂÓÒ ÔÈ ÌÂÛfiÓÂÊÚÔÈ
·Ó·Ù‡ÛÛÔÓÙ·È Î·ÓÔÓÈο (6).
™Â
ÔÓÙ›ÎÈ· ÌÂÙ·ÏÏ·Á̤ӷ ÁÈ· ÙÔ ÌÂÙ·ÁÚ·ÊÈÎfi ·Ú¿ÁÔÓÙ· Lim1, Ô ÔÔ›Ô˜ ·Ó‹ÎÂÈ ÛÙËÓ
ÔÈÎÔÁ¤ÓÂÈ· ÙˆÓ homeodomain ÚˆÙÂ˚ÓÒÓ Î·È ÂÎÊÚ¿˙ÂÙ·È Î·Ù¿ Ì‹ÎÔ˜ ÙÔ˘ ‰È¿ÌÂÛÔ˘ ÌÂÛÔ‰¤ÚÌ·ÙÔ˜, ‰ÂÓ Û¯ËÌ·Ù›˙ÔÓÙ·È Î·ıfiÏÔ˘ ÔÈ ÌÂÛfiÓÂÊÚÔÈ Î·È ÔÈ ÌÂÙ¿ÓÂÊÚÔÈ (7). √ ·Ú·¿Óˆ
Ê·ÈÓfiÙ˘Ô˜ ·ÓȯÓ‡ÂÙ·È Û ¤Ó· ÌÈÎÚfi ÔÛÔÛÙfi
ÙˆÓ ÌÂÙ·ÏÏ·ÁÌ¤ÓˆÓ ÂÌ‚Ú‡ˆÓ Î·È ÚÔηÏÂ›Ù·È ·fi ‰È·Ù·Ú·¯¤˜ ÛÙË ‰ËÌÈÔ˘ÚÁ›· ÙÔ˘ ÌÂÛÔÓÂÊÚÈÎÔ‡ fiÚÔ˘,
Î·È ÛÙË
°ÔÓ›‰ÈÔŒÎÊÚ·ÛË/ º·ÈÓfiÙ˘Ô˜
∞ÓıÚÒÈÓÔ ∞Ó·ÊÔÚ¿ ∂ÓÙÔÈÛÌfi˜(ÔÓÙÈÎfi˜)Û‡Ó‰ÚÔÌÔ
∞. ªÂÙ·ÁÚ·ÊÈÎÔ› ·Ú¿ÁÔÓÙ˜**
Pax2 ª¶, √∫, ∞™, ªª√∫: ‰ÂÓ Â¿ÁÂÙ·È,Renal coloboma74 Ÿ¯È Û ÒÚÈÌÔ˘˜ ÓÂÊÚÒÓ˜ªª: ‰ÂÓ ‰È·ÊÔÚÔÔÈ›ٷÈ
Eya1
ªª√∫: ‰ÂÓ ‰ËÌÈÔ˘ÚÁ›ٷÈ, Branchio-oto-renal 75 ªª: ‰ÂÓ Î·ıÔÚ›˙ÂÙ·È(BOR)
Lim1 ¢ªª¶: ‰ÂÓ Û¯ËÌ·Ù›˙ÂÙ·È, ¢È·Ù·Ú·¯¤˜ 8 ÛÙË ÌÔÚÊÔÔ›ËÛË ÙˆÓ ÓÂÊÚÒÓˆÓ
WT1 ªªªª: ·fiÙˆÛËWAGR*, Denys-Drash, Frasier36
Six1 ªª (Á‡Úˆ ·fi √∫)√∫: ‰ÂÓ ÂÈÛ‚¿ÏÏÂÈ Ï‹Úˆ˜ Branchio-oto-renal (BOR)19 ÛÙÔ ªª (·fiÙˆÛË)
Sall1 ªª (Á‡Úˆ ·fi √∫), √∫: ‰ÂÓ ÂÈÛ‚¿ÏÏÂÈ Ï‹Úˆ˜ Townes-Brocks21 S, ¶√¢ÛÙÔ ªª (·fiÙˆÛË)
Foxc1 MM¢ÈÏ‹ √∫, ¢ÈÏÔ› ÓÂÊÚÔ›Axenfeld-Rieger24
Emx2 OK, MM√∫: ‰ÂÓ ‰È·ÎÏ·‰›˙ÂÙ·È, 32 ªª: ·fiÙˆÛË
Brn1 ∏, ∞™∏: ‰ÂÓ ÂÈÌË·ÓÂÙ·È53
Lmx1b ¶√¢¢È·Ù·Ú·¯¤˜ ÛÙË µª ÙˆÓ ¡™Nail-patella54
HNF1 ∂Á™°Ï˘ÎÔ˙Ô˘Ú›·, ·ÌÈÓÔÍÔ˘Ú›· 59 (Û‡Ó‰ÚÔÌÔ Fanconi)
Foxd1 ™ÙÚªÂȈ̤ÓÔ˜ ·ÚÈıÌfi˜ ÓÂÊÚÒÓˆÓ, 60 ¤ÎÙÔÔÈ, Û˘Á¯ˆÓÂ˘Ì¤ÓÔÈ ÓÂÊÚÔ›
Pod1 ™ª, ¶√¢ªÂȈ̤ÓÔ˜ ·ÚÈıÌfi˜ ÓÂÊÚÒÓˆÓ63 ¶√¢: ‰ÂÓ ‰È·ÊÔÚÔÔÈÔ‡ÓÙ·È
µ. ∞˘ÍËÙÈÎÔ› ·Ú¿ÁÔÓÙ˜**
GDNF ªª√∫: ‰ÂÓ ‰ËÌÈÔ˘ÚÁ›ٷÈHirschsprung12-14
GDF11 √∫, ªª√∫: ‰ÂÓ ‰ËÌÈÔ˘ÚÁ›ٷÈ11
BMP4 ªª (Á‡Úˆ ·fi ª¶)BMP4+/-: ÀÔ-‰˘ÛÏ·Û›·, 26 ˘‰ÚÔ˘ÚËÙ‹Ú·˜
BMP7 √∫, ªªÀÔÏ·Û›·, ÌÂȈ̤ÓÔ˜ ·ÚÈıÌfi˜
76 ÓÂÊÚÒÓˆÓ, ∞™
Slit2 ª¶, ªª¶ÔÏϷϤ˜ √∫, ˘‰ÚÔ˘ÚËÙ‹Ú·˜, 23 ÔÏ˘ÏÔ‚Ò‰ÂȘ ÓÂÊÚÔ›
FGF8 ™ª, ¡™ªÂȈ̤ÓË: ‰È·ÎÏ¿‰ˆÛË √∫, 45 ‰È·ÊÔÚÔÔ›ËÛË ¡™
Wnt4 ªªªª: ‰ÂÓ ÂÈıËÏÈÔÔÈ›ٷÈ, ˘ÔÏ·Û›·41
Wnt11 ∫ÔÚ˘Ê‹ √∫√∫: ÌÂȈ̤ÓË ‰È·ÎÏ¿‰ˆÛË, ˘ÔÏ·Û›·31
Grem1 ™ª (Á‡Úˆ ·fi √∫)√∫: ‰ËÌÈÔ˘ÚÁÂ›Ù·È ·ÏÏ¿ 22 ‰ÂÓ ‰È·ÎÏ·‰›˙ÂÙ·È °. Àԉԯ›˜**
Ret √∫√∫: ‰ÂÓ ‰ËÌÈÔ˘ÚÁ›ٷÈ15
GFR·1 √∫, ªª√∫: ‰ÂÓ ‰ËÌÈÔ˘ÚÁ›ٷÈ17, 18
Robo2 ªª, ª¶ (ÂÚÈÔ¯‹ ¶ÔÏϷϤ˜ √∫, ˘‰ÚÔ˘ÚËÙ‹Ú·˜, 23 ‰ËÌÈÔ˘ÚÁ›·˜ √∫)ÔÏ˘ÏÔ‚Ò‰ÂȘ ÓÂÊÚÔ› Sprouty1 √∫¶ÔÏϷϤ˜ √∫, Ô˘ÚËÙ‹Ú˜25 ¢. ∂ÍˆÎ˘ÙÙ¿ÚȘ ÚˆÙ½Ó˜** HS2ST √∫, ªª (ÚÈÓ ¢È·Ù·Ú·¯¤˜ Û ‰È·ÎÏ¿‰ˆÛË √∫, 65 ‰È·ÊÔÚÔÔ›ËÛË ¡™)Û˘Ì‡ÎÓˆÛË ªª Nidogen1, ∂ÈıËÏȷΤ˜ µªª¶: ‰ÂÓ ÂÈÌË·ÓÂÙ·È, ·ÁÂÓÂÛ›·66 LamininÁ1 Fras1, ∂ÈıËÏȷΤ˜ µª√∫: ‰ËÌÈÔ˘ÚÁ›ٷÈ, ªª: Fraser68, 70, Frem1, ‰ÂÓ ‰È·ÊÔÚÔÔÈÂ›Ù·È 72, 73 Frem2 ∞fiÙˆÛË, ·ÁÂÓÂÛ›· ∂. ÕÏϘ**
Grip1 √∫, ÂÈı‹ÏÈ· √∫: ‰ËÌÈÔ˘ÚÁ›ٷÈ, Fraser71 ÓÂÊÚÒÓˆÓªª: ‰ÂÓ ‰È·ÊÔÚÔÔÈÂ›Ù·È Psn1, 2 ¡ÂÊÚÒÓ˜¢È·ÊÔÚÔÔ›ËÛË ¡™ ÛÙ·Ì·Ù¿ Alzheimer’s58 ÛÙÔ ÛÙ¿‰ÈÔ
∏:
ÙÔ˘ Henle, ªª: ªÂÙ·ÓÂÊÚÈÎfi ÌÂÛ¤Á¯˘Ì·, ª¶: ªÂÛÔÓÂÊÚÈÎfi˜ fiÚÔ˜, ¡™: ¡ÂÊÚÈο ÛÂÈÚ¿Ì·Ù·, √∫: Ô˘ÚËÙËÚÈ΋ ηٷ‚ÔÏ‹. ¶√¢: ¶Ô‰Ô·ÙÙ·Ú·, ™ª: ™˘Ì˘Îӈ̤ӷ ÌÂÛÂÁ¯˘Ì·ÙÈο ·ÙÙ·Ú·, ™ÙÚ: ™ÙÚÒÌ·, S: ™ÈÁÌÔÂȉ‹ ÛˆÏËÓ¿ÚÈ· *Wilms’ tumor - Aniridia - genitourinary abnormalities - mental retardation **∞ӷʤÚÂÙ·È ÛÙËÓ ÔÈÎÔÁ¤ÓÂÈ· ÙˆÓ ÚˆÙÂ˚ÓÒÓ Ô˘ Έ‰ÈÎÔÔÈÔ‡ÓÙ·È ·fi Ù· ·Ó·ÁÚ·ÊfiÌÂÓ· ÁÔÓ›‰È·
‰ËÌÈÔ˘ÚÁ›· Î·È ‰È·ÎÏ¿‰ˆÛË Ù˘ √∫, ηıÒ˜
Î·È ÛÙË ÌÔÚÊÔÔ›ËÛË (patterning) ÙˆÓ ÂÈıË-
Ï›ˆÓ ÙˆÓ ÓÂÊÚÒÓˆÓ.
∂ͤ¯ÔÓÙ· ÚfiÏÔ ÛÙË ‰È·‰Èηۛ· Ù˘ ÓÂÊÚÔ-
Á¤ÓÂÛ˘ Ê·›ÓÂÙ·È Ó· ·›˙Ô˘Ó ÔÈ ·Ú¿ÁÔÓÙ˜
Pax2 Î·È Pax8, ÔÈ ÔÔ›ÔÈ ·Ó‹ÎÔ˘Ó ÛÙËÓ ÔÈÎÔÁ¤-
ÓÂÈ· ÙˆÓ paired domain ÌÂÙ·ÁÚ·ÊÈÎÒÓ ·Ú·-
ÁfiÓÙˆÓ. ∏ ¤ÎÊÚ·ÛË ÙÔ˘ ÁÔÓȉ›Ô˘ Pax2 ·Ú¯›˙ÂÈ
·fi ÙÔ ÛÙ¿‰ÈÔ ÙÔ˘ ÚfiÓÂÊÚÔ˘. øÛÙfiÛÔ, Û ÔÓÙ›ÎÈ· Ì ¤ÏÏÂÈ„Ë Ù˘ Pax2, Ô ÌÂÛÔÓÂÊÚÈÎfi˜
fiÚÔ˜ Û¯ËÌ·Ù›˙ÂÙ·È, ·ÏÏ¿ ÂÎÊ˘Ï›˙ÂÙ·È Ôχ
ÓˆÚ›˜, Ì ·ÔÙ¤ÏÂÛÌ· Ó· ÌË Û¯ËÌ·Ù›˙ÔÓÙ·È ÙfiÛÔ Ô ÌÂÛfiÓÂÊÚÔ˜ fiÛÔ Î·È Ô ÌÂÙ¿ÓÂÊÚÔ˜ (9).
∂ӉȷʤÚÔÓ ·ÚÔ˘ÛÈ¿˙ÂÈ ÙÔ ÁÂÁÔÓfi˜ fiÙÈ, ·ÚfiÏÔ Ô˘ Ë ·ÂÓÂÚÁÔÔ›ËÛË ÙÔ˘ ÁÔÓȉ›Ô˘ Pax8
‰ÂÓ ÚÔηÏ› ‚Ï¿‚˜ ÛÙËÓ ·Ó¿Ù˘ÍË ÙˆÓ ÓÂÊÚÒÓ, ÂÓÙÔ‡ÙÔȘ, Û ‰ÈÏ¿ ÌÂÙ·ÏÏ·Á̤ӷ ˙Ò· Pax2-/-/Pax8-/-** ‰ÂÓ Û¯ËÌ·Ù›˙ÂÙ·È Ô‡ÙÂ Ô ÚfiÓÂÊÚÔ˜ (10). ∏ ·Ú·¿Óˆ ·ÔÙÂÏ› ÙË ÌÔÓ·‰È΋
ÁÓˆÛÙ‹ ÌÂÙ¿ÏÏ·ÍË Ë ÔÔ›· ÂËÚ¿˙ÂÈ ÙËÓ ÓÂÊÚÔÁ¤ÓÂÛË ÛÙÔ ÛÙ¿‰ÈÔ ÙÔ˘ ÚfiÓÂÊÚÔ˘.
¶Èı·Ófi˜ ÚfiÏÔ˜ ÛÙÔÓ Î·ıÔÚÈÛÌfi ÙÔ˘ ÌÂÙ·ÓÂÊÚÈÎÔ‡ ÌÂÛÂÁ¯‡Ì·ÙÔ˜ ¤¯ÂÈ Â›Û˘ ·Ô‰Ôı›
Î·È ÛÙËÓ ÂÎÎÚÈÓfiÌÂÓË ÚˆÙ½ÓË GDF11 (Growth differentiation factor 11), Ë ÔÔ›· ·Ó‹ÎÂÈ ÛÙËÓ ÔÈÎÔÁ¤ÓÂÈ· ÙˆÓ Transforming growth factor-‚ (TGF-‚) ·Ú·ÁfiÓÙˆÓ (11).
∂·ÁˆÁ‹ Ù˘ Ô˘ÚËÙËÚÈ΋˜ ηٷ‚ÔÏ‹˜
∆Ô Î‡ÚÈÔ ÛËÌ·ÙÔ‰ÔÙÈÎfi ÌfiÚÈÔ (signaling molecule) ÙÔ˘ ÌÂÛÂÁ¯‡Ì·ÙÔ˜ Ô˘ Â›Ó·È ˘Â‡ı˘ÓÔ ÁÈ· ÙËÓ Â·ÁˆÁ‹ Ù˘ Ô˘ÚËÙËÚÈ΋˜ ηٷ‚ÔÏ‹˜ (√∫) Â›Ó·È Ô ·Ú¿ÁÔÓÙ·˜ GDNF (Glial cell-derived neurotrophic factor), Ô ÔÔ›Ô˜ ·Ó‹ÎÂÈ ÛÙËÓ ÔÈÎÔÁ¤ÓÂÈ· ÙˆÓ TGF-‚ ÚˆÙÂ˚ÓÒÓ. ∞˘Ùfi˜ ÂÎÎÚ›ÓÂÙ·È ·fi ÙÔ Î·ıÔÚÈṲ̂ÓÔ ÌÂÙ·ÓÂÊÚÈÎfi ÌÂÛ¤Á¯˘Ì· Î·È ÚÔÛ‰¤ÓÂÙ·È Û ¤Ó· Û‡ÌÏÔÎÔ ˘Ô‰Ô¯¤ˆÓ ·ÔÙÂÏÔ‡ÌÂÓÔ ·fi ÙËÓ ÎÈÓ¿ÛË Ù˘ÚÔÛ›Ó˘ Ret Î·È ÙÔ Û˘Ó˘Ô‰Ô¯¤· GFR·1, Ô˘ ÂÓÙÔ›˙ÂÙ·È ÛÙ· ÂÈıËÏȷο ·ÙÙ·Ú· ÙÔ˘ ÌÂÛÔÓÂÊÚÈÎÔ‡ fiÚÔ˘. ∏ ÛËÌ·Û›· Ù˘ ÛËÌ·ÙÔ‰fiÙË-
Û˘ Ù˘ GDNF ÛÙË ÈÛÙÔÁ¤ÓÂÛË ÙÔ˘ ÌÂÙ¿ÓÂ-
ÊÚÔ˘ ‰È·Ê·›ÓÂÙ·È ·fi ÙÔ ÁÂÁÔÓfi˜ fiÙÈ ÙfiÛÔ Ë
·ÂÓÂÚÁÔÔ›ËÛË ÙÔ˘ ›‰ÈÔ˘ ÙÔ˘ GDNF (12-14)
·ÏÏ¿ Î·È ÙˆÓ Ret (15,16) Î·È GFRa1 (17,18)
Ô‰ËÁ› Û ·ÁÂÓÂÛ›· ÙˆÓ ÓÂÊÚÒÓ. ¶·ÚfiÏ· ·˘Ù¿, Ê·›ÓÂÙ·È fiÙÈ Ë GDNF ‰ÂÓ ·ÔÙÂÏ› ÙÔ ÌÔÓ·‰ÈÎfi ÛËÌ·ÙÔ‰ÔÙÈÎfi ÌfiÚÈÔ ÙÔ˘ ÌÂÛÂÁ¯‡Ì·ÙÔ˜.
¶ÂÚ›Ô˘ ÛÙÔ ¤Ó· ÙÚ›ÙÔ ÙˆÓ GDNF Î·È ÙˆÓ Ret
Ù˘
O∫ ηÈ
Ë ‰ËÌÈÔ˘ÚÁ›· ‰˘ÛÏ·ÛÙÈÎÒÓ ÓÂÊÚÒÓ (15,16).
∂Ӊ›ÍÂȘ Û¯ÂÙÈο Ì ÙÔ˘˜ Ì˯·ÓÈÛÌÔ‡˜
·ÁˆÁ‹˜ Ù˘ √∫ ¤¯Ô˘Ó ÚÔ·„ÂÈ ·fi ÙËÓ
·ÂÓÂÚÁÔÔ›ËÛË ÙˆÓ ÌÂÙ·ÁÚ·ÊÈÎÒÓ ·Ú·Áfi-
ÓÙˆÓ Six1 (19,20) Î·È Sall1 (21). ™˘ÁÎÂÎÚÈ̤ӷ, Û ÔÓÙ›ÎÈ· Ì ÌÂÙ·ÏÏ¿ÍÂȘ ÛÙ· ·ÓÙ›ÛÙÔȯ· ÁÔÓ›‰È· Six1 ‹ Sall1, ÙÔ ÌÂÙ·ÓÂÊÚÈÎfi ÌÂÛ¤Á¯˘Ì·
Ê·›ÓÂÙ·È Ó· ηıÔÚ›˙ÂÙ·È Î·ÓÔÓÈο, ÌÔÏÔÓfiÙÈ Ô
·ÚÈıÌfi˜ ÙˆÓ ÌÂÛÂÁ¯˘Ì·ÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ ·ÚÔ˘ÛÈ¿˙ÂÙ·È ÌÂȈ̤ÓÔ˜. ∫·Ù¿ Û˘Ó¤ÂÈ· ηÈ
ÛÙȘ ‰‡Ô ÂÚÈÙÒÛÂȘ ·Ú·ÙËÚÂ›Ù·È ·ÁÂÓÂÛ›· ‹ ˘ÔÏ·Û›· ÙˆÓ ÓÂÊÚÒÓ, Ô˘ ÔÊ›ÏÂÙ·È ÛÙÔ ÁÂÁÔÓfi˜ fiÙÈ
ÙˆÓ Six1 Î·È Sall1 ÌÂÙ·ÏÏ·ÁÌ¤ÓˆÓ ÔÓÙÈ-
ÙÔ˘
ÁÔÓȉ›Ô˘ Grem1, ÙÔ ÔÔ›Ô ÂÎÊÚ¿˙ÂÙ·È ÛÙÔ Ô˘Ú·›Ô ¿ÎÚÔ ÙÔ˘ ÌÂÛÔÓÂÊÚÈÎÔ‡ fiÚÔ˘ Î·È ÙÔ˘
ÌÂÙ·ÓÂÊÚÈÎÔ‡ ÌÂÛÂÁ¯‡Ì·ÙÔ˜ (22). ∆Ô Grem1 Έ‰ÈÎÔÔÈ› ÙËÓ ÚˆÙ½ÓË Gremlin, Ë ÔÔ›· ·ÓÙ·ÁˆÓ›˙ÂÙ·È ÙËÓ ‰Ú¿ÛË ÙˆÓ ·Ú·ÁfiÓÙˆÓ
BMP (Bone Morphogenic Protein), ΢ڛˆ˜ Ù˘
BMP2 Î·È BMP4 (‚Ï. ·Ú·Î¿Ùˆ). √ Ê·ÈÓfiÙ˘-
Ô˜ ÛÙÔ˘˜ ÓÂÊÚÔ‡˜ ÙˆÓ Grem1-/- ÔÓÙÈÎÒÓ Î·Ù·‰ÂÈÎÓ‡ÂÈ ÙË ÛËÌ·Û›· ÙÔ˘ ·ÓÙ·ÁˆÓÈÛÌÔ‡ Ù˘
ÛËÌ·ÙÔ‰ÔÙÈ΋˜ ‰Ú¿Û˘ ÙˆÓ BMP ·fi ÙË
Gremlin ÛÙË ‰ËÌÈÔ˘ÚÁ›· Ù˘ √∫.
™ËÌ·ÓÙÈÎfi˜ ÁÈ· ÙËÓ Â·ÁˆÁ‹ Ù˘ √∫ ›ӷÈ
›Û˘ Ô ÚfiÏÔ˜ ÙˆÓ ÂÎÎÚÈÓfiÌÂÓˆÓ ÚˆÙÂ˚ÓÒÓ SLIT Î·È ÙˆÓ ˘Ô‰Ô¯¤ˆÓ ÙÔ˘˜ ROBO. ∆Ô ÁÔÓ›‰ÈÔ Slit2 ÂÎÊÚ¿˙ÂÙ·È Î·Ù¿ Ì‹ÎÔ˜ ÙÔ˘ ÌÂÛÔÓÂÊÚÈÎÔ‡ fiÚÔ˘, ·ÏÏ¿ Î·È ¤ÓÙÔÓ· ÛÙÔ ‰È¿ÌÂÛÔ
ÌÂÛfi‰ÂÚÌ·, ·ÎÚÈ‚Ò˜ ÎÂÊ·ÏÈÎfiÙÂÚ· Ù˘ ÂÚÈÔ-
¯‹˜ ¤ÎÊÚ·Û˘ ÙÔ˘ GDNF. ∏ ·ÂÓÂÚÁÔÔ›ËÛË
ÙfiÛÔ ÙÔ˘ Slit2 fiÛÔ Î·È ÙÔ˘ Robo2 ÚÔηÏ› ·ÚfiÌÔÈÔ Ê·ÈÓfiÙ˘Ô, Ô ÔÔ›Ô˜ ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÙÔÓ Û¯ËÌ·ÙÈÛÌfi ÔÏÏ·ÏÒÓ √∫, ÔÈ Ôԛ˜ ÂÎʇÔÓÙ·È ÎÂÊ·ÏÈÎfiÙÂÚ· ÙÔ˘ ηÓÔÓÈÎÔ‡ ÛËÌ›Ԣ
Ù˘
(23). ™Â ÌÔÚÈ·Îfi
Ë ¤ÏÏÂÈ„Ë ÙˆÓ Slit2 Î·È Robo2 ÚÔηÏ› ¤ÎÙ·ÛË Ù˘ ¤ÎÊÚ·Û˘ ÙÔ˘ GDNF ÎÂÊ·ÏÈÎfiÙÂÚ·, Ô˘ ÛËÌ·›ÓÂÈ fiÙÈ ÙÔ Û‡ÛÙËÌ· Slit2/Robo2 Ê˘ÛÈÔÏÔÁÈο
¶·È‰È·ÙÚÈ΋ 2006;69:255-267
‰È·Ì¤ÛÔ˘ ÌÂÛÔ‰¤ÚÌ·ÙÔ˜. ™ÙÔÓ Î·ıÔÚÈÛÌfi Ù˘
ÂÚÈÔ¯‹˜ ¤ÎÊÚ·Û˘ ÙÔ˘ GDNF ÂÌϤÎÂÙ·È Î·È
Ô ÌÂÙ·ÁÚ·ÊÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ Foxc1. Ÿˆ˜ ηÈ
Ì ÙȘ Slit2/Robo2, Ë ¤ÏÏÂÈ„Ë Ù˘ Foxc1 Û ÌÂ-
Ù·ÏÏ·Á̤ӷ ÔÓÙ›ÎÈ· ÚÔηÏ› ¤ÎÙ·ÛË Ù˘
ÂÚÈÔ¯‹˜ ¤ÎÊÚ·Û˘ ÙÔ˘ GDNF ÎÂÊ·ÏÈÎfiÙÂÚ·, Ì ·ÔÙ¤ÏÂÛÌ· ÙËÓ ¤ÎÙÔË Â·ÁˆÁ‹ ÚfiÛıÂ-
Ù˘ √∫ Î·È ÙË ‰ËÌÈÔ˘ÚÁ›· ‰ÈÏÒÓ ÓÂÊÚÒÓ (24).
ƒ˘ıÌÈÛÙÈÎfi ÚfiÏÔ ÛÙËÓ ¤ÎÊÚ·ÛË ÙÔ˘ GDNF
Ê·›ÓÂÙ·È Â›Û˘ Ó· ·›˙ÂÈ Ô ·Ú¿ÁÔÓÙ·˜
Sprouty-1, Ô ÔÔ›Ô˜ ‰Ú· Û·Ó ·ÓÙ·ÁˆÓÈÛÙ‹˜
˘Ô‰Ô¯¤ˆÓ ÎÈÓ¿Û˘ Ù˘ÚÔÛ›Ó˘. ∞Ó¿ÚÎÂÈ· ÁÈ·
ÙËÓ ·Ú·¿Óˆ ÚˆÙ½ÓË Ô‰ËÁ› ÛÙË ‰ËÌÈÔ˘Ú-
Á›· ÔÏÏ·ÏÒÓ √∫ ÏfiÁˆ ·˘ÍË̤Ó˘ ·fiÎÚÈÛ˘
ÙÔ˘ ÌÂÛÔÓÂÊÚÈÎÔ‡ fiÚÔ˘ ÛÙËÓ GDNF (25).
¶Ú¿ÁÌ·ÙÈ, Ô ·Ú·¿Óˆ Ê·ÈÓfiÙ˘Ô˜ ‰ÈÔÚıÒÓÂ-
ÛÙËÓ ·ÚÔ˘Û›· ÌÂȈ̤Ó˘ ‰fiÛ˘ GDNF ÛÂ
ÂÙÂÚfi˙˘Á· GDNF ÌÂÙ·ÏÏ·Á̤ӷ ˙Ò·.
ÔÛfiÙËÙ·˜, fiÛÔ Î·È Ù˘ ÂÚÈÔ¯‹˜ ¤ÎÊÚ·Û˘
ÙÔ˘ GDNF ÛÙȘ ‰È·‰Èηۛ˜ ηıÔÚÈÛÌÔ‡ ÙÔ˘
ÌÂÛÂÁ¯‡Ì·ÙÔ˜ Î·È Â·ÁˆÁ‹˜ Ù˘
™Â ·ÓÙ›ıÂÛË Ì ÙË GDNF, Ë ÔÔ›· ‰Ú· ıÂÙÈο ÛÙË ‰ËÌÈÔ˘ÚÁ›· Ù˘ √∫, ÙÔ ÛËÌ·ÙÔ‰ÔÙÈÎfi ÌfiÚÈÔ BMP4, ÙÔ ÔÔ›Ô ·Ú¿ÁÂÙ·È ·fi ÙÔ ÌÂÛ¤Á¯˘Ì· Á‡Úˆ ·fi ÙÔÓ ÌÂÛÔÓÂÊÚÈÎfi fiÚÔ, ÂËÚ¿˙ÂÈ ·ÚÓËÙÈο ÙȘ ·Ú·¿Óˆ ‰È·‰Èηۛ˜ (26). √ Û˘Ó‰˘·ÛÌfi˜ ıÂÙÈ΋˜ Î·È ·ÚÓËÙÈ΋˜ ÛË-
Ì·ÙÔ‰ÔÙÈ΋˜ ‰Ú¿Û˘ ·fi ÙÔ ÌÂÛ¤Á¯˘Ì· ÛÙÔ
ÂÈı‹ÏÈÔ ÙÔ˘ ÌÂÛÔÓÂÊÚÈÎÔ‡ fiÚÔ˘ ÈÛÙ‡ÂÙ·È
fiÙÈ ÂÌÔ‰›˙ÂÈ ÙËÓ ÚfiˆÚË Î·È ¤ÎÙÔË ‰ËÌÈ-
Ô˘ÚÁ›· Ù˘ √∫, ‰È·ÛÊ·Ï›˙ÔÓÙ·˜ Ù·˘Ùfi¯ÚÔÓ·
ÙËÓ Â·ÁˆÁ‹ Ù˘ ÛÙÔ ÛˆÛÙfi ÛËÌ›Ô, ÛÙËÓ ÂÚÈÔ¯‹ ÙÔ˘ ÌÂÛÔÓÂÊÚÈÎÔ‡ fiÚÔ˘ ‰›Ï· ·fi ÙÔ
ÌÂÙ·ÓÂÊÚÈÎfi ÌÂÛ¤Á¯˘Ì·.
∞ÔÊ˘Á‹ ·fiÙˆÛ˘ Î·È Î˘ÙÙ·ÚÈÎfi˜ ÔÏÏ·Ï·ÛÈ·ÛÌfi˜ ÛÙÔ Î·ıÔÚÈṲ̂ÓÔ ÌÂÛ¤Á¯˘Ì· ¶ÂÈÚ¿Ì·Ù· Û in
ÛËÌ·ÙÔ‰ÔÙÈο ÌfiÚÈ· Ù· ÔÔ›· ·ÔÙÚ¤Ô˘Ó ÙÔÓ ÚÔÁÚ·ÌÌ·ÙÈṲ̂ÓÔ Î˘ÙÙ·ÚÈÎfi ı¿Ó·ÙÔ ÙÔ˘ ÌÂÛÂÁ¯‡Ì·ÙÔ˜. ∆¤ÙÔÈ· ·ÓÙÈ-·ÔÙˆÙÈ΋ ‰Ú¿ÛË ¤¯ÂÈ ‰Âȯı› ÁÈ· ÙÔÓ ·Ú¿ÁÔÓÙ· EGF (Epidermal growth factor) (27). øÛÙfiÛÔ, Ë ·ÔÙÚÔ‹ Ù˘ ·fiÙˆÛ˘ ÙˆÓ ÌÂÛÂÁ¯˘Ì·ÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ ‰ÂÓ Â›Ó·È ·fi ÌfiÓË Ù˘ ·ÚÎÂÙ‹ ÁÈ· Ó· ˘ÔÛÙËÚ›ÍÂÈ ÙËÓ ÌÂÙ¤ÂÈÙ· ·Ó¿Ù˘ÍË ÙÔ˘ ÌÂÙ¿ÓÂÊÚÔ˘. °È’ ·˘ÙfiÓ ÙÔ ÛÎÔfi ··ÈÙÂ›Ù·È Ë ·ÚÔ˘Û›· ·Ú·ÁfiÓÙˆÓ ÔÈ ÔÔ›ÔÈ ‰ÈÂÁ›ÚÔ˘Ó ÙÔÓ Î˘ÙÙ·ÚÈÎfi ÔÏÏ·Ï·ÛÈ·ÛÌfi. °ÓˆÛÙ¿ ÛËÌ·ÙÔ‰ÔÙÈο ÌfiÚÈ· Ù· ÔÔ›· ¿ÁÔ˘Ó ÙÔÓ Î˘ÙÙ·ÚÈÎfi ÔÏÏ·Ï·ÛÈ·ÛÌfi ÛÙÔ ÌÂÛ¤Á¯˘Ì· Â›Ó·È ÔÈ ·Ú¿ÁÔÓÙ˜ BMP-4 Î·È BMP-7, Ô˘ ÂÎÊÚ¿˙ÔÓÙ·È Î˘Ú›ˆ˜ ·fi Ù· ÂÈı‹ÏÈ· ÙˆÓ ‰È·ÊÔÚÔÔÈÔ‡ÌÂÓˆÓ ÓÂÊÚÒÓˆÓ (26). ∏ BMP-7 Û˘ÁÎÂÎÚÈ̤ӷ, ˘ÔÛÙËÚ›˙ÂÈ ÙfiÛÔ ÙËÓ ÂÈ‚›ˆÛË fiÛÔ Î·È ÙÔÓ ÔÏÏ·Ï·ÛÈ·ÛÌfi ÙˆÓ ÌÂÛÂÁ¯˘Ì·ÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ. ¶·ÚfiÌÔÈ· Ê·›ÓÂÙ·È Â›Û˘ Ó· Â›Ó·È Ë ‰Ú¿ÛË ÙÔ˘ ÛËÌ·ÙÔ‰ÔÙÈÎÔ‡ ÌÔÚ›Ô˘ Fibroblast growth factor (FGF)-2 (28).
¢È·ÎÏ¿‰ˆÛË (branching) Ù˘ Ô˘ÚËÙËÚÈ΋˜ ηٷ‚ÔÏ‹˜
∆Ô Î·ıÔÚÈṲ̂ÓÔ ÌÂÛ¤Á¯˘Ì· ·Ú¿ÁÂÈ ÌÈ· ÛÂÈÚ¿ ·fi ÛËÌ·ÙÔ‰ÔÙÈο ÌfiÚÈ·, Ù· ÔÔ›· ‰ÚÔ˘Ó ÛÙËÓ √∫ Î·È Â¿ÁÔ˘Ó ÌÈ· ÛÙÂÚÂfiÙ˘Ë ·ÎÔÏÔ˘ı›· ÂÈÌË·ÓÛÂˆÓ Î·È ‰È·ÎÏ·‰ÒÛÂˆÓ ÛÙÔ ÂÈı‹ÏÈÔ, ÔÈ Ôԛ˜ Ô‰ËÁÔ‡Ó ÛÙË ‰ËÌÈÔ˘ÚÁ›· ÙÔ˘ Û˘ÛÙ‹Ì·ÙÔ˜ ÙˆÓ ·ıÚÔÈÛÙÈÎÒÓ ÛˆÏËÓ·Ú›ˆÓ. ¶ÈÛÙ‡ÂÙ·È fiÙÈ ÙÔ ÚfiÙ˘Ô Ù˘ ‰È·ÎÏ¿‰ˆÛ˘ ÙÔ˘ ÂÈıËÏ›Ô˘ Ù˘ √∫ ηıÔÚ›˙ÂÙ·È ·fi ÙÔÓ Û˘Ó‰˘·ÛÌfi ÛËÌ¿ÙˆÓ Ù· ÔÔ›· ÚÔηÏÔ‡Ó Â›Ù ÙËÓ ·ÔÈÎÔ‰fiÌËÛË, ›Ù ÙË ÛÙ·ıÂÚÔÔ›ËÛË Ù˘ ÂÍˆÎ˘ÙÙ¿ÚÈ·˜ ‡Ï˘ Á‡Úˆ ·fi ÙÔ ÂÈı‹ÏÈÔ Î·È ·ÓÙ›ÛÙÔȯ· ÚÔ¿ÁÔ˘Ó ‹ ÂÌÔ‰›˙Ô˘Ó ÙËÓ ‰È·ÎÏ¿‰ˆÛ‹ ÙÔ˘.
√ ·Ú¿ÁÔÓÙ·˜ GDNF, Ô ÔÔ›Ô˜, fiˆ˜ ÚԷӷʤÚıËÎÂ, Â›Ó·È ··Ú·›ÙËÙÔ˜ ÁÈ· ÙËÓ Â·ÁˆÁ‹ Ù˘ √∫ ·fi ÙÔ ÌÂÛÔÓÂÊÚÈÎfi fiÚÔ, ÂÍ·ÎÔÏÔ˘ı› Ó· Â›Ó·È ·Ó·Áη›Ô˜ Î·È ÛÙË ‰È·‰Èηۛ· Ù˘ ‰È·ÎÏ¿‰ˆÛ˘ ÙÔ˘ ÂÈıËÏ›Ô˘. ∞˘Ùfi ÚÔ·ÙÂÈ ·fi ÂÈÚ¿Ì·Ù· Û ÈÛÙÔηÏÏȤÚÁÂȘ ÓÂÊÚÒÓ ÛÙ· ÔÔ›· ηٷÛÙ¤ÏÏÔÓÙ·˜ ÙË ‰Ú¿ÛË Ù˘ GDNF ·ÚÂÌÔ‰›˙ÂÙ·È Ë ‰È·ÎÏ¿‰ˆÛË, ÂÓÒ ·ÓÙÈı¤Ùˆ˜ ÚÔ¿ÁÂÙ·È Ì ÙËÓ ÚÔÛı‹ÎË Â͈ÁÂÓÔ‡˜ GDNF (29,30). ÀÔÏ·Û›· ÙˆÓ ÓÂÊÚÒÓ ·Ú·ÙËÚÂ›Ù·È ÛÂ Û˘Óı‹Î˜ ¤ÏÏÂȄ˘ Ù˘ ÂÎÎÚÈÓfiÌÂÓ˘ ÚˆÙ½Ó˘ Wnt11, ·ÔηχÙÔÓÙ·˜ ÙÔÓ ÚfiÏÔ ÙÔ˘ ·Ú·¿Óˆ ·Ú¿ÁÔÓÙ· ÛÙË ‰È·‰Èηۛ· Ù˘
‰È·ÎÏ¿‰ˆÛ˘ ÙÔ˘ ÂÈıËÏ›Ô˘. ∆Ô ÁÔÓ›‰ÈÔ Wnt11
ÂÎÊÚ¿˙ÂÙ·È ÛÙËÓ ÎÔÚ˘Ê‹ Ù˘ √∫ Î·È Ë ·ÂÓÂÚ-
ÁÔÔ›ËÛ‹ ÙÔ˘ ÚÔηÏ› Ì›ˆÛË ÙˆÓ ÂȤ‰ˆÓ
¤ÎÊÚ·Û˘ ÙÔ˘ GDNF (31).
∂ӉȷʤÚÔ˘Û˜ ÂӉ›ÍÂȘ Û¯ÂÙÈο Ì ÙÔ˘˜
Ì˯·ÓÈÛÌÔ‡˜ Ú‡ıÌÈÛ˘ ‰È·ÊfiÚˆÓ ·Ú·ÁfiÓÙˆÓ
ÌÂÙ¿ ÙËÓ Â·ÁˆÁ‹ Ù˘ √∫ ¤¯Ô˘Ó ÚÔ·„ÂÈ
·fi ÙË ÛÙÔ¯Â˘Ì¤ÓË ·ÂÓÂÚÁÔÔ›ËÛË ÙÔ˘ ÁÔÓÈ-
‰›Ô˘ Emx2 ÛÙÔÓ ÔÓÙÈÎfi, ÔÌfiÏÔÁÔ˘ ÙÔ˘ ÔÌÔÈÔÙÈÎÔ‡ ÁÔÓȉ›Ô˘ empty spiracles Ù˘ ‰ÚÔÛfiÊÈÏ·˜.
™˘ÁÎÂÎÚÈ̤ӷ, ˆ˜ ·ÔÙ¤ÏÂÛÌ· Ù˘ ¤ÏÏÂȄ˘
Ù˘ Emx2 ÛÙÔÓ ÔÓÙÈÎfi Ë √∫ Û¯ËÌ·Ù›˙ÂÙ·È Î·-
ÓÔÓÈο ·ÏÏ¿ ‰ÂÓ ‰È·ÎÏ·‰›˙ÂÙ·È Î·ıfiÏÔ˘, ÌÂ
·ÔÙ¤ÏÂÛÌ· ÙËÓ ÂÓÂÚÁÔÔ›ËÛË Ì˯·ÓÈÛÌÒÓ
·fiÙˆÛ˘ ÛÙÔ ÌÂÛ¤Á¯˘Ì·. √È ÌÔÚȷΤ˜ ‰È·-
Ù·Ú·¯¤˜ Ô˘ Ô‰ËÁÔ‡Ó ÛÙÔÓ ·Ú·¿Óˆ Ê·ÈÓfi-
Ù˘Ô ÈÛÙ‡ÂÙ·È fiÙÈ Â›Ó·È Ë ‰Ú·ÛÙÈ΋ Ì›ˆÛË
ÙˆÓ ·Ú¯Èο Ê˘ÛÈÔÏÔÁÈÎÒÓ ÂȤ‰ˆÓ ¤ÎÊÚ·Û˘ ÙˆÓ ÁÔÓȉ›ˆÓ Pax2, GDNF Î·È Ret (32). ∂ÈϤÔÓ ÛËÌ·ÙÔ‰ÔÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ Ô˘
ÂËÚ¿˙Ô˘Ó ÙË ‰È·‰Èηۛ· Ù˘ ‰È·ÎÏ¿‰ˆÛ˘
Ù˘ √∫ Â›Ó·È Ô HGF (hepatocyte growth factor), Ô ÔÔ›Ô˜ ÙËÓ ÚÔ¿ÁÂÈ (33), ÂÓÒ ¿ÏÏ·
ÌfiÚÈ· fiˆ˜ Ë BMP-2 (34), BMP-4 (26) ηÈ
TGF‚ (35) ‰ÚÔ˘Ó Î·Ù·ÛÙ·ÏÙÈο ÛÙËÓ ·Ú·¿-
Óˆ ‰È·‰Èηۛ·. ∂ӉȷʤÚÔÓ ·ÚÔ˘ÛÈ¿˙ÂÈ Ô ·-
Ú¿ÁÔÓÙ·˜ BMP-7, Ô ÔÔ›Ô˜ ÂÎÎÚ›ÓÂÙ·È Û ̤-
ÙÚȘ ÔÛfiÙËÙ˜ ·fi ÙÔ ÌÂÛ¤Á¯˘Ì· Î·È Û ÌÂÁ·Ï‡ÙÂÚ˜ ·fi ÙÔ ‰È·ÎÏ·‰È˙fiÌÂÓÔ ÂÈı‹ÏÈÔ.
÷ÌËÏ¿ ›‰· BMP-7 Ê·›ÓÂÙ·È Ó· ÚÔ¿ÁÔ˘Ó ÙË ‰È·ÎÏ¿‰ˆÛË Ù˘ √∫, ÂÓÒ ˘„ËÏfiÙÂÚ· ÙËÓ ÂÌÔ‰›˙Ô˘Ó (34). √ Û˘ÁÎÂÎÚÈ̤ÓÔ˜ Ì˯·-
ÓÈÛÌfi˜ ηıÒ˜ Î·È Ô Û˘Ó‰˘·ÛÌfi˜ ıÂÙÈ΋˜ Î·È ·ÚÓËÙÈ΋˜ ÛËÌ·ÙÔ‰fiÙËÛ˘ ÁÂÓÈÎfiÙÂÚ·, ÂÈÙ˘Á¯¿ÓÂÈ ÙËÓ Î·Ù·ÛÙÔÏ‹ ‹ ÙËÓ ÂÓ›Û¯˘ÛË Ù˘
‰ËÌÈÔ˘ÚÁ›·˜ Ó¤ˆÓ ‰È·ÎÏ·‰ÒÛÂˆÓ Û ÂÚÈÔ¯¤˜
ÙÔ˘ ÌÂÛÂÁ¯‡Ì·ÙÔ˜ ÏÔ‡ÛȘ ‹ ÊÙˆ¯¤˜ Û ÂÈıËÏȷΤ˜ ‰È·ÎÏ·‰ÒÛÂȘ ·ÓÙ›ÛÙÔȯ·. ŒÙÛÈ, ÂÍ·ÛÊ·Ï›˙ÂÙ·È Ë ÔÌÔÈfiÌÔÚÊË Î·Ù·ÓÔÌ‹ ÙˆÓ ‰È·ÎÏ·‰ÒÛÂˆÓ Ù˘ √∫ Û fiÏË ÙËÓ ¤ÎÙ·ÛË ÙÔ˘ ÌÂÙ·ÓÂÊÚÈÎÔ‡ ÌÂÛÂÁ¯‡Ì·ÙÔ˜.
¢ËÌÈÔ˘ÚÁ›· ÙˆÓ ÓÂÊÚÒÓˆÓ ∏ ‰È·‰Èηۛ· Ù˘ ‰È·ÊÔÚÔÔ›ËÛ˘ ÙÔ˘ ÌÂ-
ÛÂÁ¯‡Ì·ÙÔ˜ Î·È Ù˘ ‰ËÌÈÔ˘ÚÁ›·˜ ÙˆÓ ÓÂÊÚÒÓˆÓ ·Ú¯›˙ÂÈ Ì ÙËÓ ·Ú·ÁˆÁ‹ ÛËÌ·ÙÔ‰ÔÙÈÎÒÓ ÌÔÚ›ˆÓ ·fi ÙÔ ÂÈı‹ÏÈÔ Ù˘ √∫. ∞˘Ù¿ ‰ÚÔ˘Ó
·Ú¿ÁÔÓÙ˜, Ô˘ ÂÎÊÚ¿˙ÔÓÙ·È ÛÙÔ ÌÂÛ¤Á¯˘Ì·
Û ·fiÎÚÈÛË ÛÙ· Û‹Ì·Ù· ÙÔ˘ ÂÈıËÏ›Ô˘, Â›Ó·È Ô Wilms tumor (WT)-1, Ô ÔÔ›Ô˜ ·Ó‹ÎÂÈ ÛÙËÓ ÔÈÎÔÁ¤ÓÂÈ· ÙˆÓ Zn-finger ÚˆÙÂ˚ÓÒÓ. Èڛ˜ ÙËÓ
WT-1 ÙÔ ÌÂÛ¤Á¯˘Ì· ·‰˘Ó·Ù› Ó· ‰È·ÊÔÚÔÔÈËı› Û ÓÂÊÚÒÓ˜, Ì ·ÔÙ¤ÏÂÛÌ· Ó· ·ÎÔÏÔ˘ı› ‰È·‰Èηۛ˜ ÚÔÁÚ·ÌÌ·ÙÈṲ̂ÓÔ˘ ΢ÙÙ·ÚÈÎÔ‡ ı·Ó¿ÙÔ˘ (36). ™ÙË ıÂÙÈ΋ Ú‡ıÌÈÛË ÙÔ˘ WT-1
Ê·›ÓÂÙ·È Ó· ÂÌϤÎÂÙ·È Ô ·Ú¿ÁÔÓÙ·˜ FGF-2, Ô
ÔÔ›Ô˜ ·Ú¿ÁÂÙ·È ·fi Ù· ÂÈıËÏȷο ·ÙÙ·Ú·
Ù˘ √∫ (37).
ŒÂÈÙ· ·fi ÙËÓ Â·ÁˆÁ‹ ÙÔ˘ WT-1, Ù· ÌÂ-
ÛÂÁ¯˘Ì·ÙÈο ·ÙÙ·Ú· ·Ú¯›˙Ô˘Ó Ó· ˘ÎÓÒÓÔ˘Ó Î·È Ó· ‰ËÌÈÔ˘ÚÁÔ‡Ó Û˘Ì·Á‹ ÛÊ·ÈÚÈο Û˘Ûۈ̷ÙÒÌ·Ù· Á‡Úˆ ·fi ÙȘ ÎÔÚ˘Ê¤˜ Ù˘ √∫. ∞ÎÔ-
ÁÈ· ÙË ÓÂÊÚÔ-
΢Ù-
(mesenchyme-toepithelial transition). ∞fi ÂÈÚ¿Ì·Ù· Û ΢ÙÙ·-
‰È·ÈÛÙÒÓÂÙ·È Ô ÛËÌ·ÓÙÈÎfi˜ ÚfiÏÔ˜ ÙˆÓ ÂÎÎÚÈÓfiÌÂÓˆÓ ·Ú·ÁfiÓÙˆÓ FGF-2 Î·È LIF (Leukemia inhibitory factor) ÛÙË ‰È·‰Èηۛ· ·˘Ù‹. ∂ÓÒ Ë FGF-2 ÌÔÚ› Ó· ÚÔηϤÛÂÈ ÙË Û˘Ì‡ÎÓˆÛË ÙˆÓ ÌÂÛÂÁ¯˘Ì·ÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ, ‰ÂÓ ¤¯ÂÈ ÙËÓ ÈηÓfiÙËÙ· Ó· Ù· ÌÂÙ·ÙÚ¤„ÂÈ Û ÂÈıËÏȷο (38). ∞˘Ùfi ÙÔ ÂÈÙ˘Á¯¿ÓÂÈ Ë LIF, ·ÏÏ¿ ¤ÂÈÙ· ·fi ÙË ‰Ú¿ÛË Ù˘ FGF-2 (39). ™ËÌ·ÓÙÈÎfi ÚfiÏÔ ÛÙË ‰ËÌÈÔ˘ÚÁ›· ÙˆÓ ÓÂÊÚÒÓˆÓ Ê·›ÓÂÙ·È Ó· ·›˙ÂÈ ÙÔ ÁÔÓ›‰ÈÔ Pax2, ÂÊfiÛÔÓ Ë ·ÂÓÂÚÁÔÔ›ËÛË ÙÔ˘ ÛÂ Û˘ÛÙ‹Ì·Ù· in vitro ηÏÏȤÚÁÂÈ·˜ ÓÂÊÚÒÓ ÂÌÔ‰›˙ÂÈ ÙËÓ
Û˘Ûۈ̿وÛË ÙˆÓ ÌÂÛÂÁ¯˘Ì·ÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ
Î·È ÙËÓ ÌÂÙ·ÙÚÔ‹ ÙÔ˘˜ Û ÂÈıËÏȷο (40).
¶·ÚfiÌÔÈÔ˜ Ê·ÈÓfiÙ˘Ô˜ ÚÔ·ÙÂÈ ·fi ÙËÓ ¤Ï-
ÏÂÈ„Ë ÙÔ˘ ÁÔÓȉ›Ô˘ Wnt-4, ÙÔ ÔÔ›Ô Îˆ‰ÈÎÔ-
ÔÈ› ÌÈ· ÂÎÎÚÈÓfiÌÂÓË ÁÏ˘ÎÔÚˆÙ½ÓË, Ë ÔÔ›·
·Ú¿ÁÂÙ·È ·fi Ù· ÌÂÛÂÁ¯˘Ì·ÙÈο ·ÙÙ·Ú· η-
Ù¿ ÙË ‰È·‰Èηۛ· Ù˘ Û˘Ì‡ÎÓˆÛ‹˜ ÙÔ˘˜ Á‡Úˆ ·fi ÙËÓ ÎÔÚ˘Ê‹ Ù˘ √∫ (41). ∏ Wnt-4 ¿ÁÂÈ ÙËÓ ¤ÎÊÚ·ÛË ÙÔ˘ ›‰ÈÔ˘ ÙÔ˘ ÁÔÓȉ›Ô˘ Ù˘. ∞˘Ùfi˜
fiÙÈ Â›Ó·È ¤Ó·˜ Ì˯·ÓÈÛÌfi˜ Ì ÙÔÓ
Ù· ÌÂÛÂÁ¯˘Ì·ÙÈο ·ÙÙ·Ú· “ÌÂÙÚÔ‡Ó” ÙËÓ ˘ÎÓfiÙËÙ¿ ÙÔ˘˜ (42). ∞Ó ‰ËÏ·‰‹ Û˘Ì˘Îӈ̤ӷ ·ÙÙ·Ú· ÙÔ˘ ÌÂÛÂÁ¯‡Ì·ÙÔ˜ ‚ÚÂıÔ‡Ó
Wnt-4, ÒÛÙ ӷ ‰ËÌÈÔ˘ÚÁ‹ÛÔ˘Ó
ÂÚ›ÙˆÛË Ù˘ ÂÈıËÏÈÔÔ›ËÛ˘ fiÏˆÓ ÙˆÓ
΢ÙÙ¿ÚˆÓ ÙÔ˘ ÌÂÛÂÁ¯‡Ì·ÙÔ˜. ªÈ· Ù¤ÙÔÈ· ·ÚÓË-
ÙÈ΋ ‰Ú¿ÛË Ê·›ÓÂÙ·È Ó· ¤¯ÂÈ Ë ÂÎÎÚÈÓfiÌÂÓË
ÚˆÙ½ÓË sFRP-1, Ë ÔÔ›· ·Ó‹ÎÂÈ ÛÙËÓ ÔÈÎÔÁ¤-
ÓÂÈ· ÙˆÓ Wnt ˘Ô‰Ô¯¤ˆÓ Î·È ·ÓÙ·ÁˆÓ›˙ÂÙ·È
ÙËÓ ÚfiÛ‰ÂÛË Ù˘ Wnt-4 ÛÙÔÓ ˘Ô‰Ô¯¤·
Frizzled (43). ŒÓ·˜ ‰Â‡ÙÂÚÔ˜ ˘Ô‰Ô¯¤·˜ Ù˘
›‰È·˜ ÔÈÎÔÁ¤ÓÂÈ·˜, Ô sFRP-2, ·Ú¯›˙ÂÈ Ó· ·Ú¿ÁÂ-
Ù·È ·fi Ù· ÌÂÛÂÁ¯˘Ì·ÙÈο ·ÙÙ·Ú· fiÙ·Ó Û ·˘-
Ù¿ ·Ú¯›˙ÂÈ ÛÙ·‰È·Î¿ Ó· ÂÍ·ÛıÂÓ› Ë ¤ÎÊÚ·ÛË
ÙÔ˘ Wnt-4. ªÂ ·˘Ùfi ÙÔÓ ÙÚfiÔ ÂÈÙ˘Á¯¿ÓÂÙ·È
Ë ‰¤ÛÌ¢ÛË Ù˘ Wnt-4 ·fi Ù· ›‰È· Ù· ‰È·ÊÔÚÔ-
ÔÈË̤ӷ ÌÂÛÂÁ¯˘Ì·ÙÈο ·ÙÙ·Ú·, ·ÓÂÍ¿ÚÙËÙ·
·fi ÙËÓ sFRP-1, Ë ÔÔ›· ËÁ¿˙ÂÈ ·fi ÙÔ ·‰È·-
ÊÔÚÔÔ›ËÙÔ ÌÂÛ¤Á¯˘Ì·. √ Ì˯·ÓÈÛÌfi˜ ·˘Ùfi˜
ÏÂÈÙÔ˘ÚÁ› Û·Ó ·ÛÊ·ÏÈÛÙÈ΋ ‰ÈÎÏ›‰· ÒÛÙ ӷ
ÂÈÙ¢¯ı› Ô Î·ıÔÚÈÛÌfi˜ Ù˘ Ù‡¯Ë˜ ÙˆÓ Û˘-
Ì˘ÎÓˆÌ¤ÓˆÓ ÌÂÛÂÁ¯˘Ì·ÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ, ‰ËÏ·‰‹ Ë ÂÈıËÏÈÔÔ›ËÛ‹ ÙÔ˘˜. √ ÌÔÚÊÔÁÂÓÂÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ FGF8 ÂÎÊÚ¿˙ÂÙ·È ÛÙÔÓ ·Ó·Ù˘ÛÛfiÌÂÓÔ ÓÂÊÚfi, ·Ú¯Èο
ÛÙ· Û˘Ì˘Îӈ̤ӷ ÌÂÛÂÁ¯˘Ì·ÙÈο ·ÙÙ·Ú· Î·È ÛÙ·
·Ú¿ÁˆÁ· ÙÔ˘˜, Ù· ÂÈı‹ÏÈ· ÙˆÓ ÓÂÊÚÒÓˆÓ. ∏
ÂÌ‚Ú˘˚΋ ıÓËÛÈÌfiÙËÙ· Ô˘ ·Ú·ÙËÚÂ›Ù·È ÛÙÔ
ÛÙ¿‰ÈÔ Ù˘ Á·ÛÙÚȉ›ˆÛ˘ ÛÙ· FGF8-/- ÔÓÙ›ÎÈ·
‰ÂÓ Â¤ÙÚ ÙËÓ ÌÂϤÙË ÙÔ˘ ÚfiÏÔ˘ ÙÔ˘ FGF8
ÛÙËÓ ·Ó¿Ù˘ÍË ÙˆÓ ÓÂÊÚÒÓ (44). øÛÙfiÛÔ, ·˘Ù‹
η٤ÛÙË ÂÊÈÎÙ‹ Ì ÙËÓ ÈÛÙÔÂȉÈ΋ ·ÂÓÂÚÁÔÔ›-
ËÛË ÙÔ˘ FGF8 Û ÌÂÛÂÁ¯˘Ì·ÙÈÎÔ‡˜ ΢ÙÙ·ÚÈÎÔ‡˜
Ù‡Ô˘˜, Ë ÔÔ›· Ô‰‹ÁËÛ Û ˘ÔÏ·Û›· ÙˆÓ ÓÂ-
ÊÚÒÓ ÏfiÁˆ ÚÔ‚ÏËÌ·ÙÈ΋˜ ‰È·ÊÔÚÔÔ›ËÛ˘
ÙÔ˘ ÂÈıËÏ›Ô˘ ÙˆÓ ÓÂÊÚÒÓˆÓ Î·È ÛËÌ·ÓÙÈο
ÌÂȈ̤Ó˘ ‰È·ÎÏ¿‰ˆÛ˘ Ù˘ √∫ (45).
∏ ÌÂÙ·ÙÚÔ‹ ÙˆÓ ÌÂÛÂÁ¯˘Ì·ÙÈÎÒÓ Î˘ÙÙ¿-
ÚˆÓ Û ÂÈıËÏȷο Û˘Óԉ‡ÂÙ·È ·fi ÌÈ· ÛÂÈÚ¿ ·ÏÏ·ÁÒÓ ÛÙËÓ ¤ÎÊÚ·ÛË ÁÔÓȉ›ˆÓ. ™˘ÁÎÂÎÚÈ̤ӷ, Ù· Û˘Ì˘Îӈ̤ӷ ·ÙÙ·Ú· ·Ú¯›˙Ô˘Ó Ó· ÂÎÊÚ¿˙Ô˘Ó ÁÔÓ›‰È· ¯·Ú·ÎÙËÚÈÛÙÈο ÙˆÓ ÂÈıËÏÈ·ÎÒÓ Î˘ÙÙ¿ÚˆÓ, fiˆ˜ ÂÈıËÏÈ·ÎÔ‡ Ù‡Ô˘ ηÓÙÂÚ›Ó˜ Î·È ÚˆÙ½Ó˜-Û˘ÛÙ·ÙÈο ÙˆÓ ÛÙÂÓÔÛ˘Ó‰¤Û̈Ó, ÂÓÒ ·Ú¿ÏÏËÏ· ·ÓÙÈηıÈÛÙÔ‡Ó Ù· ÌfiÚÈ· ‚ÈÌÂÓÙ›Ó˘ ÙÔ˘ ΢ÙÙ·ÚÔÛÎÂÏÂÙÔ‡ ÙÔ˘˜ ÌÂ
΢ÙÔÎÂÚ·Ù›Ó˜ (46-48). ∆¤ÏÔ˜, fiˆ˜ Û fiϘ ÙȘ ÂÈıËÏȷΤ˜ ‰Ô̤˜ ·Ú·ÙËÚÂ›Ù·È Ë ‰ËÌÈÔ˘ÚÁ›· ÌÈ·˜ ηÓÔÓÈ΋˜ ‚·ÛÈ΋˜ ÌÂÌ‚Ú¿Ó˘ (49,50).
∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ˆÚ›Ì·ÓÛ˘, Ù· ·Ú¯Èο ÛÊ·ÈÚÈο ÂÈı‹ÏÈ· ÙˆÓ ÓÂÊÚÒÓˆÓ ÌÂÙ·‚¿ÏÏÔÓÙ·È Û ÛÈÁÌÔÂȉ‹ ÛˆÏËÓ¿ÚÈ·. ™ÙÔ ¿ÎÚÔ ÙˆÓ ÛˆÏËÓ·Ú›ˆÓ, fiÔ˘ ı· ‰ËÌÈÔ˘ÚÁËıÔ‡Ó ·ÚÁfiÙÂÚ· Ù· ÓÂÊÚÈο ÛÂÈÚ¿Ì·Ù·, ˘¿Ú¯ÂÈ ·˘ÍË̤ÓË ÂÓÂÚÁfiÙËÙ· ÙÔ˘ ·Ú¿ÁÔÓÙ· WT-1. H ¤ÎÊÚ·ÛË ÙÔ˘ WT-1 ÛÙËÓ ÂÚÈÔ¯‹ ·˘Ù‹ ηٷÛÙ¤ÏÏÂÈ ÙËÓ ¤ÎÊÚ·ÛË ÙÔ˘ Pax2. ∞˘Ùfi Ê·›ÓÂÙ·È Ó· ·ÔÙÂÏ› ¤Ó·Ó Ôχ ÛËÌ·ÓÙÈÎfi Ú˘ıÌÈÛÙÈÎfi Ì˯·ÓÈÛÌfi, ηıÒ˜ Û ÂÈÚ¿Ì·Ù· ¤ÎÙÔ˘ ¤ÎÊÚ·Û˘ ÙÔ˘ Pax-2 Û fiÏÔ ÙÔ ÙÌ‹Ì· ÙÔ˘ ÂÈıËÏ›Ô˘ ÙˆÓ ÓÂÊÚÒÓˆÓ ÂÌÔ‰›˙ÂÙ·È Ë ‰ËÌÈÔ˘ÚÁ›· ÙˆÓ ÓÂÊÚÈÎÒÓ ÛÂÈÚ·Ì¿ÙˆÓ (51).
∞ÍÈÔÛËÌ›ˆÙË Â›Ó·È Ë Û˘ÌÌÂÙÔ¯‹ ÙˆÓ ·Ú·ÁfiÓÙˆÓ Pax-2 Î·È WT-1 Û ÔÏÏ·Ï¿ ÛÙ¿‰È· Ù˘ ·Ó¿Ù˘Í˘ ÙˆÓ ÓÂÊÚÒÓ. ª¿ÏÈÛÙ·, ·ÎfiÌ· Î·È ÔÈ ‰È·ÊÔÚÂÙÈΤ˜ ÈÛÔÌÔÚʤ˜ Ù˘ WT-1 ¤¯Ô˘Ó Û˘Û¯ÂÙÈÛı› ̠ͯˆÚÈÛÙ¤˜ ÏÂÈÙÔ˘ÚÁ›Â˜ ÛÙËÓ ˆÚ›Ì·ÓÛË ÙˆÓ ÌÂÙ¿ÓÂÊÚˆÓ. ™˘ÁÎÂÎÚÈ̤ӷ, Û ÁÂÓÂÙÈο ÙÚÔÔÔÈË̤ӷ ÔÓÙ›ÎÈ· Ô˘ ÂÎÊÚ¿˙Ô˘Ó ÌfiÓÔ ÙËÓ ÈÛÔÌÔÚÊ‹ -KTS ‰ËÌÈÔ˘ÚÁÔ‡ÓÙ·È ÓÂÊÚÈο ÛÂÈÚ¿Ì·Ù· ÛÙ· ÔÔ›· Ù· Ô‰Ô·ÙÙ·Ú· ‰ÂÓ Û¯ËÌ·Ù›˙Ô˘Ó ÙÔ˘˜ Ù˘ÈÎÔ‡˜ Ô‰›ÛÎÔ˘˜, ÂÓÒ Ë ·ÔÎÏÂÈÛÙÈ΋ ¤ÎÊÚ·ÛË Ù˘ +KTS ÈÛÔÌÔÚÊ‹˜ Ô‰ËÁ› ÛÙËÓ ‰ËÌÈÔ˘ÚÁ›· ÂÏ¿¯ÈÛÙˆÓ Î·È ·ÓÒÚÈÌˆÓ ÓÂÊÚÈÎÒÓ ÛÂÈÚ·Ì¿ÙˆÓ (52). √ ÌÂÙ·ÁÚ·ÊÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ Brn-1 ÂÎÊÚ¿˙ÂÙ·È Î˘Ú›ˆ˜ ÛÙÔ Ó¢ÚÈÎfi Û‡ÛÙËÌ· Î·È Ë ·ÂÓÂÚÁÔÔ›ËÛË ÙÔ˘ ÚÔηÏ› ‹È˜ ·ÏÏÔÈÒÛÂȘ Û ‰Ô̤˜ ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘, ÁÂÁÔÓfi˜ Ô˘ ‰ÂÓ ‰ÈηÈÔÏÔÁ› ÙÔ ı¿Ó·ÙÔ ÙˆÓ ÌÂÙ·ÏÏ·ÁÌ¤ÓˆÓ ÔÓÙÈÎÒÓ Ì¤Û· ÛÙȘ ÚÒÙ˜ 48 ÒÚ˜ ÌÂÙ¿ ÙË Á¤ÓÓËÛË. ∞fi ÙËÓ ÚfiÛÊ·ÙË ÏÂÙÔÌÂÚ‹ ÂͤٷÛË ÙˆÓ ÓÂÊÚÒÓ ÙˆÓ Brn-1-/- ÔÓÙÈÎÒÓ ÚԤ΢„Â Ô ÛËÌ·ÓÙÈÎfi˜ ÚfiÏÔ˜ ÙÔ˘ ÂÓ ÏfiÁˆ ÁÔÓȉ›Ô˘ ÛÙË ‰È·ÊÔÚÔÔ›ËÛË ÙˆÓ ¿ˆ ÛˆÏËÓ·Ú›ˆÓ ÙˆÓ ÓÂÊÚÒÓˆÓ. ™˘ÁÎÂÎÚÈ̤ӷ, ·Ú·ÙËÚÂ›Ù·È fiÙÈ Ë ÂÈÌ‹Î˘ÓÛË Ù˘ ·Á·Ï˘ ÙÔ˘ Henle ‰ÂÓ ÂÂÎÙ›ÓÂÙ·È ÚÔ˜ Ù· ÂÛÒÙÂÚ· ÙÌ‹Ì·Ù· ÙÔ˘ ÓÂÊÚÈÎÔ‡ ·ÚÂÁ¯‡Ì·ÙÔ˜ (medulla), ·ÏÏ¿ ÛÙ·Ì·Ù¿ ÚfiˆÚ· (53). ÕÏÏÔÈ ·Ú¿ÁÔÓÙ˜ Ô˘ ÂÌϤÎÔÓÙ·È ÛÙËÓ ˆÚ›Ì·ÓÛË ÙˆÓ ÓÂÊÚÒÓˆÓ Â›Ó·È Ô Lmx-1b, Ë ·Ô˘Û›· ÙÔ˘ ÔÔ›Ô˘ ÚÔηÏ› ‰È·Ù·Ú·¯¤˜ ÛÙÔ Ê˘ÛÈÔÏÔÁÈÎfi Û¯ËÌ·ÙÈÛÌfi Ù˘ ‚·ÛÈ΋˜ ÌÂÌ‚Ú¿Ó˘ ÙˆÓ ÛÂÈÚ·Ì¿ÙˆÓ (54), ÙÔ ÌÂÌ‚Ú·ÓÈÎfi ÛËÌ·ÙÔ‰ÔÙÈÎfi ÌfiÚÈÔ Notch-2 (55) ηıÒ˜ Î·È Ô ÌÂÙ·ÁÚ·ÊÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ Lim1 (8). ¶ÚfiÛÊ·Ù˜ ÌÂϤÙ˜ ·ÔÎ¿Ï˘„·Ó ÙÔÓ ÚfiÏÔ ÙˆÓ ÚÂÛÂÓÈÏÈÓÒÓ ÛÙËÓ ‰È·ÊÔÚÔÔ›ËÛË ÙÔ˘ ÂÈıËÏ›Ô˘ ÙˆÓ ÓÂÊÚÒÓˆÓ. √È ÚÂÛÂÓÈϛӘ Â›Ó·È ‰È·ÌÂÌ‚Ú·ÓÈΤ˜ ÚˆÙ½Ó˜, ÔÈ Ôԛ˜ Â›Ó·È ÁÓˆÛÙ¤˜ ÁÈ· ÙÔÓ ÚfiÏÔ ÙÔ˘˜ ÛÙËÓ
ÙÔ˘ ‚-·Ì˘ÏÔÂȉԇ˜ Î·È ÙÔ˘ ÛËÌ·ÙÔ‰ÔÙÈÎÔ‡ ÌÔ-
Ú›Ô˘ Notch, ÂÓÒ ÌÂÙ·ÏÏ¿ÍÂȘ ÛÙ· ÁÔÓ›‰È· Ô˘
Έ‰ÈÎÔÔÈÔ‡Ó ÙȘ ÚÂÛÂÓÈϛӘ Psn-1 Î·È Psn-2
¢ı‡ÓÔÓÙ·È ÁÈ· ÙËÓ ÂÌÊ¿ÓÈÛË Ù˘ ÓfiÛÔ˘ ÙÔ˘
Alzheimer (56). ∏ Psn-1 Î·È Psn-2 ¤¯Ô˘Ó Û˘ÌÏËڈ̷ÙÈΤ˜ ÏÂÈÙÔ˘ÚÁ›Â˜, ÂÓÒ ‰ÈÏ¿ ÔÌfi˙˘Á· ÔÓÙ›ÎÈ· (Psn-1/2-/-) Âı·›ÓÔ˘Ó ÚÈÓ ÙË ‰ËÌÈ-
Ô˘ÚÁ›· ÙˆÓ ÓÂÊÚÒÓ ·fi ÚÔ‚Ï‹Ì·Ù· ‰È·ÊÔÚÔ-
Ô›ËÛ˘ ÛÙÔ˘˜ ۈ̛Ù˜ (57). ∏ ¯Ú‹ÛË ÂÓfi˜ ‰È·-
ÁÔÓȉ›Ô˘ ÙÔ ÔÔ›Ô ÂÎÊÚ¿˙ÂÈ ÙÔ Psn-1 ÛÙȘ ÂË-
Ú·˙fiÌÂÓ˜ ·fi ÙÔÓ Ê·ÈÓfiÙ˘Ô ÂÚÈÔ¯¤˜ ¤-
ÙÚ„ ÙËÓ ÂÈ‚›ˆÛË ÙˆÓ Psn-1/2-/- ˙ÒˆÓ Ì¤¯ÚÈ ÙË Á¤ÓÓËÛË Î·È ÙËÓ ·Ó¿Ï˘ÛË ÙÔ˘ Ê·ÈÓÔÙ‡Ô˘ ÛÙ·
ÓÂÊÚ¿. ∞ԉ›¯ÙËΠfiÙÈ Ë ÓÂÊÚÔÁ¤ÓÂÛË ‰ÈÂÍ¿ÁÂÙ·È Ê˘ÛÈÔÏÔÁÈο ̤¯ÚÈ Î·È ÚÈÓ ÙÔ ÛÙ¿‰ÈÔ Ù˘
‰È·ÊÔÚÔÔ›ËÛ˘ ÙÔ˘ ÂÈıËÏ›Ô˘ ÙˆÓ ÓÂÊÚÒÓˆÓ
Û ÛÈÁÌÔÂȉ‹ ÛˆÏËÓ¿ÚÈ·, Ù· ÔÔ›· ‰ÂÓ ·ÓȯÓ‡-
ÔÓÙ·È ÛÙ· ‰ÈÏ¿ ÌÂÙ·ÏÏ·Á̤ӷ ˙Ò· (58). ∏ ·-
ÚÔ˘Û›· ÛˆÏËÓ·Ú›ˆÓ Û˘Ó‰Â‰ÂÌ¤ÓˆÓ Ì ٷ ·ıÚÔÈ-
ÛÙÈο ÛˆÏËÓ¿ÚÈ· ·ÏÏ¿ Ë ÌË ‰ËÌÈÔ˘ÚÁ›· ÂÁÁ‡˜
ÛˆÏËÓ·Ú›ˆÓ ·ÔηχÙÂÈ ÙÔÓ ÚfiÏÔ ÙˆÓ ÚÂÛÂÓÈÏÈÓÒÓ ÛÙË ÌÔÚÊÔÔ›ËÛË (patterning) ÙÔ˘ ÂÈ-
ıËÏÈÔÔÈË̤ÓÔ˘ ÌÂÛÂÁ¯‡Ì·ÙÔ˜.
¶Ôχ Ï›Á· Â›Ó·È Ì¤¯ÚÈ Û‹ÌÂÚ· ÁÓˆÛÙ¿ Û¯Â-
ÙÈο Ì ÙË ‰È·ÊÔÚÔÔ›ËÛË ÙˆÓ ˘fiÏÔȈÓ
ÙÌËÌ¿ÙˆÓ ÙˆÓ ÓÂÊÚÒÓˆÓ. ∏ ·ÂÓÂÚÁÔÔ›ËÛË
ÙÔ˘ ÌÂÙ·ÁÚ·ÊÈÎÔ‡ ·Ú¿ÁÔÓÙ· HNF-1, Ô ÔÔ›Ô˜ ÂÎÊÚ¿˙ÂÙ·È Û fiÏ· Ù· ÙÌ‹Ì·Ù· ÙˆÓ ÓÂÊÚÒ-
ÓˆÓ, ÂÎÙfi˜ ·fi Ù· ÛÂÈÚ¿Ì·Ù·, ·ÔÎ¿Ï˘„ ÙÔÓ
ÚfiÏÔ Ù˘ ·Ú·¿Óˆ ÚˆÙ½Ó˘ ÛÙË Ê˘ÛÈÔÏÔÁÈ΋ ÏÂÈÙÔ˘ÚÁ›· ÙˆÓ ÂÁÁ‡˜ ÛˆÏËÓ·Ú›ˆÓ. ™˘ÁÎÂÎÚÈ̤ӷ, Ë ¤ÏÏÂÈ„Ë Ù˘ HNF-1 ÚÔηÏ› ‰Ú·ÛÙÈ΋ Ì›ˆÛË Ù˘ ¤ÎÊÚ·Û˘ ÙÔ˘ ÁÔÓȉ›Ô˘ SGLT (Sodium glucose transporter)-2, Ì ·ÔÙ¤ÏÂÛÌ· Ó· ÚÔηÏÂ›Ù·È ÓÂÊÚÈÎfi Û‡Ó‰ÚÔÌÔ Fanconi Ô˘ ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÁÏ˘ÎÔ˙Ô˘Ú›· Î·È ·ÌÈÓÔÍÔ˘Ú›· (59).
∏ Û˘Ì‚ÔÏ‹ ÙÔ˘ ÛÙÚÒÌ·ÙÔ˜ ÛÙË ‰È·ÊÔÚÔÔ›ËÛË ÓÂÊÚÈÎÒÓ Î˘ÙÙ·ÚÈÎÒÓ Ù‡ˆÓ
∆Ô ÓÂÊÚÈÎfi ÛÙÚÒÌ· ÚÔ·ÙÂÈ ·fi ÙÔ ÌÂÛ¤Á¯˘Ì·, ÙÔ ÔÔ›Ô ‰ÂÓ ÂÈıËÏÈÔÔÈÂ›Ù·È Î·Ù¿ ÙË ‰È·‰Èηۛ· ÙÔ˘ Û¯ËÌ·ÙÈÛÌÔ‡ ÙˆÓ ÓÂÊÚÒÓˆÓ. ¶·ÚfiÏÔ Ô˘ ·Ú¯Èο ›¯Â ÚÔÙ·ı› ¤Ó·˜ ηı·Ú¿ ˘ÔÛÙËÚÈÎÙÈÎfi˜ ÚfiÏÔ˜ ÙÔ˘ ÛÙÚÒÌ·ÙÔ˜ ÛÙËÓ ·Ó¿Ù˘ÍË ÙˆÓ ÓÂÊÚÒÓ, ÂÓÙÔ‡ÙÔȘ, Ù· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ· ·ÔηχÊıËÎÂ Ë ÛËÌ·ÓÙÈ΋ Û˘Ì‚ÔÏ‹ ÙÔ˘ ÛÙË ‰È·ÊÔÚÔÔ›ËÛË ÓÂÊÚÈÎÒÓ Î˘ÙÙ·ÚÈÎÒÓ Ù‡ˆÓ. √ ÌÂÙ·ÁÚ·ÊÈÎfi˜
ÓÙ·˜ Foxd1, Ô˘ ·Ó‹ÎÂÈ ÛÙËÓ ÔÈÎÔÁ¤ÓÂÈ·
ÚˆÙÂ˚ÓÒÓ Forkhead, ÂÎÊÚ¿˙ÂÙ·È ¤ÓÙÔÓ· ÛÙÔ
ÙÔ˘ ÓÂÊÚÈÎÔ‡ ÊÏÔÈÔ‡. ∏ ·ÂÓÂÚÁÔÔ›ËÛË ÙÔ˘ Foxd1 ÚÔηÏ› ÌÈ· ÛÂÈÚ¿ ·fi ÚÔ‚Ï‹Ì·Ù· ÛÙÔ˘˜ ÓÂÊÚÔ‡˜, fiˆ˜ ÂÏϷو̷ÙÈ΋
‰È·ÎÏ¿‰ˆÛË Ù˘ √∫ Î·È ‰È·ÊÔÚÔÔ›ËÛË ÙˆÓ
ÓÂÊÚÒÓˆÓ. ∂ÈϤÔÓ, ÔÈ ÓÂÊÚÔ› ·ÚÔ˘ÛÈ¿˙Ô-
ÓÙ·È Û˘Á¯ˆÓÂ˘Ì¤ÓÔÈ Î·È ÂÓÙÔ›˙ÔÓÙ·È ¤ÎÙÔ·
ÛÙËÓ ÂÚÈÔ¯‹ Ù˘ ˘¤ÏÔ˘ (60). ∏ ·ÈÙ›· ÁÈ· ÙË
ÌË Ê˘ÛÈÔÏÔÁÈ΋ ı¤ÛË Î·È ÙË Û˘Á¯ÒÓ¢ÛË ÙˆÓ
ÓÂÊÚÒÓ ÛÙ· Foxd1-/- ÔÓÙ›ÎÈ· ·Ô‰Â›¯ÙËΠfiÙÈ
Â›Ó·È Ë ÂÏÏÈ‹˜ ‰È·ÊÔÚÔÔ›ËÛË Î˘ÙÙ·ÚÈÎÒÓ
Ù‡ˆÓ Ù˘ ÓÂÊÚÈ΋˜ ο„·˜ (61). ∆Ô ÁÂÁÔÓfi˜
fiÙÈ Ë ·Ó¿ÚÎÂÈ· ÁÔÓȉ›ˆÓ fiˆ˜ ÙÔ Foxd1 Ô˘
ÙˆÓ ÓÂÊÚÒ-
Î·È Û ·˘Ù¿ Ù· ÔÔ›· ı·
ÛÙÔ˘˜ ÓÂÊÚÒÓ˜. ™Ù· ‰È·ÊÔÚÔÔÈË̤ӷ ÓÂÊÚÈο ÛÂÈÚ¿Ì·Ù· ÙÔ Pod1 ÂÎÊÚ¿˙ÂÙ·È ÂȉÈο ÛÙ· Ô‰Ô·ÙÙ·Ú· (62). ™Â
Pod1-/- ÔÓÙ›ÎÈ· ÚÔηÏÂ›Ù·È ÌÂȈ̤ÓË ‰È·ÎÏ¿‰ˆÛË Ù˘ √∫, ηı˘ÛÙÂÚË̤ÓË ÂÈıËÏÈÔÔ›ËÛË ÙÔ˘ ÌÂÛÂÁ¯‡Ì·ÙÔ˜, ÂÓÒ Ù· Ô‰Ô·ÙÙ·Ú· ‰ÂÓ ‰È·ÊÔÚÔÔÈÔ‡ÓÙ·È. ∂ÈϤÔÓ, ‰ÂÓ ·ÓȯÓ‡ÔÓÙ·È Ï‹Úˆ˜ ‰È·ÊÔÚÔÔÈË̤ӷ ÓÂÊÚÈο ÛÂÈÚ¿Ì·Ù·, ÂÓÒ ·˘Ù¿ Ô˘ ‰ËÌÈÔ˘ÚÁÔ‡ÓÙ·È Â›Ó·È
ÌÂȈ̤ӷ Û ·ÚÈıÌfi (63). ∏ ¯Ú‹ÛË ÔÌfi˙˘ÁˆÓ
ÂÌ‚Ú˘˚ÎÒÓ ‚Ï·ÛÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ ÁÈ· ÙËÓ Î·Ù·Û΢‹ ¯ÈÌ·ÈÚÈÎÒÓ ÔÓÙÈÎÒÓ ·¤‰ÂÈÍ fiÙÈ Ë ·ÚÔ˘Û›· Ù˘ Pod1 ÛÙÔ ÛÙÚÒÌ· ·ÔηıÈÛÙ¿ ÙÔÓ ¿ÁÚÈÔ˘ Ù‡Ô˘ Ê·ÈÓfiÙ˘Ô ÛÙ· ÓÂÊÚÈο ÛÂÈÚ¿Ì·Ù·, οÙÈ ÙÔ ÔÔ›Ô Û˘ÓËÁÔÚ› ˘¤Ú Ù˘ ‡·Ú͢ ÛËÌÔÙÔ‰ÔÙÈÎÒÓ ÌÔÓÔ·ÙÈÒÓ, Ù· ÔÔ›· Ë-
Á¿˙Ô˘Ó ·fi ÙÔ ÓÂÊÚÈÎfi ÛÙÚÒÌ· (64).
ÙÔ˘ ÂÓ˙‡ÌÔ˘ heparan sulfate 2-sulfotransferase (HS2ST)
ÚÔηÏ› ÓÂÊÚÈ΋ ·ÁÂÓÂÛ›·, Ô˘ ÔÊ›ÏÂÙ·È ÛÂ
ÚÔ‚ÏËÌ·ÙÈ΋ Û˘Ì‡ÎÓˆÛË ÙˆÓ ÌÂÛÂÁ¯˘Ì·ÙÈ-
ÎÒÓ Î˘ÙÙ¿ÚˆÓ Î·È ‰È·ÎÏ¿‰ˆÛË Ù˘ √∫, ÂÓÒ Ù·
·Ú¯Èο ÛÙ¿‰È· Ù˘ ‰ËÌÈÔ˘ÚÁ›·˜ ÙÔ˘ ÌÂÙ¿ÓÂ-
ÊÚÔ˘, ‰ËÏ·‰‹ Ô Î·ıÔÚÈÛÌfi˜ ÙÔ˘ ÌÂÛÂÁ¯‡Ì·ÙÔ˜
Î·È Ë Â·ÁˆÁ‹ Ù˘ √∫ ‰ÈÂÍ¿ÁÔÓÙ·È Î·ÓÔÓÈο (65). ™Ô‚·ÚfiÙÂÚÔ˜ Â›Ó·È Ô Ê·ÈÓfiÙ˘Ô˜ Ô˘
ÚÔ·ÙÂÈ ·fi ÙËÓ ÛÙÔ¯Â˘Ì¤ÓË ·ÂÓÂÚÁÔÔ›ËÛË Ù˘ ÂÚÈÔ¯‹˜ ·ÏÏËÏ›‰Ú·Û˘ Ù˘ Ï·ÌÈÓ›Ó˘-Á1 Ì ÙÔ ÓȉÔÁfiÓÔ-1. ∏ ·Ô˘Û›· ·ÏÏËÏ›-
‰Ú·Û˘ ÌÂٷ͇ Ï·ÌÈÓ›Ó˘-Á1 Î·È ÓȉÔÁfiÓÔ˘-1
ÚÔηÏ› ‰È·Ù·Ú·¯¤˜ ÛÙËÓ Û˘ÁÎÚfiÙËÛË ÙˆÓ
‚·ÛÈÎÒÓ ÌÂÌ‚Ú·ÓÒÓ Î·È Ô‰ËÁ› ¤Ó· ÌÂÁ¿ÏÔ Ô-
ÛÔÛÙfi ÙˆÓ ·Ú·¿Óˆ ÌÂÙ·ÏÏ·ÁÌ¤ÓˆÓ ˙ÒˆÓ ÛÂ
ÓÂÊÚÈ΋ ·ÁÂÓÂÛ›· Ô˘ ÔÊ›ÏÂÙ·È ÛÙËÓ ÚÔ‚ÏË-
Ì·ÙÈ΋ ÂÈÌ‹Î˘ÓÛË ÙÔ˘ ÌÂÛÔÓÂÊÚÈÎÔ‡ fiÚÔ˘ ÚÈÓ ÙË ‰ËÌÈÔ˘ÚÁ›· Ù˘ √∫ (66). ¢È·Ù·Ú·¯¤˜ ÛÙËÓ Â·ÁˆÁ‹ Î·È ÙË ‰È·ÎÏ¿‰ˆÛË Ù˘ √∫ ÚÔηÏÔ‡ÓÙ·È ·fi ÙËÓ ¤ÏÏÂÈ„Ë Ù˘ ÈÓÙÂÁÎÚ›Ó˘-·8 (67). √È ÈÓÙÂÁÎÚ›Ó˜ Â›Ó·È ‰È·ÌÂÌ‚Ú·ÓÈΤ˜ ÚˆÙ½Ó˜ ÔÈ Ôԛ˜ Û˘Ó‰¤Ô˘Ó ÙË
̤ÏÔ˜ ÌÈ·˜ ηÈÓÔ‡ÚÁÈ·˜ ÔÈÎÔÁ¤ÓÂÈ·˜ ‰ÔÌÈο Û˘ÁÁÂÓÈÎÒÓ ÂÍˆÎ˘ÙÙ¿ÚÈˆÓ ÚˆÙÂ˚ÓÒÓ. ∂ӉȷʤÚÔÓ ·ÔÙÂÏ› ÙÔ ÁÂÁÔÓfi˜ fiÙÈ ÔÈ ÚˆÙ½Ó˜ ·˘Ù¤˜, Fras1 related extracellular matrix (Frem)-1 Î·È Frem-2 ÂÌϤÎÔÓÙ·È Î·È ÏÂÈÙÔ˘ÚÁÈο ÛÙȘ ›‰È˜ ‰È·‰Èηۛ˜, ·ÊÔ‡ Ë ·Ó¿ÚÎÂÈ· ÙÔ˘˜ ÚÔηÏ› ·ÚfiÌÔÈÔ ÂÌ‚Ú˘˚Îfi Ê·ÈÓfiÙ˘Ô. ∫·È ÛÙȘ ‰‡Ô ÂÚÈÙÒÛÂȘ ÚÔ·ÙÂÈ ·ÁÂÓÂÛ›· ÙˆÓ ÓÂÊÚÒÓ ÌÂÙ¿ ÙËÓ Â·ÁˆÁ‹ Ù˘ √∫ (72,73). ∞ÍÈÔÛËÌ›ˆÙÔ Â›Ó·È ÙÔ ÁÂÁÔÓfi˜ fiÙÈ ÙÌ‹Ì·Ù· ÙˆÓ ÚˆÙÂ˚ÓÒÓ Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜ Fras ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÔÌÔÈfiÙËÙ· Ì ÂÚÈÔ¯¤˜ ÔÈ Ôԛ˜ ·ÏÏËÏÂȉÚÔ‡Ó Ì ·˘ÍËÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜. ∂Ô̤ӈ˜, ÔÈ ÚˆÙ½Ó˜ Fras1 Î·È Frem1/2 Èı·ÓfiÓ Ó· Û˘ÌÌÂÙ¤¯Ô˘Ó ˆ˜ ‰ÔÌÈο ÛÙÔȯ›· ÛÙË Û˘ÁÎÚfiÙËÛË ÙˆÓ ‚·ÛÈÎÒÓ ÌÂÌ‚Ú·ÓÒÓ, ·ÏÏ¿ Î·È ˆ˜ Ú˘ıÌÈÛÙ¤˜ ÛËÌ·ÙÔ‰ÔÙ‹ÛÂˆÓ ÌÂٷ͇ ÂÈıËÏ›Ô˘ Î·È ÌÂÛÂÁ¯‡Ì·ÙÔ˜.
Ù·˘ÙÔÔÈ‹ıËΠÙÔ ÁÔÓ›‰ÈÔ Fras1 ÙÔ ÔÔ›Ô Îˆ‰ÈÎÔÔÈ› ÌÈ· Ó¤· ÂÍˆÎ˘ÙÙ¿ÚÈ·
ÚˆÙ½ÓË, Ë ÔÔ›· ÂÓÙÔ›˙ÂÙ·È Û ÂÈıËÏȷΤ˜
‚·ÛÈΤ˜ ÌÂÌ‚Ú¿Ó˜ ηٿ ÙËÓ ÂÌ‚Ú˘ÔÁ¤ÓÂÛË (68-70). ™ÙÔ˘˜ ·Ó·Ù˘ÛÛÔ̤ÓÔ˘˜ ÓÂÊÚÔ‡˜ Ë Fras1 ÂÓÙÔ›˙ÂÙ·È ÛÙË ‚·ÛÈ΋ ÌÂÌ‚Ú¿ÓË Ù˘
√∫ Î·È ÙˆÓ ÓÂÊÚÒÓˆÓ. ªÂÙ·ÏÏ¿ÍÂȘ ÛÙo ÁÔÓ›‰ÈÔ Fras1 ÚÔηÏÔ‡Ó ÙÔ Û‡Ó‰ÚÔÌÔ Fraser ÛÙÔÓ ¿ÓıÚˆÔ, ¤Ó· ·fi Ù· ΢ڛ· ‰È·ÁÓˆÛÙÈο ¯·Ú·-
ÎÙËÚÈÛÙÈο ÙÔ˘ ÔÔ›Ô˘ Â›Ó·È Ë ·ÁÂÓÂÛ›· ÙˆÓ ÓÂ-
ÊÚÒÓ. ¶·ÚfiÌÔÈÔ˜ Ê·ÈÓfiÙ˘Ô˜ ÚԤ΢„ ·fi
ÙËÓ ÛÙÔ¯Â˘Ì¤ÓË ·ÂÓÂÚÁÔÔ›ËÛË ÙÔ˘ Fras1
ÛÙÔÓ ÔÓÙÈÎfi (68). ªÂٷ͇ ¿ÏÏˆÓ Ê·ÈÓÔÙ˘È-
ÎÒÓ ·ÏÏÔÈÒÛÂˆÓ ÂΉËÏÒÓÂÙ·È Û ¤Ó· ÛËÌ·ÓÙÈÎfi ÔÛÔÛÙfi ÙˆÓ ÌÂÙ·ÏÏ·ÁÌ¤ÓˆÓ ˙ÒˆÓ ·ÁÂÓÂÛ›· ÙÔ˘ ÂÓfi˜ ‹ Î·È ÙˆÓ ‰‡Ô ÓÂÊÚÒÓ. ∞˘Ù‹
∏ ‰È·‰Èηۛ· Ù˘ ·Ó¿Ù˘Í˘ ÙˆÓ ÓÂÊÚÒÓ ÂÍ·ÚÙ¿Ù·È Û ÌÂÁ¿ÏÔ ÔÛÔÛÙfi ·fi ÙËÓ ·ÌÊ›‰ÚÔÌË ÛËÌ·ÙÔ‰fiÙËÛË ÌÂٷ͇ ÂÈıËÏ›Ô˘ Î·È ÌÂÛÂÁ¯‡Ì·ÙÔ˜, Ë ÔÔ›· ˘fiÎÂÈÙ·È Û ·˘ÛÙËÚfi Ú˘ıÌÈÛÙÈÎfi ¤ÏÂÁ¯Ô. √È Ê·ÈÓÔÙ˘ÈΤ˜ ·ÏÏÔÈÒÛÂȘ Ô˘ ÚÔ·ÙÔ˘Ó ·fi ÙË ÛÙÔ¯Â˘Ì¤ÓË ·ÂÓÂÚÁÔÔ›ËÛË ÁÔÓȉ›ˆÓ ÛÙÔÓ ÔÓÙÈÎfi ¤¯Ô˘Ó ÂÓÈÛ¯‡ÛÂÈ ÛËÌ·ÓÙÈο ÙË ÁÓÒÛË ÁÈ· ÙÔÓ ÚfiÏÔ Û˘ÁÎÂÎÚÈÌ¤ÓˆÓ ÌÔÚ›ˆÓ ÛÙË ÓÂÊÚÔÁ¤ÓÂÛË. ¶·ÚfiÏ· ·˘Ù¿, ¤Ó· ÌÂÁ¿ÏÔ Úfi‚ÏËÌ· Ô˘ ÚÔ·ÙÂÈ Ì ٤ÙÔÈÔ˘ ›‰Ô˘˜ ÚÔÛÂÁÁ›ÛÂȘ Â›Ó·È fiÙÈ Û ·ÚÎÂÙ¤˜ ÂÚÈÙÒÛÂȘ ÚÔηÏÂ›Ù·È ÂÌ‚Ú˘˚΋ ıÓËÛÈÌfiÙËÙ· ‹ ‰È·Ù·Ú·¯¤˜ ÛÙ· ÚÒÈÌ· ÛÙ¿‰È· Ù˘ ‰ËÌÈÔ˘ÚÁ›·˜ ÙˆÓ ÓÂÊÚÒÓ Ô˘ ‰ÂÓ ÂÈÙÚ¤Ô˘Ó ÙË ÌÂϤÙË Ù˘ ÏÂÈÙÔ˘ÚÁ›·˜ ÙˆÓ ÌÔÚ›ˆÓ ÛÙȘ ÌÂÙ¤ÂÈÙ· Ê¿ÛÂȘ Ù˘ ·Ó¿Ù˘Í˘. ∂Ô̤ӈ˜, ÈÔ Û‡ÓıÂÙ˜ ÚÔÛÂÁÁ›ÛÂȘ fiˆ˜ Ë ÈÛÙÔÂȉÈ΋ ‹ Ë Â·ÁÔ̤ÓË ·ÂÓÂÚÁÔÔ›ËÛË ÁÔÓȉ›ˆÓ Â›Ó·È ··Ú·›ÙËÙ˜ ÁÈ· ÙËÓ ·ÔÎ¿Ï˘„Ë ÙÔ˘ ÚfiÏÔ˘ Û˘ÁÎÂÎÚÈÌ¤ÓˆÓ ·Ú·ÁfiÓÙˆÓ ÛÙ· ‰È·ÊÔÚÂÙÈο ÛÙ¿‰È· Ù˘ ·Ó¿Ù˘Í˘ ÙˆÓ ÓÂÊÚÒÓ. ¶Ú¿ÁÌ·ÙÈ, Ù· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ· ¤¯Ô˘Ó ·Ó·Ù˘¯ı› Û˘ÛÙ‹Ì·Ù· fiˆ˜ ÙÔ Cre / LoxP ‹ ·ÎfiÌ· ÈÔ ÚfiÛÊ·Ù· Ë Ì¤ıÔ‰Ô˜ Ù˘ ÁÔÓȉȷ΋˜ Û›ÁËÛ˘ Ì ‰›ÎψÓÔ Û˘ÌÏËڈ̷ÙÈÎfi RNA (RNAi), ÔÈ Ôԛ˜ ÂÈÙÚ¤Ô˘Ó ÙËÓ ÂÈÏÔÁ‹ ÙfiÛÔ ÙÔ˘ ÈÛÙÔ‡ fiÛÔ Î·È Ù˘ ¯ÚÔÓÈ΋˜ ÛÙÈÁÌ‹˜ ηٿ ÙËÓ ÔÔ›·
Û˘ÁÎÂÎÚÈ̤ÓÔ˘ ÁÔÓȉ›Ô˘. ∆¤ÙÔÈÔ˘ ›‰Ô˘˜ ÚÔÛÂÁÁ›ÛÂȘ ·Ó·Ì¤ÓÂÙ·È Ó· ‰È¢ڇÓÔ˘Ó ÛËÌ·ÓÙÈο ÙËÓ ˘¿Ú¯Ô˘Û· ÁÓÒÛË Û¯ÂÙÈο Ì ÙÔÓ ÚfiÏÔ ÁÔÓȉ›ˆÓ ÛÙȘ ‰È¿ÊÔÚ˜ ·Ó·Ù˘ÍȷΤ˜ ‰È·‰Èηۛ˜.
∂˘¯·ÚÈÛٛ˜
∂˘¯·ÚÈÛٛ˜ ÛÙÔ˘˜ °ÂÒÚÁÈÔ Ã·Ï¿ÎË Î·È ƒ¤Ó· ÃȈٿÎË ÁÈ· ˘Ô‰Â›ÍÂȘ Î·È ‰ÈÔÚıÒÛÂȘ ÛÙÔ Î›ÌÂÓÔ.
µÈ‚ÏÈÔÁÚ·Ê›·
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2 °’ ÃÂÈÚÔ˘ÚÁÈ΋ ∫ÏÈÓÈ΋, °ÂÓÈÎfi ¡ÔÛÔÎÔÌÂ›Ô ¶ÂÈÚ·È¿
“∆˙¿ÓÂÈÔ”
AÏÏËÏÔÁÚ·Ê›·:
∞fiÛÙÔÏÔ˜ ∑¿ÚÚÔ˜
∆.£.14172,
∆.∫.115 10, ∞ı‹Ó·
∂-mail: azarros@med.uoa.gr
∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 04-03-2005
∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 10-02-2006
√ÈÎÈ·Îfi ÂÚÈ‚¿ÏÏÔÓ: ·›ÙÈ· Î·È ÚfiÏË„Ë
ÙˆÓ ·È‰ÈÎÒÓ ·Ù˘¯ËÌ¿ÙˆÓ
∫. ∫·ÏÔ‹Ù·1, ∞. ∑¿ÚÚÔ˜1, ∞. ∞ÓÙˆÓfiÔ˘ÏÔ˜2, π.™. ¶··‰fiÔ˘ÏÔ˜1
¶ÂÚ›ÏË„Ë: ∆· ·Ù˘¯‹Ì·Ù· Ô˘ ÛËÌÂÈÒÓÔÓÙ·È ÂÓÙfi˜ ÙÔ˘ ÔÈÎÈ·ÎÔ‡ ÂÚÈ‚¿ÏÏÔÓÙÔ˜ ÛÙÔÓ ·ÔηÏÔ‡ÌÂÓÔ “·ÓÂÙ˘Á̤ÓÔ ÎfiÛÌÔ”, ·ÔÙÂÏÔ‡Ó Ì›· ÛËÌ·ÓÙÈ΋ ·ÈÙ›· ÓÔÛËÚfiÙËÙ·˜, ÚÔÛˆÚÈÓ‹˜ ‹ ÌfiÓÈÌ˘ ·Ó·ËÚ›·˜ Î·È ıÓËÙfiÙËÙ·˜ ÛÙȘ Ó·ڤ˜ ËÏÈ˘, ÙÔ ÎÔÈÓˆÓÈÎfi Î·È ÔÈÎÔÓÔÌÈÎfi ÎfiÛÙÔ˜ ÙˆÓ ÔÔ›ˆÓηٿ ÁÂÓÈ΋ ÔÌÔÏÔÁ›· - ˘ÔÙÈÌ¿Ù·È. ™ÙËÓ ·ÚÔ‡Û· ·Ó·ÛÎfiËÛË Û˘ÓÔ„›˙ÔÓÙ·È: ·) Ù· ÛËÌ·ÓÙÈÎfiÙÂÚ· ·›ÙÈ· ÚfiÎÏËÛ˘ ·È‰ÈÎÒÓ ·Ù˘¯ËÌ¿ÙˆÓ ÛÙÔÓ ¯ÒÚÔ ÙÔ˘ ÛÈÙÈÔ‡ Î·È ‚) Ù· ‚·ÛÈÎfiÙÂÚ· Ú·ÎÙÈο Î·È ıˆÚËÙÈο Ï·›ÛÈ·
ÓÔ‡˜ ÂȂϤÔÓÙ˜ ÙˆÓ
ÙÔ˘˜), ‚) ÛÙÔ˘˜ ÁÈ·ÙÚÔ‡˜ (ˆ˜ ·Úԯ›˜ ÏËÚÔÊfiÚËÛ˘ Î·È ÊÔÚ›˜ Ù˘ ÁÓÒÛ˘) Î·È Á) ÛÙËÓ ÔÏÈÙ›· (ˆ˜ ÓÔÌÔı¤ÙË Î·È ÂÏÂÁÎÙ‹ ÂÊ·ÚÌÔÁ‹˜ Ù˘ ÓÔÌÈÌfiÙËÙ·˜ ÌÂ Ù˘ÊÏfi ÁÓÒÌÔÓ· ÙËÓ ˘Á›· ÙÔ˘
Domestic environment: causes and prevention of childhood accidents
1 Department of Experimental Pharmacology, Medical School, University of Athens
2 3rd Surgical Department, “Tzaneio” General Hospital of Piraeus
Correspondence:
Apostolos Zarros Po. Box: 14172, 115 10, Athens
∂-mail: azarros@med.uoa.gr
Date of submission: 04-03-2005
Date of approval: 10-02-2006
K. Kalopita1, A. Zarros1, A. Antonopoulos2, J.S. Papadopulos1
Abstract: In the so-called “developed world”, domestic accidents constitute a significant cause of morbidity, temporary or permanent disability and death in early life, the social and economic costs of which are generally underestimated. This is a review of: a) the most common causes of childhood accidents in the home and b) the main theoretical and practical aspects of their avoidance. The prevention of domestic childhood accidents necessitates more than strict and continuous enforcement of the relevant laws and safety standards; it also requires a significant change in attitude among: a) parents (who maintain the direct responsibility for the safety of their infants), b) doctors (who act as knowledge carriers and information providers) and c) the State (as legislator and auditor of enforcement of the law in favour of the health of its citizens).
Key words: Domestic childhood accidents, causes, prevention, safety, instruction, legislation, attitude, enforcement
ÂÊ‹‚Ô˘˜, ÙfiÛÔ ÛÙȘ
Î·È Û οı ¿ÏÏË Â˘ËÌÂÚÔ‡Û· ‰˘ÙÈ΋ ÎÔÈÓˆÓ›·, ‰ËÌÈÔ˘ÚÁÒÓÙ·˜ ·Ú¿ÏÏËÏ· ÛÔ‚·Ú¿ ÚÔ‚Ï‹Ì·Ù· ·Ó·ËÚ›·˜ Î·È ÂıÓÈÎÔ‡ ÔÈÎÔÓÔÌÈÎÔ‡ ÎfiÛÙÔ˘˜ (2). ŒÚ¢ӷ ÙˆÓ Del Ciampo Î·È Û˘Ó (3) ηٷϋÁÂÈ ÛÙÔ Û˘Ì¤Ú·ÛÌ· fiÙÈ Ù· Û˘¯ÓfiÙÂÚ· ·È‰Èο ·Ù˘¯‹Ì·Ù· ·ÊÔÚÔ‡Ó Û ÙÒÛÂȘ (46,9%), ıÏ¿ÛÂȘ (26,5%) Î·È ıÏ·ÛÙÈο ÙÚ·‡Ì·Ù· (11,9%), Ì ÙËÓ ÏÂÈÔ„ËÊ›· ·˘ÙÒÓ Ó· Ï·Ì‚¿ÓÂÈ ¯ÒÚ· ÛÙÔ ÔÈÎÈ·Îfi ÂÚÈ‚¿ÏÏÔÓ
(65,7%) Î·È ·ÎÔÏÔ‡ıˆ˜ ÛÙÔÓ ‰ÚfiÌÔ (25,4%) ηÈ
ÛÙÔ Û¯ÔÏÂ›Ô (6,1%). ∆· ÂÚÈÛÛfiÙÂÚ· ·È‰Èο
·Ù˘¯‹Ì·Ù· ÛËÌÂÈÒıËÎ·Ó Î˘Ú›ˆ˜ ·ÚÁ¿ ÙÔ ·fi-
ÁÂ˘Ì· (49,6%), ÈÔ Û˘¯Ó¿ ηٿ ÙË ‰È¿ÚÎÂÈ· ÙˆÓ
ηÏÔηÈÚÈÓÒÓ ÌËÓÒÓ Î·È ÙˆÓ Û¯ÔÏÈÎÒÓ ‰È·ÎÔ-
ÒÓ (38,1%). ¶ÚÔηÏ› ȉȷ›ÙÂÚË ÂÓÙ‡ˆÛË ÙÔ
ÁÂÁÔÓfi˜ fiÙÈ Ù· ÂÚÈÛÛfiÙÂÚ· ·È‰È¿ Û˘Óԉ‡Ô-
ÓÙ·Ó fiÙ·Ó Û˘Ó¤‚Ë ÙÔ ·Ù‡¯ËÌ·, Ì ÌÂÁ·Ï‡ÙÂÚË
Û˘¯ÓfiÙËÙ· Û˘Ì‚¿ÓÙˆÓ ÛÙ· ·ÁfiÚÈ· (55,4%) ÛÂ
Û¯¤ÛË Ì ٷ ÎÔÚ›ÙÛÈ· (44,6%) (3). ∂›Û˘, ¤¯ÂÈ
·Ô‰Âȯı› fiÙÈ ÔÈÎÔÁ¤ÓÂȘ Ì ¯·ÌËÏfiÙÂÚÔ ÎÔÈ-
ÓˆÓÈÎÔÔÈÎÔÓÔÌÈÎfi Â›Â‰Ô ÙËÚÔ‡Û·Ó ÏÈÁfiÙÂÚ·
̤ÙÚ· ·ÛÊ·Ï›·˜ ÁÈ· ÙËÓ ÚÔÛÙ·Û›· ÙˆÓ ·È-
‰ÈÒÓ ÙÔ˘˜, ÂÓÒ Ï›ÁÔÈ ÁÔÓ›˜ ›¯·Ó Ù· ·È‰È¿ ˘fi
ÙËÓ ·‰È¿ÏÂÈÙË Â›‚Ï„‹ ÙÔ˘˜ (4). ∞ÍÈÔÛËÌ›-
ˆÙÔ Â›Ó·È, ›Û˘, ÙÔ ÁÂÁÔÓfi˜ fiÙÈ ÔÈ ÂÚÈÛÛfiÙÂ-
ÚÔÈ ÙÚ·˘Ì·ÙÈÛÌÔ› Á›ÓÔÓÙ·È Û ¤Ó· ÂÚÈ‚¿ÏÏÔÓ
ÙÔ ÔÔ›Ô ıˆÚÔ‡ÓÙ·Ó ·ÛʷϤ˜ Û‡Ìʈӷ Ì ٷ
ÎÚÈÙ‹ÚÈ· ÙˆÓ ÁÔÓ¤ˆÓ. ™˘ÓÂÒ˜, ÛÙÔ Ï·›ÛÈÔ
Ù˘ ÚfiÏ˄˘ ı· Ú¤ÂÈ Ó· ‰Ôı› ȉȷ›ÙÂÚË ¤ÌÊ·ÛË ÛÙËÓ ·Ó·ÁÓÒÚÈÛË ÙˆÓ Èı·ÓÒÓ ÎÈÓ‰‡ÓˆÓ
Ô˘ ÂÏÏÔ¯Â‡Ô˘Ó Û οı ÛÙ¿‰ÈÔ ·Ó¿Ù˘Í˘ ÙÔ˘
·È‰ÈÔ‡ (5). ∆· ̤ÙÚ· Ô˘ ı· Ú¤ÂÈ Ó· ÏË-
ÊıÔ‡Ó ÚÔ˜ ·˘Ù‹ ÙËÓ Î·Ù‡ı˘ÓÛË, ı· Ú¤ÂÈ
Ó· ÛÙÔ¯Â‡Ô˘Ó ÛÂ: ·) ÚÔÛ·ÚÌÔÁ‹ Î·È ·ÛÊ·Ï‹
‰È·ÌfiÚʈÛË ÙÔ˘ ÔÈÎÈ·ÎÔ‡ ÂÚÈ‚¿ÏÏÔÓÙÔ˜, ‚)
ÂÓ›Û¯˘ÛË Ù˘ ÓÔÌÔıÂÛ›·˜, ηıÒ˜ Î·È Á) ÂÎ·È‰Â˘ÙÈ΋ ̤ÚÈÌÓ· (6).
∂ÍÂÙ¿˙ÔÓÙ·˜ Ù· ÛÙ·ÙÈÛÙÈο ‰Â‰Ô̤ӷ Ô˘
·ÊÔÚÔ‡Ó ÛÙËÓ ∂ÏÏ¿‰·, ·Í›˙ÂÈ Ó· Á›ÓÂÈ ·Ó·ÊÔÚ¿
Û ̛· ÂȉËÌÈÔÏÔÁÈ΋ ¤Ú¢ӷ Ô˘ ‰ÈÂÍ‹¯ıË ·fi ÙÔ ∂ÚÁ·ÛÙ‹ÚÈÔ ÀÁÈÂÈÓ‹˜ Î·È ∂ȉËÌÈÔÏÔ-
Á›·˜ Ù˘ π·ÙÚÈ΋˜ ™¯ÔÏ‹˜ ∞ıËÓÒÓ (7). ∏ ¤Ú¢ӷ Ú·ÁÌ·ÙÔÔÈ‹ıËΠ۠·È‰È¿ ËÏÈΛ·˜ 5-14
ÂÙÒÓ (ηÙÔ›ÎÔ˘˜ ∞ıËÓÒÓ), Ù· ÔÔ›· ‰È·ÎÔÌ›ÛÙËÎ·Ó ÛÙ· ›ÁÔÓÙ· ÙÌ‹Ì·Ù· ‰‡Ô ¶·ÓÂÈÛÙËÌÈ·ÎÒÓ ¡ÔÛÔÎÔÌ›ˆÓ ¶·›‰ˆÓ (·fi ÙÔÓ ¢ÂΤ̂ÚÈÔ 1993 ¤ˆ˜ Î·È ÙÔÓ ∞Ú›ÏÈÔ 1994) ÌÂÙ¿ ·fi ÙÚ·˘Ì·ÙÈÛÌfi. ¢ÈÂÚ¢ӋıËΠÙÔ Â¿Ó Î·È ÛÂ
ÔÈÔ ÔÛÔÛÙfi ‰È¿ÊÔÚ˜ ‚ÈÔÎÔÈÓˆÓÈΤ˜ ·Ú¿-
ÌÂÙÚÔÈ ·ÔÙÂÏÔ‡Ó ·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘ ÁÈ·
ÙËÓ ·ÛÊ¿ÏÂÈ· ÙˆÓ ·È‰ÈÒÓ. ∆· ·ÔÙÂϤÛÌ·Ù·
Ù˘ ¤Ú¢ӷ˜ ¤‰ÂÈÍ·Ó fiÙÈ Ë Èı·ÓfiÙËÙ· ÂÓfi˜ ·Ù˘¯‹Ì·ÙÔ˜ Â›Ó·È ˘„ËÏfiÙÂÚË Û ·È‰È¿: ·) ÌÂ
Ó·Úfi ·Ù¤Ú·, ‚) Ì ÌËÙ¤Ú· Ì ÌË-·ÁÁÂÏÌ·ÙÈ΋ ··Û¯fiÏËÛË, Á) Ì ˘„ËÏfiÙÂÚË ÛÂÈÚ¿ ÁÂÓÓ‹Ûˆ˜, ‰) Ì ÂÈÎÚ·Ù¤ÛÙÂÚË ÙËÓ Â›‚ÏÂ„Ë ·fi ÙÚ›ÙÔ˘˜, ηıÒ˜ Î·È Â) Ì ÈÛÙÔÚÈÎfi ÚÔËÁÔ‡ÌÂÓÔ˘ ·Ù˘¯‹Ì·ÙÔ˜ (7).
¶ÚfiÛÊ·ÙË ÌÂϤÙË ÙˆÓ Petridou Î·È Û˘Ó (8)
¤‰ÂÈÍ fiÙÈ Û ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ· ÂÓfi˜ ¤ÙÔ˘˜ ÙÚ·˘Ì·Ù›˙ÂÙ·È ÔÛÔÛÙfi 28% ÙˆÓ ·È‰ÈÒÓ (ËÏÈ-
›‰È· ÌÂϤÙË, ÔÈ ÂÚ¢ÓËÙ¤˜ Û˘Û¯ÂÙ›˙Ô˘Ó ÙË ÌÈÎÚ‹ ËÏÈΛ· ÙˆÓ ÁÔÓ¤ˆÓ Î·È ÙÔ ¯·ÌËÏfi ÌÔÚʈÙÈÎfi Â›Â‰Ô ·˘ÙÒÓ, Ì ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ ÚfiÎÏËÛ˘ ·È‰ÈÎÒÓ ·Ù˘¯ËÌ¿ÙˆÓ.
∏ÏÈÎȷ΋ ηٷÓÔÌ‹ ÙˆÓ ·È‰ÈÎÒÓ
·Ù˘¯ËÌ¿ÙˆÓ
¶ÚÔÛ¯ÔÏÈ΋ ËÏÈΛ· (0-5 ÂÙÒÓ) ™Â ·˘Ù‹ ÙËÓ ËÏÈΛ· ÔÈ ÙÒÛÂȘ Â›Ó·È 2 ÊÔÚ¤˜
Û˘¯ÓfiÙÂÚ˜ ·fi fi,ÙÈ ÛÙ· ÌÂÁ·Ï‡ÙÂÚ· ·È‰È¿
(Ì ÙȘ ÂÚÈÛÛfiÙÂÚ˜ Ó· Ï·Ì‚¿ÓÔ˘Ó ¯ÒÚ·
ÙÔ˘
Î·È Ó· Û¯ÂÙ›˙ÔÓÙ·È Û˘Ó‹ıˆ˜
‹ ÙËÓ ÂÚÈÔ¯‹ ÙÔ˘ ÂÚÈÓ¤Ô˘ (9,10). ™¯ÔÏÈ΋ ËÏÈΛ· (6-13 ÂÙÒÓ)
¢È·ÈÛÙÒÓÂÙ·È fiÙÈ Û ·˘Ù‹ ÙËÓ ËÏÈΛ· Ù·
¯ÒÚ· ÛÙÔ ÔÈÎÈ·Îfi
ÂÚÈ‚¿ÏÏÔÓ ÌÂÈÒÓÔÓÙ·È (Ì ÌÈÎÚfi ÔÛÔÛÙfi ÂÁη˘Ì¿ÙˆÓ Î·È ‰ËÏËÙËÚÈ¿ÛˆÓ), ‰›ÓÔÓÙ·˜ ÙË
ı¤ÛË ÙÔ˘˜ Û ¤Ó·Ó ÌÂÁ¿ÏÔ ·ÚÈıÌfi ÙÚÔ¯·›ˆÓ
·Ù˘¯ËÌ¿ÙˆÓ (10).
¶ÚÔÂÊË‚È΋ Î·È ÂÊË‚È΋ ËÏÈΛ· (13-19
ÂÙÒÓ)
™˘¯Ó¿ Â›Ó·È Ù· ·Ù˘¯‹Ì·Ù· ηٿ ÙË ‰È¿ÚÎÂÈ·
Ù˘ ÚÔfiÓËÛ˘ ‹ ÁÂÓÈÎfiÙÂÚ· ηٿ ÙËÓ ¿ıÏËÛË. ∂›Û˘, Û ·˘Ù‹ ÙËÓ ËÏÈΛ· ˘¿Ú¯ÂÈ ÌÂÁ¿ÏË Èı·ÓfiÙËÙ· Ó· ÙÚ·˘Ì·ÙÈÛÙ› Ô ¤ÊË‚Ô˜ ˆ˜ Ô‰ËÁfi˜ ‹ ÂÈ‚¿Ù˘ Ô¯‹Ì·ÙÔ˜, ÁÂÁÔÓfi˜ Ô˘ ÂÈÙ›ÓÂÙ·È ·fi ÙËÓ ˘ÂÚ‚ÔÏÈ΋ ¯Ú‹ÛË ·ÏÎÔfiÏ Î·È Ó·ÚΈÙÈÎÒÓ, ·ÏÏ¿ Î·È ÙËÓ ÂÊË‚È΋ ·ÚÔÚÌËÙÈÎfiÙËÙ· (10).
¶·Ú¿ÁÔÓÙ˜ Ô˘ ÂÓ¤¯ÔÓÙ·È ÛÙËÓ ÚfiÎÏËÛË
ÂÓ‹ÏÈÎÔ, ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ¤ÏÏÂÈ„Ë Â›Ú·˜, ˆÚÈÌfiÙËÙ·˜ Î·È ·ÓÙ›Ï˄˘ ÙÔ˘ ÎÈÓ‰‡ÓÔ˘,
·ÂÚ›ÛÎÂÙ·. ∆Ô Ó‹ÈÔ, fï˜, ÚÔÎÂÈ̤ÓÔ˘ Ó·
ÂÍÂÚ¢ӋÛÂÈ Î¿ıÂ Ù˘¯‹ ÙÔ˘ ·ÓÙÈÎÂÈ̤ÓÔ˘ Ô˘
ÂÚÈÂÚÁ¿˙ÂÙ·È, ıˆÚ› ‡ÛÙÔ¯Ô Ó· ÙÔ Ê¤ÚÂÈ ÛÙÔ
ÛÙfiÌ· ÙÔ˘ (11). ∞ÔÙÂÏ› ‰Â, ÛÙÔÈ¯Â›Ô Ù˘ Ê˘-
ÛÈÔÏÔÁÈ΋˜ ÙÔ˘ ·Ó¿Ù˘Í˘ Ë ÚÔÛ¿ıÂÈ· Ó· ‰È-
¢ڇÓÂÈ ÙÔ ÔÙÈÎfi ÙÔ˘ ‰›Ô (̤۷ ÛÙÔÓ ÌÈÎÚfi-
ÎÔÛÌfi ÙÔ˘) ̤ۈ Ù˘ ·Ó·ÚÚ›¯ËÛ˘ Û ¤ÈÏ·
Î·È ·Ú¿ı˘Ú·, ÂÓÒ - ÏfiÁˆ Ù˘ ·Ó·ÙÔÌÈ΋˜ ÙÔ˘
ηٷÛ΢‹˜ (¯·ÌËÏfiÙÂÚÔ Î¤ÓÙÚÔ ‚¿ÚÔ˘˜ ÏfiÁˆ ·Ó·ÛÙ‹Ì·ÙÔ˜) - ÙÔ Ó‹ÈÔ ¯¿ÓÂÈ Â‡ÎÔÏ· ÙËÓ
ÈÛÔÚÚÔ›· ÙÔ˘ fiÙ·Ó Û·‚ÂÈ (11,12).
∫·ıÔÚÈÛÙÈ΋˜ ÛËÌ·Û›·˜ ·Ú¿ÁÔÓÙ˜ Ô˘
ÂÓ¤¯ÔÓÙ·È ÛÙËÓ ÚfiÎÏËÛË ·Ù˘¯‹Ì·ÙÔ˜ ›ӷÈ
ÙfiÛÔ Ë ËÏÈΛ· ÙÔ˘ ·È‰ÈÔ‡, fiÛÔ Î·È ÙÔ Ê‡ÏÔ
ÙÔ˘. ∆· ·ÁfiÚÈ· Â›Ó·È ÈÔ ÂÈÚÚ‹ Û ·Ù˘¯‹Ì·-
Ù· ÏfiÁˆ Ù˘ ·Ó‹Û˘¯Ë˜ Î·È ÈÔ ÂÈıÂÙÈ΋˜ ʇ-
Û˘ ÙÔ˘˜. ∂›Ó·È ÁÂÁÔÓfi˜ fiÙÈ ÛÙÔÓ ‰Â‡ÙÂÚÔ ¯Úfi-
ÓÔ Ù˘ ˙ˆ‹˜ ÙÔ˘˜, Ù· ·È‰È¿ Ù˘ ÚÔÛ¯ÔÏÈ΋˜
ËÏÈΛ·˜ ¤¯Ô˘Ó ÙËÓ Ù¿ÛË Ó· ÂÍÂÚ¢ÓÔ‡Ó
ÓÙ·˜ ÁÈ· ÎÚ˘Ì̤ӷ ‹ ¿ÁÓˆÛÙ· ·ÓÙÈΛÌÂÓ·
Û›ÙÈ (12). ∞ӷχÔÓÙ·˜ ÙËÓ „˘¯ÔÏÔÁÈ΋ ·ÈÙÈfiÙËÙ· ÙˆÓ ·Ù˘¯ËÌ¿ÙˆÓ, Ú¤ÂÈ Ó· ·Ó·ÊÂÚı›
fiÙÈ ÙÔ ·È‰› ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÛΤ„Ë ·ÓÒÚÈ-
ÌË, ÚÈ„ÔΛӉ˘ÓË Î·È ÂÁˆÎÂÓÙÚÈ΋. ∂ÓÂÚÁ›, ‰ËÏ·‰‹, Ì ÁÓÒÌÔÓ· ÙËÓ ÈηÓÔÔ›ËÛË Ù˘ Â-
ÚȤÚÁÂÈ¿˜ ÙÔ˘. ∂Ô̤ӈ˜, Ë Â›‚ÏÂ„Ë ÙˆÓ ÁÔ-
Ó¤ˆÓ ı· Ú¤ÂÈ Ó· Â›Ó·È ¿ÌÂÛË Î·È Ô˘ÛÈ·ÛÙÈ΋, ·Ú·Ì¤ÓÔÓÙ·˜ ¿ÓÙ· Û ÂÁÚ‹ÁÔÚÛË, ηıÒ˜ ÙÔ
·È‰› ÎÈÓÂ›Ù·È Ì ¢ÂÏÈÍ›· Î·È Ù·¯‡ÙËÙ·, ‚¿˙Ô-
ÓÙ·˜ ˆ˜ ÛÙfi¯Ô˘˜ ·ÓÙÈΛÌÂÓ· Ô˘ ‰‡ÛÎÔÏ·
ÌÔÚ› Ó· ·ÓÙÈÏËÊı› Ô ÂÓ‹ÏÈÎÔ˜ (13). ™Ù· ·È-
‰È¿ ËÏÈΛ·˜ ÌÈÎÚfiÙÂÚ˘ ÙˆÓ 2 ÂÙÒÓ, Ë Â›‚Ï„Ë
Â›Ó·È ··Ú·›ÙËÙË (‰ÂÓ ı· Ú¤ÂÈ fï˜ Ó· ·ÁÁ›-
˙ÂÈ Î·È Ù· fiÚÈ· ÙÔ˘ ˘ÂÚÚÔÛٷ٢ÙÈÛÌÔ‡, η-
ıÒ˜ Ô ÙÂÏÂ˘Ù·›Ô˜ ηıÈÛÙ¿ Ù· ·È‰È¿ ÈÔ ÂÈÚ-
Ú‹ Û ·Ù˘¯‹Ì·Ù· ÏfiÁˆ ·ÙÂÏÔ‡˜ ·Ó¿Ù˘Í˘
ÙˆÓ ·ÓÙ·Ó·ÎÏ·ÛÙÈÎÒÓ ¤Ó·ÓÙÈ ÙˆÓ ÎÈÓ‰‡ÓˆÓ ÙÔ˘
ÂÚÈ‚¿ÏÏÔÓÙÔ˜). ∂›Û˘, ȉȷ›ÙÂÚË ÛËÌ·Û›·
Ú¤ÂÈ Ó· ‰›ÓÂÙ·È Û ·È‰È¿ Ô˘ ·ÚÔ˘ÛÈ¿˙Ô˘Ó
Ì·ıËÛȷΤ˜ ‹ ÎÈÓËÙÈΤ˜ ‰˘ÛÎÔϛ˜, ·‰ÂÍÈfiÙËÙ· ‹ ÂÌÊ·Ó›˙Ô˘Ó ÂÈÏËÙÈΤ˜ ÎÚ›ÛÂȘ, ηıÒ˜
ÛÙfi¯Ô ÙÔ˘ Î·È Ó·
ÙȘ ÎÈÓ‹ÛÂȘ ÙÔ˘, ȉ›ˆ˜ Â¿Ó ÚfiÎÂÈÙ·È ÁÈ· οÔÈÔ ËÏÈÎȈ̤ÓÔ ¿ÙÔÌÔ. ŒÙÛÈ, ÙȘ ÂÚÈÛÛfiÙÂÚ˜ ÊÔÚ¤˜, ÙÔ ·È‰› ‰Ú· Ù·¯‡Ù·Ù· Î·È ÙÔ ·Ù‡¯ËÌ· Ï·Ì‚¿ÓÂÈ ¯ÒÚ· ̤۷ Û ÎÏ¿ÛÌ·Ù· ÙÔ˘ ‰Â˘ÙÂÚÔϤÙÔ˘. ∆Ô Ê˘ÛÈÎfi ÂÚÈ‚¿ÏÏÔÓ ıˆÚÂ›Ù·È ·Ú¿ÁÔÓÙ·˜ ηıÔÚÈÛÙÈ΋˜ ÛËÌ·Û›·˜, ηıÒ˜ Û ·˘Ùfi ÌÔÚÔ‡Ó Ó· Á›ÓÔ˘Ó ÔÏϤ˜ ·ÚÂÌ‚¿ÛÂȘ, .¯. ÁÈ· ¤Ó· ·ÛʷϤ˜ Û›ÙÈ ¯ˆÚ›˜ ·Á›‰Â˜ Î·È ÎÈÓ‰‡ÓÔ˘˜ ÁÈ· ÙÔ ·È‰› (16). √ ÎfiÛÌÔ˜ ÛÙÔÓ ÔÔ›Ô ˙ÂÈ Î·È ·Ó·Ù‡ÛÛÂÙ·È ¤Ó· ·È‰› Â›Ó·È Û¯Â‰È·Ṳ̂ÓÔ˜ ·fi ÂÓ‹ÏÈΘ ÁÈ· ÂÓ‹ÏÈΘ, Î·È fi¯È (ηÈ) ÁÈ· ·È‰È¿. ∏ ·Ô˘Û›· ηٷÛ΢·ÛÙÈÎÒÓ Ì¤ÙÚˆÓ ÚfiÏ˄˘ ·È‰ÈÎÒÓ ·Ù˘¯ËÌ¿ÙˆÓ ÛÙÔ˘˜ ·ÓÂÏ΢ÛÙ‹Ú˜, ÛÙ· ·Ú¿ı˘Ú·, ÛÙȘ Á˘¿ÏÈÓ˜ ÌÂÛfiÔÚÙ˜, ÛÙ· Á˘¿ÏÈÓ· Ì·ÏÎfiÓÈ·, ÛÙÔ ‡„Ô˜ Ô˘ ¤¯Ô˘Ó Ù· οÁÎÂÏ· Î·È ÛÙȘ Îϛ̷Θ, ÔÊ›ÏÂÙ·È ÛÙÔ ÁÂÁÔÓfi˜ fiÙÈ ·˘Ù¿ ‰ËÌÈÔ˘ÚÁÔ‡ÓÙ·È Ì ÁÓÒÌÔÓ· ÙËÓ Ù·‡ÙÈÛË Ì ÙËÓ ·ÈÛıËÙÈ΋ Ù˘ ÂÔ¯‹˜ Î·È fi¯È ÙËÓ ÚÔÛÙ·Û›· ÙˆÓ ÂÓÔ›ÎˆÓ Ô˘ ÊÈÏÔÍÂÓÔ‡Ó. ∂ȉÈÎfiÙÂÚ·, ÛÙË ¯ÒÚ· Ì·˜ ˘¿Ú¯ÂÈ Ë Ù¿ÛË Ó· ÌÂÙ·ÙÚ¤ÂÙ·È Ë ÎÔ˘˙›Ó· (Ì fiÏÔ˘˜ ÙÔ˘˜ ÎÈÓ‰‡ÓÔ˘˜ Ô˘ ·˘Ù‹ ÂÓ¤¯ÂÈ) Û ‰ˆÌ¿ÙÈÔ ·Ó¿ıÚ„˘ ÙˆÓ ·È‰ÈÒÓ. ∂¿Ó ·Ó·ÏÔÁÈÛÙ› ηÓ›˜ ÙȘ ·Á›‰Â˜ Ô˘ ÂÏÏԯ‡ÂÈ ÁÈ· ÙÔ ·È‰› ·˘Ùfi˜ Ô ¯ÒÚÔ˜ (ËÏÂÎÙÚÈΤ˜ Û˘Û΢¤˜, η˘Ù¿ ˘ÁÚ¿, ·È¯ÌËÚ¿ ·ÓÙÈΛÌÂÓ·, ·ÔÚÚ˘·ÓÙÈο Î.¿.), ‰ÂÓ ı· Ú¤ÂÈ Ó· ·ÔÙÂÏ› ¤ÎÏËÍË ÙÔ ÁÂÁÔÓfi˜ fiÙÈ Ë ÎÔ˘˙›Ó· ·ÔÙÂÏ› ÙÔÓ ¯ÒÚÔ ÂÓÙfi˜ ÙÔ˘ ÛÈÙÈÔ‡ fiÔ˘ Á›ÓÔÓÙ·È Û˘¯ÓfiÙÂÚ· ·Ù˘¯‹Ì·Ù· (17).
¶·È‰Èο ·Ù˘¯‹Ì·Ù· ÛÙÔ ÔÈÎÈ·Îfi ÂÚÈ‚¿ÏÏÔÓ
™ÙÔÓ ¶›Ó·Î· 1, fiÔ˘ ·ÚÔ˘ÛÈ¿˙ÂÙ·È Ë ‰È·Î‡Ì·ÓÛË ÙÔ˘ Û˘ÓÙÂÏÂÛÙ‹ ÎÈÓ‰‡ÓÔ˘ ı·Ó¿ÙÔ˘ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ‰È·ÊfiÚˆÓ Î·Ù·ÛÙ¿ÛÂˆÓ Û ¢ËÌÂÚÔ‡Û˜ ‰˘ÙÈΤ˜ ÎÔÈӈӛ˜, Ê·›ÓÂÙ·È Ë ı¤ÛË Ô˘ η٤¯ÂÈ ÙÔ ÔÈÎÈ·Îfi ·Ù‡¯ËÌ· (18). ™ÙÔÓ ¶›Ó·Î· 2 ·ÚÔ˘ÛÈ¿˙ÂÙ·È Ë Ì¤ÛË ÂÙ‹ÛÈ· ıÓËÛÈÌfiÙËÙ·
¶›Ó·Î·˜ 1. ™˘ÓÙÂÏÂÛÙ‹˜ ÎÈÓ‰‡ÓÔ˘ ı·Ó¿ÙÔ˘ ‰˘ÙÈÎÒÓ ÎÔÈÓˆÓÈÒÓ (18) ∞ÈÙÈÔÏÔÁ›·∆ÈÌ‹ Û˘ÓÙÂÏÂÛÙ‹
∫¿ÓÈÛÌ· (1 ·Î¤ÙÔ/Ë̤ڷ)7.932
∫·ÚΛÓÔ˜ ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡1.008
∫·Ù·Ó¿ÏˆÛË ·ÏÎÔÔÏÔ‡¯ˆÓ591
∆ÚÔ¯·›Ô ·Ù‡¯ËÌ·216
√ÈÎÈ·Îfi ·Ù‡¯ËÌ·182
£‡Ì· ÙÚÔ¯·›Ô˘ ˆ˜ Â˙fi˜47
¢ÔÏÔÊÔÓ›·24
∞ÂÚÔÔÚÈÎfi ·Ù‡¯ËÌ·2
¶›Ó·Î·˜ 2. ª¤ÛË ÂÙ‹ÛÈ· ıÓËÛÈÌfiÙËÙ· ·fi ·Ù˘¯‹Ì·Ù· (ª£) ·Ó¿ 1.000.000 ¿ÙÔÌ·, ·Ó¿ÏÔÁ· Ì ÙËÓ ·ÈÙ›· ÙÔ˘ ·Ù˘¯‹Ì·ÙÔ˜
ηٿ ÙËÓ ÂÍ·ÂÙ›· 1990-1995 ÛÙËÓ ∂ÏÏ¿‰· (ÚˆÙÔÁÂÓ‹ ÛÙÔȯ›· Ù˘ ∂ıÓÈ΋˜ ™Ù·ÙÈÛÙÈ΋˜ ÀËÚÂÛ›·˜ Ù˘ ∂ÏÏ¿‰Ô˜) (19)
∫·ÙËÁÔÚ›· ηٿ ICD-9ª£ (0-4 ÂÙÒÓ)ª£ (5-14 ÂÙÒÓ)
∆˘¯·›Â˜ ÙÒÛÂȘ (∂880-∂888)6,92,5
∞Ù˘¯‹Ì·Ù· ·fi ˘ÚηÁÈ¿ (∂890-∂899)5,42,2
∆˘¯·›ÔÈ ÓÈÁÌÔ› Î.Ï. (∂910-∂915)68,110,5
∞Ù˘¯‹Ì·Ù· ·fi fiÏ· (∂922)0,01,1
£ÂÚÌÈο ·Ù˘¯‹Ì·Ù·, Ú‡̷ Î.Ï. (∂923-∂928)1,91,6
·Ó¿ 1.000.000 ¿ÙÔÌ·, ·fi ÔÚÈṲ̂Ó˜ ηÙËÁÔڛ˜ ·Ù˘¯ËÌ¿ÙˆÓ (‚¿ÛÂÈ Ù˘ Ù·ÍÈÓfiÌËÛ˘ ICD9: International Classification of Diseases-9) ÛÙËÓ ∂ÏÏ¿‰· (19).
∞ÎÔÏÔ˘ı› ÂÚÈÁÚ·Ê‹ ÙˆÓ ÛËÌ·ÓÙÈÎfiÙÂÚˆÓ ·Ù˘¯ËÌ¿ÙˆÓ Ô˘ ÛËÌÂÈÒÓÔÓÙ·È Ì¤Û· ‹
Á‡Úˆ ·fi ÙÔ Û›ÙÈ (›Ù ·˘Ùfi ÚfiÎÂÈÙ·È ÁÈ·
‰È·Ì¤ÚÈÛÌ· ÔÏ˘Î·ÙÔÈΛ·˜, ›Ù ÁÈ· ÌÔÓÔη-
ÙÔÈΛ· Û ·ÛÙÈÎfi ‹ ·ÁÚÔÙÈÎfi ÂÚÈ‚¿ÏÏÔÓ). ∏ ·‰Ú‹ ·˘Ù‹ Ù·ÍÈÓfiÌËÛË ·ÔÛÎÔ› ÛÙËÓ Î·Ï‡ÙÂÚË ·ÚÔ˘Û›·ÛË ÙˆÓ Èı·ÓÒÓ ÎÈÓ‰‡ÓˆÓ ηٿ
ÂÚ›ÙˆÛË, ηıÒ˜ ÙÔ 60-80% ÙÔ˘ Û˘ÓfiÏÔ˘ ÙˆÓ ·Ù˘¯ËÌ¿ÙˆÓ Ì¤¯ÚÈ ÙËÓ ËÏÈΛ· ÙˆÓ 4 ÂÙÒÓ,
Ï·Ì‚¿ÓÂÈ ¯ÒÚ· ÂÓÙfi˜ ÙÔ˘ ÔÈÎÈ·ÎÔ‡ ÂÚÈ‚¿ÏÏÔÓÙÔ˜ (20).
¢ËÏËÙËÚÈ¿ÛÂȘ
√ ¶›Ó·Î·˜ 3 ·ÚÔ˘ÛÈ¿˙ÂÈ ÙËÓ ËÏÈÎȷ΋ η-
Ù·ÓÔÌ‹ › ÙÔ˘ Û˘ÓfiÏÔ˘ ÙˆÓ ‰ËÏËÙËÚÈ¿ÛˆÓ
ÁÈ· Ù· ¤ÙË 1992 Î·È 1999, Ì ‚¿ÛË Ù· ÛÙÔȯ›·
Ô˘ ÂÏ‹ÊıËÛ·Ó ·fi ÙÔ ∂ıÓÈÎfi ∫¤ÓÙÚÔ ¢ËÏËÙËÚÈ¿ÛÂˆÓ (21). °›ÓÂÙ·È ·ÓÙÈÏËÙfi fiÙÈ Ù· ·È-
‰È¿ ËÏÈΛ·˜ 2-4 ÂÙÒÓ Û˘ÁÎÂÓÙÚÒÓÔ˘Ó ÙËÓ ˘„ËÏfiÙÂÚË Û˘¯ÓfiÙËÙ· ‰ËÏËÙËÚÈ¿ÛÂˆÓ ·fi οıÂ
¿ÏÏË (·Ó¿ÏÔÁÔ˘ ‡ÚÔ˘˜) ËÏÈÎȷ΋ ÔÌ¿‰·. ∆Ô 1999 ηٷÁÚ¿ÊËÎ·Ó Û˘ÓÔÏÈο 46.385 ÎÏ‹ÛÂȘ
ÚÔ˜ ÙÔ ∂ıÓÈÎfi ∫¤ÓÙÚÔ ¢ËÏËÙËÚÈ¿ÛˆÓ, ÂÎ ÙˆÓ ÔÔ›ˆÓ ÙÔ 57% ›¯Â ˆ˜ ÚԤϢÛË ÙÔ ‰È·Ì¤ÚÈÛÌ· ∞ÙÙÈ΋˜ Î·È ÙÔ ˘fiÏÔÈÔ 43% ÙËÓ ˘fiÏÔÈË ¯ÒÚ·. ∆Ô 2001 ÔÈ ·ÓÙ›ÛÙÔȯ˜ ÎÏ‹ÛÂȘ ‹Ù·Ó 44.809, ÂÎ ÙˆÓ ÔÔ›ˆÓ ÔÛÔÛÙfi 46%
¶›Ó·Î·˜ 3. ∏ÏÈÎȷ΋ ηٷÓÔÌ‹ › ÙÔ˘ Û˘ÓfiÏÔ˘ ÙˆÓ
‰ËÏËÙËÚÈ¿ÛÂˆÓ ÁÈ· Ù· ¤ÙË 1992 Î·È 1999 (21)
∏ÏÈΛ·1992 (%)1999 (%)
<1 ¤ÙÔ˘˜6,45,6 2-4 ÂÙÒÓ33,129,7 5-9 ÂÙÒÓ7,07,4 10-14 ÂÙÒÓ3,83,6 15-19 ÂÙÒÓ5,46,2 20+ ÂÙÒÓ44,347,5
™‡ÓÔÏÔ100100
(20.711 ÎÏ‹ÛÂȘ) ·ÊÔÚÔ‡Û Û ·È‰È¿ ËÏÈΛ·˜
¤ˆ˜ 14 ÂÙÒÓ (21,22).
√ ¶›Ó·Î·˜ 4 ·ÚÔ˘ÛÈ¿˙ÂÈ ÙËÓ ÔÛÔÛÙÈ·›· ηٷÓÔÌ‹ ÙˆÓ ·ÈÙÈÔÏÔÁÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ÁÈ· ÙÔ Û‡ÓÔÏÔ ÙˆÓ ‰ËÏËÙËÚÈ¿ÛÂˆÓ (fiÏˆÓ ÙˆÓ ËÏÈÎÈÒÓ)
Ô˘ ¤Ï·‚·Ó ¯ÒÚ· ηٿ Ù· ¤ÙË 1961-1966, 19681972, 1980, 1992 Î·È 1999 (20,22). ∆Ô 2001, ÙÔ 46% ÙˆÓ ‰ËÏËÙËÚÈ¿ÛÂˆÓ ÔÊÂÈÏfiÙ·Ó Û ʿÚ̷η, ÙÔ 23% Û ·ÔÚÚ˘·ÓÙÈο, ÙÔ 5% Û ηÏÏ˘ÓÙÈο Î·È ÙÔ 3,3% Û ÁˆÚÁÈο Ê¿Ú̷η (21,23). ∂Î ÙˆÓ Ê·ÚÌ·ÎÔÁÂÓÒÓ ‰ËÏËÙËÚÈ¿ÛˆÓ, ÙÔ 44% ÔÊ›ÏÂÙ·È Û ηٷӿψÛË „˘¯ÔÊ·Ú̿ΈÓ, ·ÓÙÈ‚ÈÔÙÈÎÒÓ Î·È ·Ó·ÏÁËÙÈÎÒÓ (23,24).
∞˘Ùfi ÔÊ›ÏÂÙ·È ÛÙËÓ ·˘ÍË̤ÓË Â˘Ú›· ¯Ú‹ÛË ÙÔ˘˜ Ù· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ·, ÙfiÛÔ ·fi ÓÂfiÙÂÚ· fiÛÔ Î·È ·fi ËÏÈÎȈ̤ӷ ¿ÙÔÌ· (Ì ‹ ¯ˆÚ›˜ È·ÙÚÈ΋ Û˘ÓÙ·Á‹), ηıÒ˜ Î·È ÛÙÔ ÁÂÁÔÓfi˜ fiÙÈ ÔÛÔÛÙfi ÂÚ›Ô˘ 15% ·˘ÙÒÓ ÙˆÓ ÂÚÈÙÒÛÂˆÓ ·ÓÙÈÚÔۈ‡ÂÈ ·fiÂÈÚ˜ ·˘ÙÔÎÙÔÓ›·˜.
™ÙËÓ Î·ÙËÁÔÚ›· ÙˆÓ ‰ËÏËÙËÚÈ¿ÛÂˆÓ ·fi
›‰Ë ÔÈÎȷ΋˜ ¯Ú‹Ûˆ˜ ı· Ú¤ÂÈ Ó· Á›ÓÂÈ ·Ó·ÊÔÚ¿ ÛÙ· ·ÎfiÏÔ˘ı·: ·) Â›Ó·È Û˘¯Ófi ÙÔ Ê·ÈÓfi-
ÌÂÓÔ ·Ó¿ÌÂÈ͢ ¯ÏˆÚ›Ó˘ Ì Ôͤ· ÛÙÔÓ ¯ÒÚÔ
ÙÔ˘ Ì¿ÓÈÔ˘ (¤ÎÏ˘ÛË ¯ÏˆÚ›Ô˘), Ì ·ÔÙ¤ÏÂ-
ÛÌ· ÙËÓ ÚfiÎÏËÛË ÛÔ‚·ÚÒÓ ·Ó·Ó¢ÛÙÈÎÒÓ
ÚÔ‚ÏËÌ¿ÙˆÓ ÛÙ· ·È‰È¿, ‚) Ë ‰ËÏËÙËÚ›·ÛË ÌÂ
ÔÈÓfiÓÂ˘Ì· (Ê·ÈÓfiÌÂÓÔ ÔÍ›·˜ ̤ı˘) ηχ-
ÙÂÈ ÌÂÁ¿ÏÔ ·ÚÈıÌfi ÂÚÈÙÒÛÂˆÓ (·ÚÎÂÙ¤˜ ηÈ
ÛÔ‚·Ú¤˜ Â›Ó·È ÔÈ ‰ËÏËÙËÚÈ¿ÛÂȘ ·fi ÎÔÏfiÓȘ)
Î·È Á) Ë ÏÂÈÔ„ËÊ›· ÙˆÓ Î·ÏÏ˘ÓÙÈÎÒÓ Ô˘ ¢ı‡ÓÔÓÙ·È ÁÈ· ÂÚÈÙÒÛÂȘ ‰ËÏËÙËÚ›·Û˘ ÛÙ·
·È‰È¿, ‰ÂÓ Â›Ó·È ÙÔÍÈο (ÂÎÙfi˜ ·fi οÔȘ
‚·Ê¤˜ Ì·ÏÏÈÒÓ). ¢ÂÓ ÈÛ¯‡ÂÈ ÙÔ ›‰ÈÔ fï˜ Î·È ÁÈ· ÙË Ì¿ÛËÛË
(21,23)
∞Èٛ˜ ‰ËÏËÙËÚÈ¿ÛˆÓ1961-19661968-1972198019921999 (%)(%)(%)(%)(%)
º¿Ú̷η9,430,550,542,446,1
∂›‰Ë ÔÈÎȷ΋˜ ¯Ú‹Ûˆ˜13,8 (+)11,8 (+)15,5 (+)25,122,6
∫·ÏÏ˘ÓÙÈο---6,05,2
¢È¿ÊÔÚ˜ ¯ËÌÈΤ˜ Ô˘Û›Â˜31,0 (+)23,9 (+)3,0 (+)1,4 (+)7,1
°ÂˆÚÁÈο Ê¿Ú̷η---4,53,6
∆ÚÔʤ˜----3,2
¢‹ÁÌ·Ù· ˙ÒˆÓ----1,9
º˘Ù¿----0,5
¢È¿ÊÔÚ˜ ¿ÏϘ ·Èٛ˜(45,8)(33,8)(31)(20,6)9,8 ™‡ÓÔÏÔ100100100100100
Î·È Ë ÂÚ›Ô‰Ô˜ ·Ì¤Ûˆ˜ ÌÂÙ¿ ÙÔÓ ÙÔÎÂÙfi
·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘ ÁÈ· Ù· ‹‰Ë
ÁÂÓÓËı¤ÓÙ· ·È‰È¿ Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜. ™˘ÁÎÂÎÚÈ̤ӷ, Ù· ·È‰È¿ ÙˆÓ ÔÔ›ˆÓ ÔÈ ÌËÙ¤Ú˜ ΢ÔÊÔ-
ÚÔ‡Û·Ó ‹ Á¤ÓÓËÛ·Ó ¤Ó·Ó ·‰ÂÏÊfi/·‰ÂÏÊ‹, ‹Ù·Ó
2 ÊÔÚ¤˜ ÈÔ Èı·Ófi Ó· ‰ËÏËÙËÚÈ·ÛÙÔ‡Ó ·fi Û›-
‰ËÚÔ Û ۇÁÎÚÈÛË Ì ÂΛӷ ÙˆÓ ÔÔ›ˆÓ Ë ÌËÙ¤Ú· ‰ÂÓ Á¤ÓÓËÛ (26). ∆· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ· ·Ú·ÙËÚÂ›Ù·È ÙÔ Ê·È-
ÓfiÌÂÓÔ Ù˘ ‰ËÏËÙËÚ›·Û˘ ÂÍ·ÈÙ›·˜ Ù˘ ηٿ-
ÔÛ˘ Ù·ÌÏÂÙÒÓ Î·ı·ÚÈÛÌÔ‡ ÔÚıÔ‰ÔÓÙÈÎÒÓ
ÚÔÛı¤ÛÂˆÓ Î·È Ê·ÎÒÓ Â·Ê‹˜ (27). ∏ Ù˘¯·›·
ηٿÔÛ‹ ÙÔ˘˜ ÌÔÚ› Ó· ÔÊ›ÏÂÙ·È ÛÙË Û˘¯Ó‹
¯Ú‹ÛË ÙÔ˘˜ ·fi Ù· ËÏÈÎȈ̤ӷ ¿ÙÔÌ· Ô˘ Û˘-
¯Ó¿ ÊÚÔÓÙ›˙Ô˘Ó Ù· ·È‰È¿, ·ÏÏ¿ Î·È ÛÙË Ï·Óı·-
Ṳ̂ÓË ÙÔÔı¤ÙËÛ‹ ÙÔ˘˜ ·fi ÙÔ˘˜ ÁÔÓ›˜ ÛÂ
ÚÔÛÂÁÁ›ÛÈÌ· ÁÈ· ÙÔ ·È‰› ÛËÌ›· ÙÔ˘ ÛÈÙÈÔ‡.
∫Ô˘˙›Ó· (Ô ÈÔ ÂÈΛӉ˘ÓÔ˜ ¯ÒÚÔ˜ ÙÔ˘ ÛÈÙÈÔ‡) ∆· ÈÔ Û˘¯Ó¿ ·È‰Èο ·Ù˘¯‹Ì·Ù· ·ÊÔÚÔ‡Ó Û ÂÁη‡Ì·Ù· ·fi ˙ÂÛÙfi ÓÂÚfi ÁÈ·
Á) ÔÈ ËÏÂÎÙÚÈΤ˜ Û˘Û΢¤˜, ‰) ÔÈ ‰È·ÎfiÙ˜ ÎÔ˘˙›Ó·˜ (ȉȷ›ÙÂÚ· ·Ó Â›Ó·È ·Î¿Ï˘ÙÔÈ) Î·È Â) Ù· ÔχÚÈ˙· Î·È Ù· ÂÎÙÂıÂÈ̤ӷ ηÏ҉ȷ (28). √È ËÏÂÎÙÚÈÎÔ› Ù·¯˘‚Ú·ÛÙ‹Ú˜ (ÔÈ ÔÔ›ÔÈ ·ÓÙÈη٤ÛÙËÛ·Ó ÙÔÓ
(29), fiˆ˜ Î·È ÔÈ
ÛÙÔ ÏÔ˘ÙÚfi-Ì¿ÓÈÔ ¶ÚfiÛÊ·Ù· ÛÙËÓ ∂ÏÏ¿‰·, Ù· ªª∂ ·Û¯ÔÏ‹ıËÎ·Ó ¤ÓÙÔÓ· Ì ÙËÓ ÂÚ›ÙˆÛË ‚Ú¤ÊÔ˘˜ Ô˘ ¿ÓÔÈÍ ÙË ‚Ú‡ÛË ÙÔ˘ ˙ÂÛÙÔ‡ ÓÂÚÔ‡ (ÛÙÔ ¯ÚÔÓÈÎfi
ÁÈ· ÙÔÓ ÚÔ˘¯ÈÛÌfi ÙÔ˘). ∆· ÂÁη‡Ì·Ù· ÛÙÔ Ì¿ÓÈÔ ·ÔÙÂÏÔ‡Ó Û˘¯Ófi ·ÔÙ¤ÏÂÛÌ· ·È‰ÈÎÒÓ ·Ù˘¯ËÌ¿ÙˆÓ Î·È ÔÊ›ÏÔÓÙ·È Î˘Ú›ˆ˜ ÛÙËÓ ·ÚÔÛÂÍ›· Î·È ÛÙËÓ ¤ÏÏÂÈ„Ë ÚÔÓÔËÙÈÎfiÙËÙ·˜ ÙˆÓ ÂÓËÏ›ÎˆÓ ÁÈ· ÙÔ˘˜ ÎÈÓ‰‡ÓÔ˘˜ Ô˘ ÂÏÏÔ¯Â‡Ô˘Ó ÛÙÔÓ ¯ÒÚÔ ·˘Ùfi (31). π‰È·›ÙÂÚ· ÂÈΛӉ˘ÓÔÈ (·ÏÏ¿ fi¯È ·ÎfiÌ· ‰È·‰Â‰Ô̤ÓÔÈ ÛÙËÓ ∂ÏÏ¿‰·) Â›Ó·È ÔÈ ‚Ú·ÛÙ‹Ú˜ ÓÂÚÔ‡ (ıÂÚÌÔۛʈÓ˜) Ô˘ ÏÂÈÙÔ˘ÚÁÔ‡Ó Ì ·¤ÚÈÔ, ηıÒ˜ Ë ‰È·Ê˘Á‹ ÌÔÓÔÍÂȉ›Ô˘ ÙÔ˘ ¿Óıڷη (CO) ηٿ ÙË ‰È¿ÚÎÂÈ· ÏÂÈÙÔ˘ÚÁ›·˜ Ù˘ Û˘Û΢‹˜ ¤¯ÂÈ ÂÓÔ¯ÔÔÈËı› ÁÈ· ·ÚÎÂÙ¿ ı·Ó·ÙËÊfiÚ· ÂÚÈÛÙ·ÙÈο ‰ÈÂıÓÒ˜ (32).
™Â ÌÂϤÙË Ù˘ Consumer Product Safety Commission (33) ÙˆÓ ∏¶∞, Ô˘ η٤ÁÚ·„ ÙÔ˘˜ ‚ÚÂÊÈÎÔ‡˜ ı·Ó¿ÙÔ˘˜ ·fi ÓÈÁÌfi (ηٿ ÙËÓ ÂÚ›Ô‰Ô 1983-1995), ÛËÌÂÈÒÓÔÓÙ·È Ù· ÂÍ‹˜: ·) ÔÈ ÂÚÈÛÛfiÙÂÚÔÈ ı¿Ó·ÙÔÈ (84%) ÛËÌÂÈÒıËÎ·Ó Û ·È‰È¿ ËÏÈΛ·˜ 5-15 ÌËÓÒÓ Ì ̤ÛË ËÏÈΛ· ÙÔ˘˜ 8 Ì‹Ó˜, ‚) ÙÔ 90% ÙˆÓ ·Ù˘¯ËÌ¿ÙˆÓ ·Ô‰fiıËΠ۠ÂÏÏÈ‹ ›‚ÏÂ„Ë ·fi ÙÔ˘˜ ÁÔÓ›˜ (Ì ̤ÛÔ ¯ÚfiÓÔ ·Ô˘Û›·˜ Ù· 6 ÏÂÙ¿) Î·È Á) ·ÚÎÂÙÔ› ·fi ÙÔ˘˜ ÁÔÓ›˜ (‹ ÙȘ babysitters) ¯ÚËÛÈÌÔÔÈÔ‡Û·Ó ÁÈ· ÙË ‰È¢ÎfiÏ˘ÓÛ‹ ÙÔ˘˜ ÂȉÈο ·È‰Èο ηı›ÛÌ·Ù· Ì¿ÓÈÔ˘.
¶ÙÒÛÂȘ (Ì·ÏÎfiÓÈ·, Ù·Ú¿ÙÛ˜, Îϛ̷Θ) ∏ ÏÂÈÔÓfiÙËÙ· ÙˆÓ ÙÒÛˆÓ
ÂΛ ηٿ ÙÔ˘˜ ˙ÂÛÙÔ‡˜ Ì‹Ó˜ ÙÔ˘ ¤ÙÔ˘˜, ¯ˆÚ›˜
Ó· ·ÓÙÈÏ·Ì‚¿ÓÔÓÙ·È ÙÔÓ Î›Ó‰˘ÓÔ ÁÈ· Ù· ·È‰È¿.
∞fi ¤Ú¢ӷ Ô˘ ‰ÈÂÍ‹¯ıË ÛÙËÓ ∆Ô˘ÚΛ· (34), ‚Ú¤ıËΠfiÙÈ ÙÔ 54,5% fiÏˆÓ ÙˆÓ ÙÒÛÂˆÓ Û˘Ì‚·›ÓÂÈ Î·Ù¿ ÙÔ ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ· ÌÂٷ͇ ª·˝Ô˘-∞˘ÁÔ‡ÛÙÔ˘. ∆· ÎÈÁÎÏȉÒÌ·Ù· ÛÙȘ ‚Â-
Ú¿ÓÙ˜ Î·È ÛÙȘ Ù·Ú¿ÙÛ˜ Û˘Ó‹ıˆ˜ ‰ÂÓ ÙËÚÔ‡Ó ÙȘ ηıÔÚÈṲ̂Ó˜ ÚԉȷÁڷʤ˜ ·ÛÊ·Ï›·˜
(ÛˆÛÙfi ‡„Ô˜), ηıÒ˜ Â›Ó·È Û¯Â‰È·Ṳ̂ӷ ‚¿ÛÂÈ
·ÈÛıËÙÈÎÒÓ ÎÚÈÙËÚ›ˆÓ. ∆Ú›· Â›Ó·È Ù· ‚·ÛÈο
ÛËÌ›· ÚfiÎÏËÛ˘ ·È‰ÈÎÒÓ ·Ù˘¯ËÌ¿ÙˆÓ ÛÙȘ
ÂÚÈÙÒÛÂȘ ·˘Ù¤˜: ·) Ù· ¯·ÌËÏ¿ οÁÎÂÏ· ÙˆÓ
Ì·ÏÎÔÓÈÒÓ, Ù· ÔÔ›· ›Ù ‰ÂÓ Â›Ó·È ÛÙ·ıÂÚ¿
ηٷÛ΢·Ṳ̂ӷ, ›Ù ‰È·ı¤ÙÔ˘Ó ÌÂÁ¿Ï· ‰È¿-
ÎÂÓ·, ‚) Ë ‰˘Ó·ÙfiÙËÙ· ÂχıÂÚ˘ ÚfiÛ‚·Û˘
ÙˆÓ ·È‰ÈÒÓ ÚÔ˜ ÙËÓ Ù·Ú¿ÙÛ· (ȉȷ›ÙÂÚ· ηٿ ÙÔ ‰È¿ÛÙËÌ· Ô˘ Ô ÁÔÓ¤·˜ ‹ Ô ÂȂϤˆÓ
Â›Ó·È ··Û¯ÔÏË̤ÓÔ˜) Î·È Á) ÔÈ ÌÂÁ¿Ï˜ ÂÈÊ¿ÓÂȘ Ù˙·ÌÈÒÓ Ô˘ ‰ÂÓ ‰È¢ÎÔχÓÔ˘Ó ÙË ‰˘Ó·ÙfiÙËÙ· ‰È¿ÎÚÈÛ˘-·ÓÙ›Ï˄˘ ÙÔ˘ Á˘¿ÏÈÓÔ˘
¯ˆÚ›ÛÌ·ÙÔ˜ (35).
ÃÚ‹ÛË ·ÓÂÏ΢ÛÙ‹ÚˆÓ
ªÔÚ› Ë Û¯ÂÙÈ΋ ÈӷΛ‰· Ó· ·ÔÙÚ¤ÂÈ
ÙË ¯Ú‹ÛË ·ÓÂÏ΢ÛÙ‹ÚˆÓ Û ·È‰È¿ ËÏÈΛ·˜ οو ÙˆÓ 14 ÂÙÒÓ, ·˘Ùfi fï˜ Û¿ÓÈ· ÌÔÚ› Ó· ÂÊ·ÚÌÔÛÙ› ÛÙËÓ Ú¿ÍË. ∆· ·È‰Èο ·Ù˘¯‹Ì·Ù· ·fi Ï·Óı·Ṳ̂ÓË ¯Ú‹ÛË ·ÓÂÏ΢ÛÙ‹Ú· ÂÚÈ-
Ï·Ì‚¿ÓÔ˘Ó: ·) ··Á¯ÔÓÈÛÌÔ‡˜, ‚) ÛÔ‚·ÚfiÙ·-
Ù˜ ηÎÒÛÂȘ Î·È Á) ËÏÂÎÙÚÔÏËÍ›·.
∂Áη‡Ì·Ù·
∏ ÓËȷ΋ ËÏÈΛ· Â›Ó·È Ë Ï¤ÔÓ ÂÈÚÚ‹˜
Û ÂÁη‡Ì·Ù·. ™Ù· Ôχ ÌÈÎÚ¿ ·È‰È¿ (1-2 ÂÙÒÓ) ·ÈÙ›· ÙÔ˘ ÂÁη‡Ì·ÙÔ˜ Â›Ó·È Û˘Ó‹ıˆ˜ Ë
·Ó·ÙÚÔ‹ ‰Ô¯Â›Ô˘ Ì ˙ÂÛÙfi ÓÂÚfi ÛÙËÓ ÂÛÙ›·
Ù˘ ÎÔ˘˙›Ó·˜ ‹ ÛÙÔ ÙÚ·¤˙È, ÂÓÒ ÛÙ· ÌÂÁ·Ï‡ÙÂ-
Ú· ·È‰È¿ Â›Ó·È ÂÚÈÛÛfiÙÂÚÔ Û˘¯Ó‹ Ë Û‡ÁÎÚÔ˘-
Û‹ ÙÔ˘˜ Ì ÂÓ‹ÏÈΘ Ô˘ ÎÚ·ÙÔ‡Ó ‰Ô¯Â›Ô Ì ˙Â-
ÛÙfi ÓÂÚfi ‹ Ê·ÁËÙfi (36). ™Ô‚·Ú¿ ÂÁη‡Ì·Ù·
ÌÔÚ› Ó· ÚÔÎÏËıÔ‡Ó Î·È fiÙ·Ó ÙÔ ·È‰› ÂÈÚ·Ì·Ù›˙ÂÙ·È Ì ۛÚÙ· Î·È ÔÈÓfiÓÂ˘Ì·. ™˘¯ÓfiÙÂÚ· Â›Ó·È Ù· ÂÁη‡Ì·Ù· ÛÙ· ·È‰È¿ Ô˘ ˙Ô˘Ó Û ‰È·ÌÂÚ›ÛÌ·Ù· ÔÏ˘Î·ÙÔÈÎÈÒÓ, ÂÓÒ fiÛÔÓ ·ÊÔÚ¿ ÛÙÔ Ê‡ÏÔ, Ë Û˘¯ÓfiÙËÙ· ÛÙ· ·ÁfiÚÈ· Â›Ó·È ‰‡Ô ÊÔÚ¤˜ ÌÂÁ·Ï‡ÙÂÚË Û ۯ¤ÛË Ì ٷ ÎÔÚ›ÙÛÈ·. ™ËÌ·ÓÙÈÎfi ΛӉ˘ÓÔ ·ÔÙÂÏ› Î·È ÙÔ ËÏÂÎÙÚÈÎfi Û›‰ÂÚÔ, ȉȷ›ÙÂÚ· ÁÈ· Ù· ·ÁfiÚÈ· ËÏÈΛ·˜ 1-2 ÂÙÒÓ (37). ∂ÎÙfi˜ ÙˆÓ ÂÚÈÙÒÛˆÓ
ۈ̷ÙÈ΋˜ ηÎÔÔ›ËÛ˘. ∏ ÎÏÈÓÈ΋ ÂͤٷÛË Î·È Ë ÌÂÙ¤ÂÈÙ· ¤Ú¢ӷ ·ÔÎ¿Ï˘„·Ó ‚’ ‚·ıÌÔ‡ ÂÁη‡Ì·Ù· Î·È ÛÙȘ ‰‡Ô Ï¢ڤ˜, Ô˘ ÚÔÎÏ‹ıËÎ·Ó ·fi ÙÔÓ Û‡-
ÓÙÚÔÊÔ Ù˘ ÌËÙ¤Ú·˜ ̤ۈ Ù˘ ›ÂÛ˘ ÂÓfi˜ ¯ÂÈÚÔΛÓËÙÔ˘ ÛÙÂÁÓˆÙ‹Ú· Ì·ÏÏÈÒÓ ¿Óˆ ÛÙÔ ‰¤ÚÌ· ÙÔ˘ ·È‰ÈÔ‡ (38).
∂›Û˘, ȉȷ›ÙÂÚ· ÂÈΛӉ˘Ó˜ Â›Ó·È Î·È ÔÈ
¿Ó˜ ÙÔ˘ ̈ÚÔ‡, ÔÈ Ôԛ˜ Û‡Ìʈӷ Ì ÎÏÈÓÈΤ˜ ÌÂϤÙ˜ ¤¯Ô˘Ó ·Ô‰Âȯı› ÂÍ·ÈÚÂÙÈο ‡ÊÏÂÎÙ˜ (39).
ÕÏϘ ÌÔÚʤ˜ ·Ù˘¯ËÌ¿ÙˆÓ
·fi Ú‡̷ ¯·ÌËÏ‹˜ Ù¿Ûˆ˜ ·ÔÙÂÏ› Û˘¯Ófi ·È‰ÈÎfi ·Ù‡¯ËÌ· Ô˘ ‰‡Ó·Ù·È Ó· ÚÔηϤÛÂÈ ÙÂÙ·Ó›·, ηډȷΤ˜ ‰È·Ù·-
‚Ï¿‚˜, ·ÎfiÌ· (Ê˘-
ı¿Ó·ÙÔ (40).
Û ÔÈÛÔÊ·ÁÈ΋ ÛÙ¤ÓˆÛË (41). ∞ÚÎÂÙ¿ ÂÚÈÛÙ·ÙÈο ÔÊ›ÏÔÓÙ·È Î·È Û ηٿÔÛË ËÏÂÎÙÚÈÎÒÓ Ì·Ù·ÚÈÒÓ, ÁÂÁÔÓfi˜ Ô˘ ÚÔηÏ› Î·È ÂÁη‡Ì·Ù· ÂÍ·ÈÙ›·˜ ÙˆÓ ˘ÁÚÒÓ Ô˘ ‰È·ÚÚ¤Ô˘Ó ÛÙÔ˘˜ Á‡Úˆ
Ì·Ï·ÎÔ‡˜ ÈÛÙÔ‡˜ (42). ™Â ·˘Ù¤˜ ÙȘ ÂÚÈÙÒÛÂȘ Û˘ÓÈÛÙ¿Ù·È ÚÒÈÌÔ˜ ÂÓ‰ÔÛÎÔÈÎfi˜ ¤ÏÂÁ-
¯Ô˜ ÚÔÎÂÈ̤ÓÔ˘ Ó· ηıÔÚÈÛÙ› Ë ÛÔ‚·ÚfiÙËÙ· Ù˘ ‚Ï¿‚˘. øÛÙfiÛÔ, Â¿Ó ÙÔ ·È‰› Â›Ó·È ·Û˘Ìو̷ÙÈÎfi, ı· ‹Ù·Ó ÈÔ ·ÛʷϤ˜ Ó· ·ÔÊ¢-
¯ı› Ô ÂÓ‰ÔÛÎÔÈÎfi˜ ¤ÏÂÁ¯Ô˜, ÚÔÎÂÈ̤ÓÔ˘ Ó·
ÌËÓ ÚÔÎÏËıÔ‡Ó ÂÚ·ÈÙ¤Úˆ ÚÔ‚Ï‹Ì·Ù· (43).
™ÙÔ ÔÈÎÈ·Îfi ÂÚÈ‚¿ÏÏÔÓ Â›Ó·È Èı·Ófi Ó·
·Ú·ÙËÚËıÔ‡Ó ÙÒÛÂȘ ÙÔ˘ ·È‰ÈÔ‡ ·fi ÙÔ ÎÚ‚¿ÙÈ, ÙËÓ ÎÔ˘Î¤Ù· (44) ‹ ·fi „ËϤ˜ ηڤÎϘ (45). ∏ ¯ÂÈÚÔΛÓËÙË ÎÔ‡ÓÈ· ¤¯ÂÈ ÂÓÔ¯ÔÔÈËı› ÁÈ· ÙËÓ ÚfiÎÏËÛË .¯. ·ÚÎÂÙÒÓ Î·Ù·Á-
Ì¿ÙˆÓ, ÂÓÒ ÛÙ· ·Ù˘¯‹Ì·Ù· Ô˘ ÂÓ¤¯Ô˘Ó ÙÒÛÂȘ ·fi ηڤÎϘ, Ù· ̈ڿ ÚÔÛ·ıÔ‡Ó Ó·
ÛËΈıÔ‡Ó ¯ˆÚ›˜ ÙÔ Î¿ıÈÛÌ· Ó· ¤¯ÂÈ ÂÚȉÂı› ‹ Ó· ¤¯Ô˘Ó ÏËÊı› ÚÔÏËÙÈο ̤ÙÚ· ÁÈ· ÙË
ÛÙ·ıÂÚfiÙËÙ· Ù˘ ηڤÎÏ·˜ (46). ™ÙË ¯ÒÚ· Ì·˜, Û˘Ì‚·›ÓÔ˘Ó Û˘ÓÔÏÈο ÂÚ›Ô˘ 4.400 ÙÚ·˘Ì·ÙÈÛÌÔ› ·È‰ÈÒÓ ·fi ÙÒÛÂȘ Û ÂÙ‹ÛÈ· ‚¿ÛË (47). ∂Í ·˘ÙÒÓ, ÔÛÔÛÙfi 10% ÂÚ›Ô˘ ··ÈÙ› ÓÔÛËÏ›· (47).
∞Í›˙ÂÈ, ›Û˘, Ó· ÛËÌÂȈı› Ô Û˘Ó¯Ҙ ·˘Í·ÓfiÌÂÓÔ˜ ·ÚÈıÌfi˜ ÙˆÓ ·È‰È·ÙÚÈÎÒÓ ÙÚ·˘Ì¿ÙˆÓ ÛÙÔ ÎÂÊ¿ÏÈ ÂÍ·ÈÙ›·˜ Ù˘ ÙÒÛ˘ Ù˘ Û˘Û΢‹˜ Ù˘ ÙËÏÂÔÚ¿Ûˆ˜, ÛÂ Û˘Ó¿ÚÙËÛË
Û˘Ì‚·›ÓÂÈ Û ·È‰È¿ ËÏÈΛ·˜ 1-3 ÂÙÒÓ, Ù· ÔÔ›·
·Ú¤ÌÂÈÓ·Ó ÛÙÔ Û›ÙÈ ¯ˆÚ›˜ ›‚Ï„Ë. ∆Ô Ô-
ÛÔÛÙfi ıÓËÛÈÌfiÙËÙ·˜ ‹Ù·Ó ȉȷ›ÙÂÚ· ˘„ËÏfi, ÂÈ-
‰Èο ÛÙ· ·È‰È¿ Ì ·ÎfiÏÔ˘ıÔ ÙÚ·‡Ì· ÛÙÔ
ÎÂÊ¿ÏÈ (48,49).
™ÙȘ ·ÓÒÙÂÚ˜ ÔÈÎÔÓÔÌÈο Ù¿ÍÂȘ ·Ú·ÙË-
ÚÂ›Ù·È ÙÔ Ê·ÈÓfiÌÂÓÔ ÙÔ˘ ÓÈÁÌÔ‡ ‹ ÙÔ˘ ‰Â˘ÙÂ-
ÚÔ·ıÔ‡˜ ÓÈÁÌÔ‡ Û ÈÛ›Ó·, Û˘¯ÓfiÙÂÚ· ı‡-
Ì·Ù· ÙˆÓ ÔÔ›ˆÓ Â›Ó·È Ù· ·È‰È¿ ËÏÈΛ·˜ 1-4
ÂÙÒÓ Î·È ÂȉÈο Ù· ·ÁfiÚÈ· ‹ Ù· ·È‰È¿ Ô˘ ¿-
Û¯Ô˘Ó ·fi ÂÈÏË„›·. ∆Ô 46% ·˘ÙÒÓ ÙˆÓ ·Ù˘-
¯ËÌ¿ÙˆÓ Û˘Ó¤‚Ë Î·Ù¿ ÙÔ˘˜ ¯ÂÈÌÂÚÈÓÔ‡˜ Ì‹-
Ó˜, ÂÓÒ Ù· ÌÈÛ¿ ·fi ·˘Ù¿ ÔÊ›ÏÔÓÙ·Ó Û ¤Ï-
ÏÂÈ„Ë Î¿ÔÈÔ˘ ̤ÛÔ˘ ÚÔÛÙ·Û›·˜ (.¯. ÚÔ-
Ûٷ٢ÙÈÎfi ‰›¯Ù˘) (50).
∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ·È¯ÓȉÈÔ‡ ÂÓ‰¤¯ÂÙ·È Ó· ÚÔÎÏËıÔ‡Ó ÙÚ·˘Ì·ÙÈÛÌÔ›
Î·È ÙˆÓ ·˘ÙÈÒÓ. Œ¯ÂÈ ‚ÚÂı› fiÙÈ ‰È·ÂÚ·ÛÙÈο ÙÚ·‡Ì·Ù· ÙÔ˘ ÔÊı·ÏÌÔ‡
ÛÙÔ ÔÈÎÈ·Îfi ÂÚÈ‚¿ÏÏÔÓ ÏfiÁˆ ·È¯ÌËÚÒÓ ÂÚÁ·Ï›ˆÓ ‹ ·ÓÙÈÎÂÈÌ¤ÓˆÓ Ô˘ ‚Ú›ÛÎÔÓÙ·È
ÛÙÔ ¿ÙˆÌ· (51). ∂Í›ÛÔ˘ ÂÈΛӉ˘Ó· Â›Ó·È Î·È Ù· baby-walkers (·È‰ÈΤ˜ “ÛÙÚ¿Ù˜”), ÂÓÒ
¤¯Ô˘Ó ÛËÌÂȈı› ·ÚÎÂÙ¿ ·Ù˘¯‹Ì·Ù· Ì ÙÔ˘˜
ÈÌ¿ÓÙ˜ ÙˆÓ ÔÚÁ¿ÓˆÓ Á˘ÌÓ·ÛÙÈ΋˜ Ô˘ ‚Ú›-
ÛÎÔÓÙ·È Ì¤Û· ÛÙÔ Û›ÙÈ (52). ∂ÈϤÔÓ, ›ӷÈ
·˘ÙÔÓfiËÙÔ fiÙÈ Ë ·ÛÊ·Ï‹˜ ·Ôı‹Î¢ÛË Î·È
ʇϷÍË ÙˆÓ fiÏˆÓ Û ¿ÁÓˆÛÙ· Î·È Ï›·Ó ‰˘-
ÛÚfiÛÈÙ· ÁÈ· ÙÔ ·È‰› ̤ÚË, ·ÔÙÂÏ› ··Ú·›-
ÙËÙË ÚÔ¸fiıÂÛË ÔÏÔηÙÔ¯‹˜ ÔÔÈÔ˘‰‹Ô-
ÙÂ ÁÔÓ¤· (53).
™ÙÔ Ï·›ÛÈÔ Ì›·˜ ·ÁÚÔÙÈ΋˜ ‹ ÚÔ·ÛÙȷ΋˜
ηÙÔÈΛ·˜, ÔÈ Î›Ó‰˘ÓÔÈ ÁÈ· ÙÔ ·È‰› ·˘Í¿ÓÔÓÙ·È. ¶·Ú·ÙËÚÔ‡ÓÙ·È ÂÚÈÛÛfiÙÂÚ· ÎÚÔ‡ÛÌ·Ù· ‰ËÏËÙËÚ›·Û˘ ·fi ÁˆÚÁÈο Ê¿Ú̷η (54), ÙÒÛÂȘ ·fi ÛοϘ Ô˘ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È Û˘¯Ó¿ (55), ÙÚ·‡Ì·Ù· ·fi Ì˯·Ó¤˜ ÂÚÈÔ›ËÛ˘ ÁÚ·-
ÛȉÈÔ‡ (56), ·ÎfiÌË Î·È ·fi ÌË ÂÏÂÁ¯fiÌÂÓË ÏÂÈ-
ÙÔ˘ÚÁ›· ÙÔ˘ ȉȈÙÈÎÔ‡ ¯ÒÚÔ˘ ÛÙ¿ıÌ¢Û˘. ™ÙËÓ ∂ÏÏ¿‰·, ηٷÁÚ¿ÊËÎ·Ó Û˘ÓÔÏÈο 4.326
·Ù˘¯‹Ì·Ù· Û ·ÁÚÔÙÈÎfi ÂÚÈ‚¿ÏÏÔÓ Î·Ù¿ ÙËÓ
ÂÓÙ·ÂÙ›· 1996-2000, ÂÎ ÙˆÓ ÔÔ›ˆÓ Ë ÏÂÈÔ„ËÊ›· ÙˆÓ ·È‰ÈÎÒÓ ·Ù˘¯ËÌ¿ÙˆÓ ·ÊÔÚÔ‡Û ÛÂ
η΋ ¯Ú‹ÛË ÙˆÓ ÁˆÚÁÈÎÒÓ Ô¯ËÌ¿ÙˆÓ (57).
∏ ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ Ì›˙ÔÓÔ˜ ÚÔ‚Ï‹Ì·ÙÔ˜ ÙˆÓ ·È‰ÈÎÒÓ ·Ù˘¯ËÌ¿ÙˆÓ Û˘ÓÔ„›˙ÂÙ·È ÛÙËÓ ÚÔÛ¿ıÂÈ· ÁÈ· ÙËÓ ÚfiÏË„‹ ÙÔ˘˜. ∏ ÚfiÏË„Ë Û˘Ó›ÛÙ·Ù·È ÛÙËÓ ÂÈÙ˘¯‹ ‰È·ÌfiÚʈÛË Î·È ÂÊ·ÚÌÔÁ‹: ·) ÔÚÁ¿ÓˆÛ˘ Î·È ÓÔÌÔıÂÙÈÎÒÓ Ì¤ÙÚˆÓ, ‚) ¤Ú¢ӷ˜ Î·È Á) Âη›‰Â˘Û˘. ¶¿Óˆ ·fi fiÏ·, ˆÛÙfiÛÔ, Ë ÚfiÏË„Ë ÂÈÙ˘Á¯¿ÓÂÙ·È Ì ·ÏÏ·Á‹ ÓÔÔÙÚÔ›·˜ (22). ∏ ‰È·ÌfiÚʈÛË ÂÓÈ·›·˜ ÂıÓÈ΋˜ ÔÏÈÙÈ΋˜ ÛÙÔÓ ÙÔ̤· Ù˘ ÚfiÏ˄˘ ÙˆÓ ·È‰ÈÎÒÓ ·Ù˘¯ËÌ¿ÙˆÓ, Ë ‡·ÚÍË Û˘ÓÙÔÓÈÛÙÈÎÔ‡ ÊÔÚ¤· Î·È Ë ÓÔÌÔıÂÙÈ΋ Ï·ÈÛ›ˆÛË Ù˘ ‰ÈÂÚ‡ÓËÛ˘ οı ·È‰ÈÎÔ‡ ·Ù˘¯‹Ì·ÙÔ˜ Â›Ó·È ÙÔ ÚÒÙÔ ‚‹Ì· ÚÔ˜ ·˘Ù‹ ÙËÓ Î·Ù‡ı˘ÓÛË. ∏ ÂÊ·ÚÌÔÁ‹
ÁÚ·ÌÌ¿ÙˆÓ (Ì ‚¿ÛË Ù· ÂÏÏËÓÈο ̤ÙÚ· Î·È ‰Â‰Ô̤ӷ) ¤Ú¢ӷ˜ Î·È ‰ÈÂÚ‡ÓËÛ˘, ÙfiÛÔ ÙˆÓ ·ÈÙÈÔÏÔÁÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ÚfiÎÏËÛ˘ ·È‰ÈÎÒÓ ·Ù˘¯ËÌ¿ÙˆÓ fiÛÔ Î·È Ù˘ ÂÊ·ÚÌÔÁ‹˜ Ù˘ Û¯ÂÙÈ΋˜ ÓÔÌÔıÂÛ›·˜, ı· ʤÚÂÈ ÛÙÔ Êˆ˜ ÔÏÏ¿ ÛÙÔȯ›· Û¯ÂÙÈο Ì ÙÔ˘˜ ÂοÛÙÔÙ ÂÌÏÂÎfiÌÂÓÔ˘˜ ·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘, ÙËÓ ÂÈÎÈÓ‰˘ÓfiÙËÙ· ÙˆÓ ÂÈÎÚ·ÙÔ‡ÓÙˆÓ ¯ˆÚÔÙ·ÍÈÎÒÓ ÚԉȷÁÚ·ÊÒÓ, ·ÏÏ¿ Î·È ÙË ÓÔÛÔÎÔÌÂȷ΋ ÓÔÛËÏ›· Î·È ÙÔ ÎfiÛÙÔ˜ Û ¤Ì„˘¯Ô Î·È ÌË ˘ÏÈÎfi. ∆¤ÏÔ˜, Ë Âη›‰Â˘ÛË ı· Ú¤ÂÈ Ó· ÂÈÎÂÓÙÚˆı› ÛÙËÓ ÂÎfiÓËÛË Î·È ÂÊ·ÚÌÔÁ‹ ÚÔÁÚ·ÌÌ¿ÙˆÓ ˘Á›·˜ Ô˘ ı· ÚÔ‚¿ÏÏÔ˘Ó ÙË ÛËÌ·Û›· Ù˘ ÚfiÏ˄˘ ÙˆÓ ·Ù˘¯ËÌ¿ÙˆÓ, ÙÔÓ›˙ÔÓÙ·˜ ÙËÓ ·Ó·ÁηÈfiÙËÙ· Âη›‰Â˘Û˘ ÙÔ˘ ÁÔÓ¤·, ÙÔ˘ ‰·ÛοÏÔ˘, ·ÏÏ¿ Î·È ÙÔ˘ ›‰ÈÔ˘ ÙÔ˘ ·È‰ÈÔ‡. ∞fi ÏÂ˘Ú¿˜ ÁÔÓ¤ˆÓ Â›Ó·È ·Ó·Áη›· Ë ÂÈÌfiÚʈÛË Î·È Ë ·Ú·ÎÔÏÔ‡ıËÛË ÂȉÈÎÒÓ ÚÔÁÚ·ÌÌ¿ÙˆÓ, ¯ˆÚ›˜ ˆÛÙfiÛÔ Ó· ˘ÂÚÙÔÓ›˙ÂÙ·È Ë ·Ó¿ÁÎË ÁÈ· Û˘Ó¯‹ ›‚ÏÂ„Ë Û ‚·ıÌfi Ô˘ Ó· ÂÚÈÔÚ›˙ÂÈ ÙËÓ ·ÓÂÍ·ÚÙËÛ›· ÙÔ˘ ·È‰ÈÔ‡ (59). ∏ Û˘ÌÂÚÈÊÔÚ¿ ÙˆÓ ÁÔÓ¤ˆÓ ÔÊ›ÏÂÈ Ó· ÚÔÛ·ÚÌfi˙ÂÙ·È ·Ó¿ÏÔÁ· Ì ÙËÓ ËÏÈΛ· ÙÔ˘ ·È‰ÈÔ‡, ηıÒ˜ ÔÈ Î›Ó‰˘ÓÔÈ - fiˆ˜ ÚԷӷʤÚıËΠ- ‰È·ÊÔÚÔÔÈÔ‡ÓÙ·È Û οı ÛÙ¿‰ÈÔ Ù˘ ˙ˆ‹˜ ÙÔ˘˜. ∏ Âη›‰Â˘ÛË ÙˆÓ ›‰ÈˆÓ ÙˆÓ ·È‰ÈÒÓ Â›Ó·È Â›Û˘ ÎÂÊ·Ï·ÈÒ‰Ô˘˜ ÛËÌ·Û›·˜, ηıÒ˜ Ë ÁÓÒÛË ÙÔ˘ ÎÈÓ‰‡ÓÔ˘ ÌÔÚ› Ó· ÙÔ ·ÔÙÚ¤„ÂÈ ·fi ÛËÌ·ÓÙÈο Ï¿ıË. ∞˘Ùfi ·ÔÎÙ¿ ȉȷ›ÙÂÚË ÛËÌ·Û›·, Â¿Ó ÔÈ ÁÔÓ›˜ ·ÓÙÈÏËÊıÔ‡Ó fiÙÈ Ë ÚÔÛˆÈÎfiÙËÙ· ‹ Ë „˘¯ÔÛ‡ÓıÂÛË ÙÔ˘ ·È‰ÈÔ‡ ÙÔ˘˜ ÙÔ Ô‰ËÁ› Û ȉȷ›ÙÂÚ· ÂÈΛӉ˘Ó˜ ÂÓ¤ÚÁÂȘ. º˘ÛÈο, Ë Âη›‰Â˘ÛË ÙˆÓ ·È‰ÈÒÓ ‰ÂÓ ¤¯ÂÈ
ÛËÌ·Û›· fiÙ·Ó ÚfiÎÂÈÙ·È .¯. ÁÈ· ‚Ú¤ÊË (fiÔ˘ Ë Â›‚ÏÂ„Ë ÎÚ›ÓÂÙ·È ÂÈÙ·ÎÙÈ΋) (60).
ÛÙÔ ÔÈÎÈ·Îfi ÂÚÈ‚¿ÏÏÔÓ (fiÔ˘ ·˘Ù¿, Ê˘ÛÈο, ÂÊ·ÚÌfiÛÙËηÓ) ¤¯Ô˘Ó ·Ô‰Âȯı› ȉȷÈÙ¤Úˆ˜
·ÔÙÂÏÂÛÌ·ÙÈο ÛÙËÓ ·ÔÙÚÔ‹ ÙˆÓ ·È‰ÈÎÒÓ
·Ù˘¯ËÌ¿ÙˆÓ. ∂›Ó·È ÎÔÈÓ‹ ›ÛÙË ÙˆÓ ÂÚ¢ÓËÙÒÓ
fiÙÈ Ù· ÚÔÁÚ¿ÌÌ·Ù· ·˘Ù¿ ÌÔÚÔ‡Ó Ó· Â›Ó·È È‰È-
·›ÙÂÚ· ˆÊ¤ÏÈÌ·, Û˘Ì‚¿ÏÏÔÓÙ·˜ ÛÙËÓ ·ÏÏ·Á‹
Ù˘ Û˘ÌÂÚÈÊÔÚ¿˜ ÙˆÓ ÂÓËÏ›ÎˆÓ (61,62).
™ËÌ·ÓÙÈÎfi ÛÙÔÈ¯Â›Ô ÁÈ· ÙËÓ ÚfiÏË„Ë ÙˆÓ
·Ù˘¯ËÌ¿ÙˆÓ ·ÔÙÂÏ› Ë ‡·ÚÍË Û˘Ó‰˘·ÛÌÔ‡
ÓfiÌˆÓ Î·È Î·ÓfiÓˆÓ, ÈηÓÒÓ Ó· ·Ó·ÚÔÛ·ÚÌfi-
˙ÔÓÙ·È ‰È·ÚÎÒ˜ ÛÙȘ ÌÂÙ·‚·ÏÏfiÌÂÓ˜ ‚ÈÔÙÈΤ˜
Û˘Óı‹Î˜. √Úıfi˜ Î·È ·ÛÊ·Ï‹˜ ·Ú¯ÈÙÂÎÙÔÓÈÎfi˜
ۯ‰ȷÛÌfi˜ ÛËÌ·›ÓÂÈ: ·) ·ÔÊ˘Á‹ ηٷÛ΢‹˜
·fiÙÔÌˆÓ Î·È ¯ˆÚ›˜ ÎÔ˘·ÛÙ‹ ÎÏÈ̿ΈÓ, ‚)
‡·ÚÍË Î·Ù¿ÏÏËÏÔ˘ ʈÙÈÛÌÔ‡ ̤۷ Î·È Á‡Úˆ
·fi ÙÔ Û›ÙÈ, Á) ÔÚı‹ ηٷÛ΢‹ ÎÈÁÎÏȉˆÌ¿-
ÙˆÓ Û ‚ÂÚ¿ÓÙ˜ Î·È Ù·Ú¿ÙÛ˜ (‡„Ô˜ ÙÔ˘Ï¿¯È-
ÛÙÔÓ 105 cm Î·È ·ÔÌ¿ÎÚ˘ÓÛË ÔÚÈ˙fiÓÙÈˆÓ ‰È·-
Û˘Ó‰¤ÛÂˆÓ Û ¯·ÌËÏ¿ ‡„Ë), ‰) Î¿Ï˘„Ë Î¿ıÂ
¯ÒÚÔ˘ Û˘ÏÏÔÁ‹˜ ‡‰·ÙÔ˜ (ËÁ¿‰È·, ÛÙ¤ÚÓ˜,
ÈÛ›Ó˜), Â) ·ÔÎÏÂÈÛÌfi˜ Ù˘ ÚfiÛ‚·Û˘ ÚÔ˜
ÙËÓ Ù·Ú¿ÙÛ· (fi¯È fï˜ Ì ÏԢΤÙÔ, ηıÒ˜ Ú¤-
ÂÈ Ó· ÏÂÈÙÔ˘ÚÁ› Î·È ˆ˜ ‰›Ô‰Ô˜ ‰È·Ê˘Á‹˜ ÛÂ
ÂÚ›ÙˆÛË ˘ÚηÁÈ¿˜), ÛÙ) ÙÔÔı¤ÙËÛË ·˘Ùfi-
Ì·ÙÔ˘ ‰È·ÎfiÙË ÛÙÔÓ ›Ó·Î· ÙÔ˘ ËÏÂÎÙÚÈÎÔ‡
ÁÈ· ÙËÓ ÚfiÏË„Ë ËÏÂÎÙÚÔÏËÍ›·˜ Î·È ˙) ‰˘Ó·-
ÙfiÙËÙ· Ù·ÎÙÈÎÔ‡ ÂϤÁ¯Ô˘ Ù˘ ‰È·Ê˘Á‹˜ ·ÂÚ›Ô˘.
ŸÛÔÓ ·ÊÔÚ¿ Û ÈÔ Ú·ÎÙÈο-ηıËÌÂÚÈÓ¿
˙ËÙ‹Ì·Ù·: ·) ÔÙ¤ ‰ÂÓ ÂÈÌË·ÓÔ˘Ì ÙÔ ÎÔÓÙfi
ηÏÒ‰ÈÔ ÙÔ˘ Ù·¯˘‚Ú·ÛÙ‹Ú·, ¿ÓÙ· ÙÔÔıÂÙԇ̠ÙÔ Î·¿ÎÈ ÚÈÓ, ηٿ ÙË ‰È¿ÚÎÂÈ· Î·È ÌÂÙ¿ ÙË ¯Ú‹ÛË ÙÔ˘, ÂÓÒ ÙÔÔıÂÙԇ̠ÙÔÓ Ù·¯˘‚Ú·ÛÙ‹Ú· ηٿ ÚÔÙ›ÌËÛË Û „ËÏfi, ÌË-ÚÔÛ‚¿ÛÈÌÔ ÁÈ· Ù· ·È‰È¿ ÛËÌ›Ô, ‚) ‰ÂÓ ¯ÚËÛÈÌÔÔÈԇ̠ÛÙ· ÔχÚÈ˙· ÌÂÁ¿ÏÔ ·ÚÈıÌfi Û˘Û΢ÒÓ (ı¤ÚÌ·ÓÛË ÙÔ˘ ηψ‰›Ô˘, Èı·Ófi ‚Ú·¯˘Î‡Îψ̷), Á) ÂÈıˆÚԇ̠ٷ ηÏ҉ȷ (ȉȷ›ÙÂÚ· fiÛ· ‚Ú›ÛÎÔÓÙ·È ÛÙÔ ¿ÙˆÌ·) ÁÈ· ÛËÌ›· ÊıÔÚ¿˜ Î·È fiÙ·Ó ÎÚÈı› ·Ó·Áη›Ô Ù· ·ÏÏ¿˙Ô˘Ì (‰ÂÓ Ù· ÂȉÈÔÚıÒÓÔ˘ÌÂ), ‰) ‰›ÓÔ˘Ì ÌÂÁ¿ÏË
ÚÔÛÔ¯‹ ÛÙË ıÂÚÌÔÎÚ·Û›· ÙÔ˘ ÓÂÚÔ‡ Ô˘ ı· ¯ÚËÛÈÌÔÔÈËı› ÁÈ· ÙÔ Ì¿ÓÈÔ ÙÔ˘ ·È‰ÈÔ‡ (¿ÓÙ· ÚÔËÁÂ›Ù·È ¤ÏÂÁ¯Ô˜ Ì ÙÔ ¯¤ÚÈ Ì·˜, ÙÔ ÔÔ›Ô ·Ê‹ÓÔ˘Ì ·ÚÎÂÙ‹ ÒÚ· ̤۷ ÛÙÔ ÓÂÚfi), Â) ·ÔÌ·ÎÚ‡ÓÔ˘Ì οı ›‰Ô˘˜ ËÏÂÎÙÚÈΤ˜ Û˘Û΢¤˜ ·fi ÙÔ Ì¿ÓÈÔ, ÛÙ) ÙÔÔıÂÙԇ̠·˘ÙÔÎfiÏÏËÙ· ÛÙ· Ù˙¿ÌÈ· ÛÙÔ ‡„Ô˜ ÙˆÓ Ì·ÙÈÒÓ ÙÔ˘ ·È‰ÈÔ‡, ÂÓÒ ˙) Û¯ÂÙÈο Ì ÙË ¯Ú‹ÛË ÙÔ˘ ·ÓÂÏ΢ÛÙ‹Ú·, Ì·ı·›ÓÔ˘Ì ÛÙÔ ·È‰› ÙË ¯Ú‹ÛË ÙÔ˘ ÎÔ˘‰Ô˘ÓÈÔ‡ Û˘Ó·ÁÂÚÌÔ‡ Î·È ÙÔ ·ÔÙÚ¤Ô˘ÌÂ
¤ÓÙÔÓ· ·fi ÙÔ Ó· ·›˙ÂÈ Ì ÙÔ ÎÔ˘Ì› STOP,
·Ú¿ ÌfiÓÔ Â¿Ó ˘¿ÚÍÂÈ ·Ó¿ÁÎË (.¯. “È·ÛÙ›”
fi‰È ‹ ¯¤ÚÈ) (2,63,64).
∞‰È·ÌÊÈÛ‚‹ÙËÙ·,
ˆÛÙfiÛÔ, ·ÚÈÔ˜ ·Ú¿ÁÔÓÙ·˜ ÁÈ· ÙËÓ ·ÔÙÚÔ‹ ·Ù˘¯ËÌ¿ÙˆÓ Â›Ó·È Ë ·Ó·‰È·ÌfiÚʈÛË Ù˘ ·ÓıÚÒÈÓ˘ Û˘ÌÂÚÈÊÔÚ¿˜ (Ë
ÌÂÙ·‚ÔÏ‹ Ù˘ ÓÔÔÙÚÔ›·˜ ÙÔ˘ ÏËı˘ÛÌÔ‡) ηÈ
Ô ÙÚfiÔ˜ Ô˘ ·˘Ù‹ ÌÔÚ› Ó· ‰È·ÙËÚËı› ÛÙÔÓ
¯ÚfiÓÔ: ·) Ì ‰È·Ú΋ ÂÓË̤ڈÛË, ‚) Ì ‰È·ÚΛ˜
ÂϤÁ¯Ô˘˜ ÂÊ·ÚÌÔÁ‹˜ ÙˆÓ ÓÔÌÔıÂÙËÌ¿ÙˆÓ, Á) ÌÂ
Û˘Ó¯‹ Î·È ·‰È¿ÎÚÈÙË (¯ˆÚ›˜ ÂÍ·ÈÚ¤ÛÂȘ) ηٿ
ÙÔÓ ÓfiÌÔ ÂÈ‚ÔÏ‹ ΢ÚÒÛÂˆÓ Î·È ‰) ÌÂ Û˘ÓÂȉËÙÔÔ›ËÛË Ù˘ Èı·ÓfiÙËÙ·˜ Ó· ˘Ô‚·ıÌÈÛÙ› - ‹ ·ÎfiÌ· Î·È Ó· ÛÙÂÚËı› - Ë ÔÈfiÙËÙ· ˙ˆ‹˜ ÂÓfi˜
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∞ı‹Ó·: ∂ıÓÈÎfi & ∫·Ô‰ÈÛÙÚÈ·Îfi ¶·ÓÂÈÛÙ‹ÌÈÔ ∞ıËÓÒÓ; 1996.
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Harvilchuck JD. In-home injury prevention practices for infants and toddlers: the role of parental beliefs, barriers, and housing quality. Health Educ Q 1995;22:85-95.
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1 ∂ÚÁ·ÛÙ‹ÚÈÔ ÀÁÈÂÈÓ‹˜,
π·ÙÚÈÎfi ∆Ì‹Ì·
¶·ÓÂÈÛÙËÌ›Ô˘ ¶·ÙÚÒÓ
2 ™¯ÔÏ‹ ∂·ÁÁÂÏÌ¿ÙˆÓ
ÀÁ›·˜, ∞.∆.∂.π. ¶·ÙÚÒÓ
3 ∂ÚÁ·ÛÙ‹ÚÈÔ º˘ÛÈÔÏÔÁ›·˜,
π·ÙÚÈÎfi ∆Ì‹Ì·, ¢ËÌÔÎÚ›ÙÂÈÔ ¶·ÓÂÈÛÙ‹ÌÈÔ
£Ú¿Î˘
4 ¶·ıÔÏÔÁÈ΋ ∫ÏÈÓÈ΋, “∫·Ú·Ì·Ó‰¿ÓÂÈÔ” °ÂÓÈÎfi ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ
¶·ÙÚÒÓ
AÏÏËÏÔÁÚ·Ê›·:
πˆ¿ÓÓ˘ ¢ÂÙÔÚ¿Î˘
∂ÚÁ·ÛÙ‹ÚÈÔ ÀÁÈÂÈÓ‹˜,
π·ÙÚÈÎfi ∆Ì‹Ì·
¶·ÓÂÈÛÙËÌ›Ô˘ ¶·ÙÚÒÓ
∆.∫. 265 00
∂-mail: pbhealth@med. upatras.gr
∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 05-07-2005
∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 28-03-2006
∞Ù˘¯‹Ì·Ù· Û ·È‰È¿ Û¯ÔÏÈ΋˜ ËÏÈΛ·˜ ÛÙËÓ
fiÏË
∞. ∞ÏÂÍfiÔ˘ÏÔ˜1, π. ¢ÂÙÔÚ¿Î˘1, ∂. ¢ÂÙÔÚ¿Î˘2, Ÿ. ¶·ÁˆÓÔÔ‡ÏÔ˘3, °. ∫ÚÔÎȉ¿˜4
¶ÂÚ›ÏË„Ë
1 Laboratory of Public Health, Medical School, University of Patras
2 School of Health Professions, Technical Institute of Patras
3 Laboratory of Physiology, Medical School, Democritus University of Thrace
4 ”Karamandanio” Children’s Hospital
2003
∂ÈÛ·ÁˆÁ‹: ™ÎÔfi˜ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ ‹Ù·Ó Ë Îˆ‰ÈÎÔÔ›ËÛË Î·È ·Ó¿Ï˘ÛË ÙˆÓ ÛÙÔȯ›ˆÓ ·fi Ù· ·Ú¯Â›· ·È‰ÈÎÒÓ ·Ù˘¯ËÌ¿ÙˆÓ ÙˆÓ Â͈ÙÂÚÈÎÒÓ È·ÙÚ›ˆÓ ÙÔ˘ “∫·Ú·Ì·Ó‰¿ÓÂÈÔ˘” ¡ÔÛÔÎÔÌ›Ԣ ¶·›‰ˆÓ ¶·ÙÚÒÓ, ÚÔÎÂÈ̤ÓÔ˘ Ó· ‰ÈÂÚ¢ÓËıÔ‡Ó Ù· ÂȉËÌÈÔÏÔÁÈο ÙÔ˘˜ ¯·Ú·ÎÙËÚÈÛÙÈο, Ì ÛÙfi¯Ô ÙË ‰È¢ÎÚ›ÓËÛË ÙˆÓ ·ÈÙÈÒÓ Ô˘ Ù· ÚÔηÏÔ‡Ó Î·È ÙË Ï‹„Ë ÚÔÏËÙÈÎÒÓ Ì¤ÙÚˆÓ. ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ: ÃÚËÛÈÌÔÔÈ‹ıËÎ·Ó Ù· ·Ú¯Â›· ηٷÁÚ·Ê‹˜ ÂÂÈÁfiÓÙˆÓ ÂÚÈÛÙ·ÙÈÎÒÓ ÙˆÓ Â͈ÙÂÚÈÎÒÓ È·ÙÚ›ˆÓ ÙÔ˘ “∫·Ú·Ì·Ó‰¿ÓÂÈÔ˘” ¡ÔÛÔÎÔÌ›Ԣ ¶·ÙÚÒÓ, ÙˆÓ ÂÙÒÓ 2000 Î·È 2003. ∏ ÛÙ·ÙÈÛÙÈ΋ ÂÂÍÂÚÁ·Û›·
Ì ÙË ‰ÔÎÈÌ·Û›· x2 Î·È ÙË ‰ÔÎÈÌ·Û›· Ù˘ ·Ó¿Ï˘Û˘ ηٿ Û˘ÛÙ¿‰Â˜. ∞ÔÙÂϤÛÌ·Ù·: ∞fi ÙȘ 2820 ÂÚÈÙÒÛÂȘ, ÔÛÔÛÙfi 62,1% ·ÊÔÚ¿ Û ·ÁfiÚÈ· Î·È 37,9% Û ÎÔÚ›ÙÛÈ· Û¯ÔÏÈ΋˜ ËÏÈΛ·˜ (6-12 ÂÙÒÓ). ∆· ·Ù˘¯‹Ì·Ù· ·ÊÔÚÔ‡Û·Ó Û ÙÒÛÂȘ (66,7%), ·Ù˘¯‹Ì·Ù· Ì ÌÂÙ·ÊÔÚÈÎfi ̤ÛÔ (15,4%), ‰ËÏËÙËÚÈ¿ÛÂȘ (0,43%), ËÏÂÎÙÚÔÏË͛˜ (0,4%) Î·È ÂÁη‡Ì·Ù· (0,2%). ∆· ÙÚ·‡Ì·Ù· ÂÓÙÔ›˙ÔÓÙ·Ó Î˘Ú›ˆ˜ ÛÙ· ¿Óˆ Î·È Î¿Ùˆ ¿ÎÚ· (29,3% Î·È 22%, ·ÓÙ›ÛÙÔȯ·), ηıÒ˜ Î·È ÛÙËÓ ÎÂÊ·Ï‹ (20%). ∆· ·Ù˘¯‹Ì·Ù· Û˘Ì‚·›ÓÔ˘Ó Î˘Ú›ˆ˜ ÛÙÔ Û›ÙÈ (36,1%), Û ¯ÒÚÔ˘˜ ̤۷ Î·È Á‡Úˆ ·fi ÙÔ Û¯ÔÏÂ›Ô (14,3%), Û ·È‰ÈΤ˜ ¯·Ú¤˜ (13,5%) ‹ ÛÙÔÓ ‰ÚfiÌÔ (10,9%), Û˘Ó‹ıˆ˜ ˘fi ÙËÓ Â›‚ÏÂ„Ë ÁÔÓ¤ˆÓ ‹ ÎˉÂÌfiÓˆÓ (67,4%). ∏ ·Ó¿Ï˘ÛË Î·Ù¿ Û˘ÛÙ¿‰Â˜ ·Ó¤‰ÂÈÍ ٤ÛÛÂÚȘ ÔÌ¿‰Â˜, Ì ÔÛÔÛÙ¿ Û˘ÌÌÂÙÔ¯‹˜ 37,7%, 39,5%, 10,2% Î·È 12,7% ·ÓÙ›ÛÙÔȯ·, Ô˘ ‰È·ÊÔÚÔÔÈÔ‡Ó Ù· ·Ù˘¯‹Ì·Ù· Ì ‚¿ÛË ÙÔÓ ÙfiÔ, ÙËÓ ËÏÈΛ·, ÙÔ Â›‰Ô˜, ÙËÓ ÂÓÙfiÈÛË, ·ÏÏ¿ Î·È ÙÔ˘˜ ·˘ÙfiÙ˜ Ì¿ÚÙ˘Ú˜. √ ̤ÛÔ˜ ·ÚÈıÌfi˜ ÙˆÓ ÂÚÈÛÙ·ÙÈÎÒÓ ÙÔ ¤ÙÔ˜ 2000 ‹Ù·Ó 3,5 ·Ó¿ Ë̤ڷ, ÂÓÒ ÙÔ 2003 ‹Ù·Ó 4,2 ·Ó¿ Ë̤ڷ, ·˘ÍË̤ÓÔ˜ ηٿ 18%. ∏ ‰È·ÊÔÚ¿ ·˘Ù‹ ·ÊÔÚÔ‡Û ΢ڛˆ˜ Û ·‡ÍËÛË ÙˆÓ ·Ù˘¯ËÌ¿ÙˆÓ Ô˘ Û˘Ó¤‚ËÛ·Ó ÛÙÔÓ ‰ÚfiÌÔ (·‡ÍËÛË 46,4%) Î·È ÛÙÔ Û›ÙÈ (·‡ÍËÛË 28,8%), ÂÓÒ ·ÍÈÔÛËÌ›ˆÙË ‹Ù·Ó Ë Ì›ˆÛË ÙˆÓ ·Ù˘¯ËÌ¿ÙˆÓ ÛÙ· Û¯ÔÏ›· ÙËÓ ›‰È· ÂÚ›Ô‰Ô (Ì›ˆÛË 25,3%).
™˘ÌÂÚ¿ÛÌ·Ù·: ∆· Â˘Ú‹Ì·Ù· Ù˘ ÌÂϤÙ˘ ÂȂ‚·ÈÒÓÔ˘Ó ·ÓÙ›ÛÙÔȯ· Â˘Ú‹Ì·Ù· ¿ÏÏˆÓ ÂÚ¢ÓÒÓ, Û‡Ìʈӷ Ì ٷ ÔÔ›· Ë Û˘¯ÓfiÙËÙ· ÙˆÓ ·Ù˘¯ËÌ¿ÙˆÓ ‰È·ÊÔÚÔÔÈÂ›Ù·È ·Ó¿ÏÔÁ· Ì ÙÔ Ê‡ÏÔ, ÙËÓ ËÏÈΛ·, ÙÔÓ ÙfiÔ Î·È ÙËÓ ÂÔ¯‹ ÙÔ˘ ¤ÙÔ˘˜ Ô˘ ·˘Ù¿ Û˘Ó¤‚ËÛ·Ó. ¶·Ú¿ ÙÔ ÁÂÁÔÓfi˜ fiÙÈ Ë Ê‡ÛË ÙˆÓ ·È‰ÈÎÒÓ ·Ù˘¯ËÌ¿ÙˆÓ ‰‡ÛÎÔÏ· ÂÈÙÚ¤ÂÈ ÙËÓ ÂÍ·ÁˆÁ‹ ·ÛÊ·ÏÒÓ Û˘ÌÂÚ·ÛÌ¿ÙˆÓ, Ë ÁÂÓÈ΋ ÂÔ›ıËÛË Â›Ó·È fiÙÈ Û ÌÂÁ¿ÏÔ ‚·ıÌfi ÌÔÚÔ‡Ó Ó· ÚÔ‚ÏÂÊıÔ‡Ó Î·È Ó· ÂÚÈÔÚÈÛÙÔ‡Ó, ÙÔ˘Ï¿¯ÈÛÙÔÓ ÛÙ· ›‰· ¿ÏÏˆÓ ¯ˆÚÒÓ, Ì ÙË Ï‹„Ë ÙˆÓ Î·Ù¿ÏÏËÏˆÓ Ì¤ÙÚˆÓ.
ÎÏÂȉȿ
Accidents in children 6-12 years old in Patras during the years 2000 and 2003
A. Alexopoulos1, I. Detorakis1, E. Detorakis2, O. Pagonopoulou3, G. Krokidas4
Abstract
Background: The aim of this study was the evaluation of key epidemiological characteristics of children’s accidents in a major Greek city.
Methods: All available records concerning children’s injuries from the outpatient department of the “Karamandanio” Children’s Hospital in Patras, Greece, were recoded and analyzed, using descriptive statistics and chi-square and hierarchical cluster analysis.
Results: A total of 2,820 cases covering the years 2000 and 2003, were included, of which 62.1% involved boys and 37.9% girls aged 6 to 12 years. Fall related injuries (66.7%) predominated followed by traffic
¶·È‰È·ÙÚÈ΋
accidents (15.4%), poisoning (0.43%), electric shock (0.4%) and burns (0.2%). Most of the trauma involved the upper and lower limbs (29.3% and 22%, respectively) and the head (20%). In 36.1% of cases the accident occurred at home, 14.3% at school (inside or outside), 13.5% in public playgrounds and 10.9% in the street, and usually the parents were present (67.4%). Hierarchical cluster analysis using sex, place, age, type, localization and presence of eye witness as variables, revealed four discrete clusters which accounted for 37.7%, 39.5%, 10.2% and 12.7% of all injuries respectively. In the year 2000 there were 3.5 admissions per day, while in 2003 there was an increase to 4.2 per day, mainly as a result of an increase in street accidents (by 46.6%) and home accidents (by 28.8%). Conversely there was a notable decrease in accidents occurring at school (by 25.3%). All the other findings of the study were in accordance with earlier reports on the different frequencies of injuries according to the sex, the age group and the season.
Conclusions: This retrospective study confirms that the majority of childhood accidents are environment related, and therefore theoretically preventable when sound strategies are implemented.
Key words: Child injury, prevention, injury epidemiology.
ÙËÓ Î‡ÚÈ· ·ÈÙ›· ı·Ó¿ÙÔ˘ ·È‰ÈÒÓ ÛÙȘ ¯ÒÚ˜ Ù˘ ∂˘Úˆ·˚΋˜ ŒÓˆÛ˘ (1-4).
∏ ‰ÈÂÚ‡ÓËÛË ÙˆÓ ÂȉËÌÈÔÏÔÁÈÎÒÓ ¯·Ú·ÎÙËÚÈÛÙÈÎÒÓ ÙˆÓ ·Ù˘¯ËÌ¿ÙˆÓ Û˘Ì‚¿ÏÏÂÈ ÛÙË
‰È¢ÎÚ›ÓËÛË ÙˆÓ ·ÈÙ›ˆÓ Ô˘ Ù· ÚÔηÏÔ‡Ó
Î·È ÙË Ï‹„Ë Ì¤ÙÚˆÓ ÚfiÏË„‹˜ ÙÔ˘˜. ¶ÚÔÛ¿ıÂȘ ÚÔ˜ ·˘Ù‹ ÙËÓ Î·Ù‡ı˘ÓÛË ‚Ú›ÛÎÔÓÙ·È Û ÂͤÏÈÍË ÙËÓ ÙÂÏÂ˘Ù·›· ‰ÂηÂÙ›· Û ·-
ÓÂ˘Úˆ·˚Îfi, ·ÏÏ¿ Î·È ÂıÓÈÎfi ›‰Ô, fiˆ˜
Â›Ó·È ÁÈ· ·Ú¿‰ÂÈÁÌ· Ë ‚¿ÛË ‰Â‰Ô̤ӈÓ
EDISS, ÙËÓ ÔÔ›· ‰ËÌÈÔ‡ÚÁËÛÂ Î·È ‰È·¯ÂÈÚ›˙ÂÙ·È ÙÔ ∫¤ÓÙÚÔ ŒÚ¢ӷ˜ Î·È ¶ÚfiÏ˄˘ ¶·È‰ÈÎÒÓ ∞Ù˘¯ËÌ¿ÙˆÓ (∫∂¶¶∞) Ì ·ÍÈÔÛËÌ›ˆÙ·
·ÔÙÂϤÛÌ·Ù· (5).
¶·ÚfiÌÔȘ ÂÚ¢ÓËÙÈΤ˜ ÚÔÛ¿ıÂȘ, ·ÎfiÌË Î·È Û ÌÈÎÚfiÙÂÚË Îϛ̷η, ı· ÌÔÚÔ‡Û·Ó Ó· Û˘Ì‚¿ÏÔ˘Ó ÛÙËÓ ÂÚ·ÈÙ¤Úˆ ηٷÓfiËÛË ÙÔ˘ ȉȷ›ÙÂÚÔ˘ ·˘ÙÔ‡ ÚÔ‚Ï‹Ì·ÙÔ˜ (6) ηÈ
ÛÙËÓ ·‡ÍËÛË Ù˘ ¢·ÈÛıËÛ›·˜ ÙˆÓ ·ÚÌfi‰ÈˆÓ ·Ú¯ÒÓ ÁÈ· ÙËÓ Â͇ÚÂÛË Ï‡ÛÂˆÓ Ì¤Ûˆ ηٿÏÏËÏˆÓ ·ÚÂÌ‚¿ÛÂˆÓ ÚfiÏ˄˘.
ÙˆÓ ÂÙÒÓ 2000 Î·È 2003, Ù· ÔÔ›· ‹Ù·Ó ‰È·ı¤ÛÈÌ· Î·È Ù· ÔÔ›· Ï¿‚·Ì ÌÂÙ¿ ·fi Û¯ÂÙÈ΋ ¿‰ÂÈ· Ù˘ ‰ÈÔ›ÎËÛ˘ ÙÔ˘ π‰Ú‡Ì·ÙÔ˜. √È Î·Ù·Áڷʤ˜ ·˘Ù¤˜ Έ‰ÈÎÔÔÈ‹ıËÎ·Ó Î·Ù¿ÏÏËÏ· Î·È ÂÈÛ‹¯ıËÛ·Ó Û ËÏÂÎÙÚÔÓÈ΋ ‚¿ÛË ÚÔ˜ ÂÂÍÂÚÁ·Û›·. √È ÌÂÙ·‚ÏËÙ¤˜ Ô˘ ÂÚÈÂÏ¿Ì‚·Ó·Ó Ù· ·Ú¯Â›· ·˘Ù¿ ·ÊÔÚÔ‡Û·Ó ÙfiÛÔ Û ·ÙÔÌÈο ·ÓÙÈÎÂÈÌÂÓÈο ¯·Ú·ÎÙËÚÈÛÙÈο ÙÔ˘ οı ·È‰ÈÔ‡ (ʇÏÔ, ËÏÈΛ·, Ù¿ÍË Û¯ÔÏ›Ԣ), fiÛÔ Î·È Û ‰Â‰Ô̤ӷ ÙÔ˘ ÔÈÎÔÁÂÓÂÈ·ÎÔ‡ ÙÔ˘ ÂÚÈ‚¿ÏÏÔÓÙÔ˜ (ÔÈÎÔÁÂÓÂȷ΋ ηٿÛÙ·ÛË Î·È ÌÔÚʈÙÈÎfi Â›Â‰Ô ÁÔÓ¤ˆÓ, ‡·ÚÍË ‹ ÌË ·‰ÂÏÊÒÓ, ··Û¯fiÏËÛË ÌËÙ¤Ú·˜, ÛÂÈÚ¿ Á¤ÓÓËÛ˘ ÙÔ˘ ·È‰ÈÔ‡), Û ¯·Ú·ÎÙËÚÈÛÙÈο ÙÔ˘ ·Ù˘¯‹Ì·ÙÔ˜ (ËÌÂÚÔÌËÓ›·, ÙfiÔ˜, ÒÚ·, ›‰Ô˜ ‹ ı¤ÛË Î¿ÎˆÛ˘), ηıÒ˜ Î·È Û ÔÚÈṲ̂ӷ ˘ÔÎÂÈÌÂÓÈο ÛÙÔȯ›· Ù· ÔÔ›· ·Ó·Ê¤ÚıËÎ·Ó ·fi ÙÔÓ ÁÔÓ¤· ‹ ¿ÏÏÔ Û˘ÓÔ‰fi ÙÔ˘ ·È‰ÈÔ‡ ηٿ ÙËÓ ÚÔۤϢÛË (¯·Ú·ÎÙËÚÈÛÙÈο Û˘ÌÂÚÈÊÔÚ¿˜ ÙÔ˘ ·È‰ÈÔ‡, ÁÂÓÈ΋ Û¯ÔÏÈ΋ ›‰ÔÛË, Û¯¤ÛË Ì ÙÔ ·È‰› fiÛˆÓ ‹Ù·Ó ·ÚfiÓÙ˜ ÛÙÔ ·Ù‡¯ËÌ·, ÈÛÙÔÚÈÎfi ·Ù˘¯‹Ì·ÙÔ˜/·Ù˘¯ËÌ¿ÙˆÓ Ô˘ ¯ÚÂÈ¿ÛÙËÎÂ/·Ó ›Û˘ È·ÙÚÈ΋ ÊÚÔÓÙ›‰· ηٿ ÙÔ ·ÚÂÏıfiÓ). ∆· ÙÂÏÂ˘Ù·›· ÛÙÔȯ›· ıˆڋıËÎ·Ó ˘ÔÎÂÈÌÂÓÈο, ‰Â‰Ô̤ÓÔ˘ fiÙÈ ·Ë¯Ô‡Û·Ó ÙËÓ ÚÔÛˆÈ΋ ÁÓÒÌË ÙÔ˘ Û˘ÓÔ‰Ô‡ Î·È Ë ‰È·ÛÙ·‡ÚˆÛË Ù˘ ·ÎÚ›‚ÂÈ¿˜ ÙÔ˘˜ ‰ÂÓ ‹Ù·Ó ‰˘Ó·Ù‹. ∆Ô ÌÔÚʈÙÈÎfi Â›Â‰Ô ÙˆÓ ÁÔÓ¤ˆÓ Έ‰ÈÎÔÔÈ‹ıËΠ·Ó¿ÏÔÁ· Ì ÙÔ ·ÔÏ˘Ù‹ÚÈÔ Ô˘ ·˘ÙÔ› ηÙ›¯·Ó. ™˘ÓÔÏÈο, Î·È ÁÈ· Ù· ‰‡Ô ¤ÙË Û˘ÁÎÂÓÙÚÒıËÎ·Ó 2820 ηٷÁڷʤ˜, 1294 ÁÈ· ÙÔ ¤ÙÔ˜ 2000 Î·È 1526 ÁÈ· ÙÔ ¤ÙÔ˜ 2003. ¢˘ÛÙ˘¯Ò˜, ÚÔÁÂÓ¤ÛÙÂÚ˜ ‹ ¤ÛÙˆ ÂӉȿÌÂÛ˜ ηٷÁڷʤ˜ ‰ÂÓ ‹Ù·Ó ‰È·ı¤ÛÈ̘ ·fi Ù· ·Ú¯Â›· ÙÔ˘ ¡ÔÛÔÎÔÌ›Ԣ Î·È ‰ÂÓ ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È ÛÙËÓ ·ÚÔ‡Û· ÌÂϤÙË. ™ÙȘ 848 ÂÚÈÙÒÛÂȘ (30%) ÔÈ
Correspondence: Ioannis Detorakis Laboratory of Public Health Medical School, University of Patras 26500, Patras Greece E-mail: pbhealth@med.upatras.gr
Date of submission: 05-07-2005
Date of approval: 28-03-2006
Î·È fiÏ· Ù· ·È‰È¿ ÙˆÓ ÔÔ›ˆÓ Ë ËÏÈΛ·
‹Ù·Ó ÌÈÎÚfiÙÂÚË ÙˆÓ 6 ‹ ÌÂÁ·Ï‡ÙÂÚË ÙˆÓ 12 ÂÙÒÓ.
√ ÛÙ·ÙÈÛÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ ·ÊÔÚÔ‡Û ÛÙȘ Û˘ÁÎÚ›ÛÂȘ
ÈÓ·ÎÔÔÈËÌ¤ÓˆÓ ÙÈÌÒÓ (›Ó·Î˜ Û˘Ó¿ÊÂÈ·˜) Ì ÙË
‰ÔÎÈÌ·Û›· x2 Î·È ÙËÓ ÔÌ·‰ÔÔ›ËÛË ÙˆÓ ÂÚÈÙÒÛˆÓ
ÁÈ· ÙËÓ ·Ó¿‰ÂÈÍË ‰È·ÎÚÈÙÒÓ ÔÌ¿‰ˆÓ Ì ÎÔÈÓ¿ ¯·Ú·ÎÙË-
ÚÈÛÙÈο Ì ÙË ‰ÔÎÈÌ·Û›· ·Ó¿Ï˘Û˘ ηٿ Û˘ÛÙ¿‰Â˜ (ÈÂÚ·Ú¯È΋ Ù·ÍÈÓfiÌËÛË). ∆fiÛÔ Ë ÂÚÈÁÚ·ÊÈ΋ fiÛÔ Î·È Ë
ÂÎÙÈÌËÙÈ΋ ÛÙ·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË Ú·ÁÌ·ÙÔÔÈ‹ıËηÓ
Ì ÙÔ ÏÔÁÈÛÌÈÎfi ·Î¤ÙÔ SPSS (v12, 2004).
∞ÔÙÂϤÛÌ·Ù·
∞fi ÙȘ 2820 ÂÚÈÙÒÛÂȘ, ÔÈ 1751 (62,1%)
·ÊÔÚÔ‡Û·Ó Û ·ÁfiÚÈ· Î·È ÔÈ 1069 (37,9%) ÛÂ
ÎÔÚ›ÙÛÈ· ËÏÈΛ·˜ 6-12 ÂÙÒÓ. ™ÙÔÓ ¶›Ó·Î· 1 ·-
ÚÔ˘ÛÈ¿˙ÔÓÙ·È Ù· ÎÔÈÓˆÓÈÎÔ-‰ËÌÔÁÚ·ÊÈο ¯·-
Ú·ÎÙËÚÈÛÙÈο ÙˆÓ ·Ù˘¯ËÌ·ÙÈÒÓ, fiˆ˜ Ë ËÏÈΛ·,
ÙÔ Ê‡ÏÔ Î·ıÒ˜ Î·È Ù· ¯ÚÔÓÈο ‰Â‰Ô̤ӷ ÙÔ˘
ÂÚÈÛÙ·ÙÈÎÔ‡. º·›ÓÂÙ·È fiÙÈ Ù· ÂÚÈÛÛfiÙÂÚ· ·fi ·˘Ù¿ ·ÊÔÚÔ‡Ó Û ·ÁfiÚÈ·, Ì ·È¯Ì¤˜ ÙȘ
ËÏÈ˘ ÙˆÓ 6, 10 Î·È 12 ÂÙÒÓ. ∆Ô 2000 Ë Ì¤ÛË
ËÌÂÚ‹ÛÈ· Û˘¯ÓfiÙËÙ· ÂÚÈÛÙ·ÙÈÎÒÓ ‹Ù·Ó
3,5/Ë̤ڷ, ÂÓÒ ÙÔ 2003 ÂÌÊ·Ó›˙ÂÙ·È ·˘ÍË̤ÓË Û 4,2/Ë̤ڷ (+18%). ™¯Â‰fiÓ ¤Ó· ÛÙ· ÙÚ›· ·Ù˘¯‹Ì·Ù· Û˘Ó¤‚Ë Î·Ù¿ ÙÔ˘˜ ·ÚÈÓÔ‡˜ Ì‹Ó˜ (30,3%) ηıÒ˜ Î·È ÙȘ ·ÔÁÂ˘Ì·ÙÈÓ¤˜ ÒÚ˜ (29,1%), ÂÓÒ ‰ÂÓ ·ÚÔ˘ÛÈ¿˙ÂÙ·È È‰È·›ÙÂÚË Û˘ÛÛÒÚ¢ÛË ·Ù˘¯ËÌ¿ÙˆÓ Û οÔÈ· ·fi ÙȘ Ë̤Ú˜ Ù˘ ‚‰ÔÌ¿‰·˜. ŸÛÔÓ ·ÊÔÚ¿ ÛÙÔ Â›‰Ô˜ ÙÔ˘ ·Ù˘¯‹Ì·ÙÔ˜ (¶›Ó·Î·˜ 2), ·˘Ù¿ ÛÙÔ ÌÂÁ·Ï‡ÙÂÚÔ ÔÛÔÛÙfi ÙÔ˘˜
·ÊÔÚÔ‡Ó Û ÙÒÛÂȘ (66,7%), Ì ٷ ·Ù˘¯‹Ì·Ù·
·fi ÌÂÙ·ÊÔÚÈÎfi ̤ÛÔ Ó· ¤ÔÓÙ·È (15,4%). ªÈ-
ÎÚfiÙÂÚ· ÔÛÔÛÙ¿ ·È‰ÈÒÓ ˘¤ÛÙËÛ·Ó ·Ù˘¯‹Ì·Ù· Ô˘ ÔÊ›ÏÔÓÙ·Ó Û ‰ËÏËÙËÚÈ¿ÛÂȘ (0,43%),
ËÏÂÎÙÚÔÏË͛˜ (0,4% ) Î·È ÂÁη‡Ì·Ù· ΢ڛˆ˜ ·fi ¯Ú‹ÛË ‚ÂÁÁ·ÏÈÎÒÓ (0,20%). ∏ ÂÓÙfiÈÛË ÙÔ˘
ÙÚ·‡Ì·ÙÔ˜ ·ÊÔÚÔ‡Û ΢ڛˆ˜ Û ηÎÒÛÂȘ ÛÙ·
¿Óˆ Î·È Î¿Ùˆ ¿ÎÚ· (29,3% Î·È 22,0%, ·ÓÙ›ÛÙÔȯ·), ηıÒ˜ Î·È ÛÙËÓ ÎÂÊ·Ï‹ (20,0%). ™ÙÔÓ ¶›Ó·Î· 3 ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È ÔÚÈṲ̂ӷ ·fi Ù· ¯·Ú·ÎÙËÚÈÛÙÈο ÙÔ˘ ‰Â›ÁÌ·ÙÔ˜, Ù·
ÔÔ›· ıˆÚÔ‡ÓÙ·È ˆ˜ Èı·ÓÔ› ·Ú¿ÁÔÓÙ˜ ÎÈÓ-
¶›Ó·Î·˜ 1. ∫·Ù·ÓÔÌ‹ ÙˆÓ 2820 ÂÚÈÛÙ·ÙÈÎÒÓ ·È‰ÈÎÒÓ ·Ù˘¯ËÌ¿ÙˆÓ Ô˘ ηٷÁÚ¿ÊËÎ·Ó ÛÙ· Â͈ÙÂÚÈο È·ÙÚ›· ÙÔ˘ “∫·Ú·Ì·Ó‰¿ÓÂÈÔ˘” ¡ÔÛÔÎÔÌ›Ԣ ¶·›‰ˆÓ ¶·ÙÚÒÓ Ù· ¤ÙË 2000 Î·È 2003, Ì ‚¿ÛË ‰ËÌÔÁÚ·ÊÈο ¯·Ú·ÎÙËÚÈÛÙÈο ÙˆÓ ·È‰ÈÒÓ Î·È ¯ÚÔÓÈο ¯·Ú·ÎÙËÚÈÛÙÈο ÙÔ˘ ·Ù˘¯‹Ì·ÙÔ˜ ¶·Ú¿ÌÂÙÚÔ˜¡¶ÔÛÔÛÙfi (%)
∏ÏÈΛ· 6-9149052,8 10-12133047,2 ™‡ÓÔÏÔ 2820100,0
º‡ÏÔ
∞ÁfiÚÈ·175162,1 ∫ÔÚ›ÙÛÈ·106937,9 ™‡ÓÔÏÔ 2820100,0
ŒÙÔ˜ ·Ù˘¯‹Ì·ÙÔ˜ 2000129445,9 2003152654,1 ™‡ÓÔÏÔ 2820100,0
∂Ô¯‹ ÃÂÈÌÒÓ·˜67423,9 ÕÓÔÈÍË85530,4 ∫·ÏÔη›ÚÈ64722,9 ºıÈÓfiˆÚÔ64422,8 ™‡ÓÔÏÔ 2820100,0
∏̤ڷ ·Ù˘¯‹Ì·ÙÔ˜ ™·‚‚·ÙÔ·ÚÈ·ÎÔ71225,2 ∫·ıËÌÂÚÈÓ‹210874,8 ™‡ÓÔÏÔ 2820100,0
ÃÚÔÓÈ΋ ÛÙÈÁÌ‹ ¶Úˆ› (6:00-12:00)60121,3 ªÂÛË̤ÚÈ (12:00-17:00)77127,3
∞fiÁÂ˘Ì· (17:00-20:00)82029,1 µÚ¿‰˘ (20:00-6:00)62822,3 ™‡ÓÔÏÔ 2820100,0
ÂÚˆÙ‹ÛÂȘ, ıˆÚÂ›Ù·È fiÙÈ Â›Ó·È Èηӿ Ó· ·ÔÙÂϤÛÔ˘Ó ÙÔ˘Ï¿¯ÈÛÙÔÓ ÙË ‚¿ÛË ÁÈ· ÚÔ‚ÏËÌ·ÙÈÛÌfi. ŒÙÛÈ, ·Ú·ÙËÚÂ›Ù·È fiÙÈ ÌÂÁ¿ÏÔ ÔÛÔÛÙfi ÙÔ˘ ‰Â›ÁÌ·ÙÔ˜ ·ÊÔÚ¿ Û ·È‰È¿ ΢ڛˆ˜ ÙˆÓ ‰‡Ô ÚÒÙˆÓ Ù¿ÍÂˆÓ ÙÔ˘ ‰ËÌÔÙÈÎÔ‡ (41,2%), ÙˆÓ ÔÔ›ˆÓ ÔÈ ÁÔÓ›˜ Â›Ó·È ·fiÊÔÈÙÔÈ ÚˆÙÔ‚¿ıÌÈ·˜ ‹ ‰Â˘ÙÂÚÔ‚¿ıÌÈ·˜ Âη›‰Â˘Û˘ Î·È ÔÈ ÌËÙ¤Ú˜ ÙÔ˘˜ - ˆ˜ › ÙÔ Ï›ÛÙÔÓ (71,8%)ÂÚÁ·˙fiÌÂÓ˜. ∞Ó Î·È ‰ÂÓ ·ÔÙÂÏ› ·ÍÈfiÈÛÙÔ ÛÙÔȯ›Ô, ÂÓÙ‡ˆÛË ÚÔηÏ› ÙÔ ÁÂÁÔÓfi˜ fiÙÈ Û ÔÛÔÛÙfi 73% ÔÈ Û˘ÓÔ‰Ô› ÙˆÓ ·È‰ÈÒÓ Â¤ÏÂÍ·Ó ÙË Ï¤ÍË “¿Ù·ÎÙÔ” ÁÈ· Ó· ÂÚÈÁÚ¿„Ô˘Ó ·‰Ú¿ ÙËÓ ÚÔÛˆÈÎfiÙËÙ· ÙÔ˘ ·È‰ÈÔ‡ Û ۯÂÙÈ΋ ÂÚÒÙËÛË Ô˘ ÙÔ˘˜ ›¯Â ÙÂı›. ™ÙËÓ ÏÂÈÔ„ËÊ›· ÙÔ˘˜ (77,6%) Ù· ·È‰È¿ ‰ÂÓ Â›¯·Ó ÈÛÙÔÚÈÎfi ÚÔËÁÔ‡ÌÂÓÔ˘ ÛÔ‚·ÚÔ‡ ·Ù˘¯‹Ì·ÙÔ˜. √ ÙÚ·˘Ì·ÙÈÛÌfi˜ ›¯Â Û˘Ì‚Â› ΢ڛˆ˜ ÛÙÔ Û›ÙÈ (36,1%), ·ÏÏ¿ Î·È Û ¯ÒÚÔ˘˜ ̤۷ Î·È Á‡Úˆ ·fi ÙÔ Û¯ÔÏÂ›Ô (14,3%), Û ·È‰ÈΤ˜ ¯·Ú¤˜ (13,5%) ‹ ÛÙÔÓ ‰ÚfiÌÔ (10,9%). øÛÙfiÛÔ, Û οı ÂÚ›ÙˆÛË Ë ÏÂÈÔ„ËÊ›· ÙˆÓ ÂÚÈÛÙ·ÙÈÎÒÓ ¶·È‰È·ÙÚÈ΋ 2006;69:278-285
ÌfiÓÔ Ô ¤Ó·˜ ÛÙÔ˘˜ ÙÚÂȘ Û˘ÓÔ‰Ô‡˜ ·¿ÓÙËÛ ÛÙȘ ·ÓÙ›ÛÙÔȯ˜
¶›Ó·Î·˜
∫·Ù·ÓÔÌ‹ ÙˆÓ 2820 ÂÚÈÛÙ·ÙÈÎÒÓ ·È‰ÈÎÒÓ ·Ù˘¯ËÌ¿ÙˆÓ Ô˘ ηٷÁÚ¿ÊËÎ·Ó ÛÙ· Â͈ÙÂÚÈο È·ÙÚ›· ÙÔ˘ “∫·-
Ú·Ì·Ó‰¿ÓÂÈÔ˘” ¡ÔÛÔÎÔÌ›Ԣ ¶·›‰ˆÓ ¶·ÙÚÒÓ Ù· ¤ÙË 2000
¶·Ú¿ÌÂÙÚÔ˜¡¶ÔÛÔÛÙfi (%)
∂›‰Ô˜ ·Ù˘¯‹Ì·ÙÔ˜
¶ÙÒÛË188066,67
ªÂÙ·ÊÔÚÈÎfi ̤ÛÔ43315,35
∞ȯÌËÚ¿ Î·È 2669,43
‰ÈÂÈÛ‰˘ÙÈο ·ÓÙÈΛÌÂÓ·
∂ÎÚËÎÙÈÎfi ˘ÏÈÎfi60,21
∫·˘Ù‹ ‡ÏË ‹ ·ÓÙÈΛÌÂÓÔ772,73
∏ÏÂÎÙÚÈÎfi Ú‡̷100,35
¢ËÏËÙËÚ›·ÛË ·fi Ê¿Ú̷η100,35
¢ËÏËÙËÚ›·ÛË ·fi ÙÚÔÊ‹10,04
¢‹ÁÌ· ˙ÒÔ˘1374,86
™‡ÓÔÏÔ 2820100,0
£¤ÛË/›‰Ô˜ ÙÚ·‡Ì·ÙÔ˜
∫ÂÊ·Ï‹56420,0
ÕÓˆ ¿ÎÚ·82529,26
∫¿Ùˆ ¿ÎÚ·61921,95
¶ÔÏÏ·Ïfi ÙÚ·‡Ì·822,91
∂Í¿ÚıÚËÌ·, ‰È¿ÛÙÚÂÌÌ·742,62
∞ÓÔȯÙfi ÙÚ·‡Ì·34612,27
ªÒψ˜923,26
ÕÏÏÔ2187,73
™‡ÓÔÏÔ 2820100,0
Û˘Ó¤‚Ë ·Ú¿ ÙËÓ Â›‚ÏÂ„Ë ÁÔÓ¤ˆÓ ‹ Û˘ÁÁÂÓÒÓ (67,4%).
∏ ·Ó¿Ï˘ÛË Î·Ù¿ Û˘ÛÙ¿‰Â˜ Ì ÈÂÚ·Ú¯È΋ Ù·-
ÍÈÓfiÌËÛË - ÁÈ· ÙȘ ÌÂÙ·‚ÏËÙ¤˜ fiÔ˘ ‹Ù·Ó ‰È·-
ı¤ÛÈÌ· Ï‹ÚË ‰Â‰Ô̤ӷ - ·¤‰ˆÛ ٤ÛÛÂÚȘ
ÔÌ¿‰Â˜ (¶›Ó·Î·˜ 4). ∏ ÚÒÙË (37,7%) ·ÊÔÚ¿
Û ·È‰È¿ (ËÏÈΛ·˜ 6-9 ÂÙÒÓ) ÛÙ· ÔÔ›· Ù· ·Ù˘-
¯‹Ì·Ù· Û˘Ó¤‚ËÛ·Ó Î˘Ú›ˆ˜ ÛÙÔ Û›ÙÈ Î·È ÂÚÈÂ-
Ï¿Ì‚·Ó·Ó ÙÒÛÂȘ, ÂÁη‡Ì·Ù·, ËÏÂÎÙÚÔÏË͛˜, ‰ËÏËÙËÚÈ¿ÛÂȘ Î·È ÙÚ·‡Ì·Ù· ·fi ·È¯ÌËÚ¿ ·ÓÙÈΛÌÂÓ· ΢ڛˆ˜ ˘fi ÙËÓ Â›‚ÏÂ„Ë ÁÔÓ¤ˆÓ ‹ Û˘ÁÁÂÓÒÓ, ¯ˆÚ›˜ Âԯȷ΋ ‰È·Î‡Ì·ÓÛË.
∆Ë ‰Â‡ÙÂÚË ÔÌ¿‰· (39,5%) ··ÚÙ›˙Ô˘Ó
·È‰È¿ ÌÂÁ·Ï‡ÙÂÚ˘ ËÏÈΛ·˜ (9-12 ÂÙÒÓ), Û˘Ó‹ıˆ˜ ·ÁfiÚÈ·, Ù· ÔÔ›· ÙÚ·˘Ì·Ù›˙ÔÓÙ·È Û ·È‰ÈΤ˜ ¯·Ú¤˜, ÛÙËÓ ‡·ÈıÚÔ ‹ Û ¿ÏÏÔ˘˜ ¯ÒÚÔ˘˜.
∂Ó ÚÔÎÂÈ̤ӈ, Ù· ÂÚÈÛÛfiÙÂÚ· ·Ù˘¯‹Ì·Ù·
ÚÔηÏÔ‡ÓÙ·È ·fi ÙÒÛÂȘ, ·fi ·È¯ÌËÚ¿ ‹ ‰ÈÂÈÛ‰˘ÙÈο ·ÓÙÈΛÌÂÓ· Î·È ·fi ‰‹ÁÌ·Ù· ˙ÒˆÓ ‹ ÂÓÙfïÓ. ∞˘Ù¿ Ê·›ÓÂÙ·È Ó· Û˘Ì‚·›ÓÔ˘Ó Î˘Ú›ˆ˜ ÙÔ Î·ÏÔη›ÚÈ Î·È ·ÚfiÓÙ˜ ÛÙÔ Û˘Ì‚¿Ó Â›Ó·È Û˘Ó‹ıˆ˜ ÙÚ›ÙÔÈ (.¯. Û˘ÓÔÌ‹ÏÈÎÔÈ) Î·È ÏÈÁfiÙÂÚÔ Û˘¯Ó¿ ÁÔÓ›˜ ‹ Û˘ÁÁÂÓ›˜. ∏ ÙÚ›ÙË ÔÌ¿‰· (10,2%) ·ÊÔÚ¿ Û ·Ù˘¯‹Ì·Ù· Ô˘ Û˘Ó¤‚ËÛ·Ó ÛÙÔÓ ‰ÚfiÌÔ Ì ÌÂÙ·ÊÔÚÈο ̤۷, ÙËÓ ¿ÓÔÈÍË ‹ ÙÔ Î·ÏÔη›ÚÈ, ·ÚÔ˘Û›· ÁÔÓ¤ˆÓ ‹ Û˘ÁÁÂÓÒÓ Î·È ·ÊÔÚÔ‡Ó Û ·È‰È¿ ËÏÈΛ·˜ 9-12
¶›Ó·Î·˜ 3. ∫·Ù·ÓÔÌ‹ ÙˆÓ 2820 ÂÚÈÛÙ·ÙÈÎÒÓ ·È‰ÈÎÒÓ ·Ù˘-
¯ËÌ¿ÙˆÓ Ô˘ ηٷÁÚ¿ÊËÎ·Ó ÛÙ· Â͈ÙÂÚÈο È·ÙÚ›· ÙÔ˘ “∫·Ú·Ì·Ó‰¿ÓÂÈÔ˘” ¡ÔÛÔÎÔÌ›Ԣ ¶·›‰ˆÓ ¶·ÙÚÒÓ Ù· ¤ÙË 2000
Î·È 2003, ·Ó¿ÏÔÁ· Ì ÙÔ˘˜ Èı·ÓÔ‡˜ ·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘
¶·Ú¿ÌÂÙÚÔ˜¡¶ÔÛÔÛÙfi (%)
∆¿ÍË ÊÔ›ÙËÛ˘ (¢ËÌÔÙÈÎfi)
1Ë-2Ë 116341,2
3Ë-4Ë 80228,4
5Ë-6Ë 85530,3
™‡ÓÔÏÔ 2820100,0
ªÔÚʈÙÈÎfi Â›Â‰Ô ·Ù¤Ú·
∞/‚¿ıÌÈÔ - µ/‚¿ıÌÈÔ66881,0
°/‚¿ıÌÈÔ15719,0
™‡ÓÔÏÔ (825)100,0
ªÔÚʈÙÈÎfi Â›Â‰Ô ÌËÙ¤Ú·˜
∞/‚¿ıÌÈÔ - µ/‚¿ıÌÈÔ71386,7
°/‚¿ıÌÈÔ10913,3
™‡ÓÔÏÔ (822)100,0
√ÈÎÔÁÂÓÂȷ΋ ηٿÛÙ·ÛË ÁÔÓ¤ˆÓ
ŒÁÁ·ÌÔÈ68692,3 ¢È·˙¢Á̤ÓÔÈ506,7 ™‡ÓÔÏÔ (736)100,0
∞·Û¯fiÏËÛË ÌËÙ¤Ú·˜
∂ÚÁ·˙fiÌÂÓË60971,8 √ÈÎȷο23928,2 ™‡ÓÔÏÔ (848)100,0
⁄·ÚÍË ‹ ÌË ·‰ÂÏÊÒÓ
ªÔÓ·¯Ô·›‰È16018,9 1 42450,0 2 23327,5
331 3,7
™‡ÓÔÏÔ (848)100,0
πÛÙÔÚÈÎfi ·Ù˘¯‹Ì·ÙÔ˜ ¯Ú‹˙ÔÓÙÔ˜ È·ÙÚÈ΋˜ ÊÚÔÓÙ›‰·˜
ªÂ ÈÛÙÔÚÈÎfi 63222,4
ÚÔÁÂÓ¤ÛÙÂÚÔ˘ ·Ù˘¯‹Ì·ÙÔ˜
Èڛ˜ ÈÛÙÔÚÈÎfi 218877,6
ÚÔÁÂÓ¤ÛÙÂÚÔ˘ ·Ù˘¯‹Ì·ÙÔ˜
™‡ÓÔÏÔ 2820100,0
¶·ÚfiÓÙ˜ ÛÙÔ ·Ù‡¯ËÌ·
∫·Ó¤Ó·˜712,5
°ÔÓ›˜140349,8 ™˘ÁÁÂÓ›˜49517,6
√ÈÎȷ΋ ‚ÔËıfi˜, ʇϷη˜250,9 ÕÏÏÔÈ82629,3
™‡ÓÔÏÔ 2820100,0
ÃÒÚÔ˜ ·Ù˘¯‹Ì·ÙÔ˜ ™›ÙÈ101836,1
™¯ÔÏÂ›Ô (̤۷ ‹ Á‡Úˆ)40214,3
¶·È‰È΋ ¯·Ú¿38013,5 ¢ÚfiÌÔ˜30810,9 ⁄·ÈıÚÔ˜1455,1
ÕÏÏÔ˜ ¯ÒÚÔ˜56720,1
™‡ÓÔÏÔ 2820100,0
√Ì¿‰·
¶·Ú¿ÌÂÙÚÔ˜1Ë 2Ë 3Ë 4Ë
∏ÏÈΛ·
6-9620 (42,7%)540 (37,2%)136 (9,4%)156 (10,7%)
10-12416 (32,1%)545 (42,1%)143 (11,0%)192 (14,8%)
º‡ÏÔ
∞ÁfiÚÈ·644 (37,7%)689 (40,3%)169 (9,9%)207 (12,1%)
∫ÔÚ›ÙÛÈ·392 (37,7%)396 (38,1%)110 (10,6%)141 (13,6%)
∆fiÔ˜ ·Ù˘¯‹Ì·ÙÔ˜
™›ÙÈ974 (98,5%)15 (1,5%)0 (0,0%)0 (0,0%)
™¯ÔÏÂ›Ô (̤۷ ‹ Á‡Úˆ)0 (0,0%)59 (14,8%)2 (0,5%)338 (84,7%)
¶·È‰È΋ ¯·Ú¿1 (0,3%)379 (99,7%)0 (0,0%)0 (0,0%)
¢ÚfiÌÔ˜0 (0,0%)9 (3,0%)277 (93,6%)10 (3,4%)
⁄·ÈıÚÔ˜1 (0,8%)124 (99,2%)0 (0,0%)0 (0,0%)
ÕÏÏÔ˜ ¯ÒÚÔ˜60 (10,7%)499 (89,3%)0 (0,0%)0 (0,0%)
∆‡Ô˜ ·Ù˘¯‹Ì·ÙÔ˜
¶ÙÒÛË769 (41,7%)726 (39,4%)0 (0,0%)347 (18,8%)
ªÂÙ·ÊÔÚÈÎfi ̤ÛÔ17 (4,0%)136 (31,8%)275 (64,3%)0 (0,0%)
∞ȯÌËÚ¿ Î·È ‰ÈÂÈÛ‰˘ÙÈο ·ÓÙÈΛÌÂÓ·124 (47,3%)138 (52,7%)0 (0,0%)0 (0,0%)
∂ÎÚËÎÙÈÎfi ˘ÏÈÎfi4 (66,7%)2 (33,3%)0 (0,0%)0 (0,0%)
ŒÁη˘Ì·70 (93,3%)2 (2,7%)2 (2,7%)1 (1,3%)
∏ÏÂÎÙÚÈÎfi Ú‡̷8 (100,0%)0 (0,0%)0 (0,0%)0 (0,0%)
¢ËÏËÙËÚ›·ÛË (·fi Ê¿Ú̷η)7 (100,0%)0 (0,0%)0 (0,0%)0 (0,0%)
¢‹ÁÌ· ˙ÒÔ˘36 (30,3%)81 (68,1%)2 (1,7%)0 (0,0%)
∂Ô¯‹ ·Ù˘¯‹Ì·ÙÔ˜
ÃÂÈÌÒÓ·˜238 (36,1%)244 (37,9%)65 (9,9%)112 (17,0%)
ÕÓÔÈÍË318 (38,3%)283 (34,1%)91 (11,0%)138 (16,6%)
∫·ÏÔη›ÚÈ241 (38,1%)303 (47,9%)89 (14,1%)0 (0,0%)
ºıÈÓfiˆÚÔ239 (38,2%)255 (40,7%)34 (5,4%)98 (15,7%)
¶·ÚfiÓÙ˜
°ÔÓ›˜/Û˘ÁÁÂÓ›˜1035 (53,8%)644 (33,5%)244 (12,7%)0 (0,0%)
ÕÏÏÔÈ 1 (0,1%)441 (53,5%)35 (4,2%)348 (42,2%)
Û˘ÌÌ·ıËÙ¤˜ ‹ Ê›ÏÔ˘˜ Î·È fi¯È ¿ÙÔÌ· ÙÔ˘ Û˘ÁÁÂ-
ÓÈÎÔ‡ ÂÚÈ‚¿ÏÏÔÓÙÔ˜. ™˘ÁÎÚ›ÓÔÓÙ·˜ Ù· ·Ù˘¯‹Ì·Ù· ·Ó¿ÌÂÛ· ÛÙ·
‰‡Ô ¤ÙË, ·Ú·ÙËÚÂ›Ù·È ·‡ÍËÛË ÙÔ˘ ·ÚÈıÌÔ‡
ÙÔ˘˜ ÙÔ ¤ÙÔ˜ 2003 Û ÔÛÔÛÙfi 18% Û ۯ¤ÛË ÌÂ
ÙÔ 2000. ∏ ·‡ÍËÛË ·˘Ù‹ Ê·›ÓÂÙ·È fiÙÈ Â›Ó·È Û˘-
Ó¿ÚÙËÛË Ù˘ ·‡ÍËÛ˘ ÙˆÓ ·Ù˘¯ËÌ¿ÙˆÓ Ô˘
Û˘Ì‚·›ÓÔ˘Ó Î˘Ú›ˆ˜ ÛÙÔ Û›ÙÈ (+28,8%) Î·È ÛÙÔÓ ‰ÚfiÌÔ (+46,4%). ¶·Ú·ÙËÚÂ›Ù·È Ù·˘Ùfi-
¯ÚÔÓ· ÛËÌ·ÓÙÈ΋ Ì›ˆÛË ÙˆÓ ·Ù˘¯ËÌ¿ÙˆÓ Ô˘ Û˘Ì‚·›ÓÔ˘Ó Ì¤Û· Î·È Á‡Úˆ ·fi ÙÔ Û¯ÔÏÂ›Ô (-25,3%), ηıÒ˜ Î·È ÛÙ·ÛÈÌfiÙËÙ· ÛÙË Û˘¯ÓfiÙËÙ· ÙˆÓ ·Ù˘¯ËÌ¿ÙˆÓ
‰ÈΤ˜ ¯·Ú¤˜ ‹ ÛÙËÓ ‡·ÈıÚÔ. ŸÛÔÓ ·ÊÔÚ¿ ÛÙËÓ ÂÓÙfiÈÛË ÙÔ˘ ÙÚ·‡Ì·ÙÔ˜, ·Ú·ÙËÚÂ›Ù·È ·ÍÈÔÛËÌ›ˆÙË ·‡ÍËÛË ÙÔ˘ ÙÚ·˘-
ÙˆÓ ¿ÎÚˆÓ ÙÔ 2003 (63,4%) Û ۯ¤ÛË Ì ٷ ·ÓÙ›ÛÙÔȯ· ÙÚ·‡Ì·Ù· ÙÔ˘ 2000 (36,9%),
Û˘ÁÁÂÓÒÓ (fi¯È fï˜ ÁÔÓ¤ˆÓ) ·fi 4,3% Û 28,8% (x2: 327,9, p=0,000). ™ËÌÂÈÒÓÂÙ·È, ›Û˘, Ë ·Ô˘Û›· ·Ù˘¯ËÌ¿ÙˆÓ ˘fi ÙËÓ Â›‚ÏÂ„Ë Ê‡Ï·Î· (baby sitter) ηٿ ÙÔ ¤ÙÔ˜ 2003, Û ·ÓÙ›ıÂÛË Ì ٷ 25 ·Ù˘¯‹Ì·Ù· (1,9% ÙÔ˘ ÂÙ‹ÛÈÔ˘ Û˘ÓfiÏÔ˘) ÙÔ˘ 2000. √È Û˘ÁÎÚ›ÛÂȘ ÙˆÓ ‰È·ÊfiÚˆÓ ÌÂÙ·‚ÏËÙÒÓ ·Ó¿ÌÂÛ· ÛÙ· ‰‡Ô ʇϷ ‹Ù·Ó ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈΤ˜ (‰ÔÎÈÌ·Û›· x2) ˆ˜ ÚÔ˜ ÙÔ Â›‰Ô˜ ÙÔ˘ ·Ù˘¯‹Ì·ÙÔ˜ (x2: 20,18, p=0,01) Î·È ÙË ı¤ÛË ÙÔ˘ ÙÚ·‡Ì·ÙÔ˜ (x2: 34,58, p=0,00).
∆· ÎÔÚ›ÙÛÈ· ÚÔÛ‹Ïı·Ó ÛÙ· Â͈ÙÂÚÈο È·ÙÚ›· ÙÔ˘ ȉڇ̷ÙÔ˜ Ì ÌÂÁ·Ï‡ÙÂÚË Û˘¯ÓfiÙËÙ· ·fi fi,ÙÈ Ù· ·ÁfiÚÈ·, ÂÍ·ÈÙ›·˜ ·Ù˘¯‹Ì·ÙÔ˜ Ì ÂÌÏÔ΋ ÌÂÙ·ÊÔÚÈÎÔ‡ ̤ÛÔ˘ ηıÒ˜ Î·È ÂÁη˘Ì¿ÙˆÓ, ËÏÂÎÙÚÔÏËÍ›·˜ ‹ ‰ËÏËÙËÚ›·Û˘ ·fi ¯ËÌÈΤ˜ Ô˘Û›Â˜, ÂÓÒ Ù· ÙÚ·‡Ì·Ù¿ ÙÔ˘˜ ÂÓÙÔ›˙ÔÓÙ·Ó Î˘Ú›ˆ˜ ÛÙ· ¿ÎÚ·. ∞ÓÙ›ıÂÙ·, Ù· ·ÁfiÚÈ· ÂÌÊ¿ÓÈ˙·Ó ÌÂÁ·Ï‡ÙÂÚË
ÛÒÓ ÙÒÛˆÓ. ∂›Û˘, ·Ú·ÙËÚÂ›Ù·È ·‡ÍËÛË ÙˆÓ ·È‰ÈÎÒÓ ·Ù˘¯ËÌ¿ÙˆÓ
ÙËÓ Â›‚Ï„Ë
·ÚÈıÌfi ÙˆÓ ÂÂÈÛÔ‰›ˆÓ ÛÙȘ ÙÒÛÂȘ ‰ÂÓ ·Ú·ÙËÚ‹ıËΠ·Ó¿ÌÂÛ· ÛÙ· ‰‡Ô ʇϷ. √ ¤ÏÂÁ¯Ô˜ ÙˆÓ ÈÓ¿ÎˆÓ Û˘Ó¿ÊÂÈ·˜ ¤‰ÂÈÍ ›Û˘ ÛËÌ·-
ÓÙÈ΋ ‰È·ÊÔÚ¿ ˆ˜ ÚÔ˜ ÙËÓ Âԯȷ΋ ηٷÓÔ-
Ì‹ ÙˆÓ ·Ù˘¯ËÌ¿ÙˆÓ ·Ó¿ÌÂÛ· Û ·ÁfiÚÈ· Î·È ÎÔ-
Ú›ÙÛÈ· (x2: 9,12, p=0,02), Ì ٷ ÚÒÙ· Ó· ÙÚ·˘Ì·Ù›˙ÔÓÙ·È Û˘¯ÓfiÙÂÚ· ηٿ ÙÔ˘˜ ηÏÔηÈÚÈÓÔ‡˜ Ì‹Ó˜ Û ۯ¤ÛË Ì ٷ ‰Â‡ÙÂÚ·.
™˘˙‹ÙËÛË
∏ ·Ó¿Ï˘ÛË ÙˆÓ ·ÔÙÂÏÂÛÌ¿ÙˆÓ Ù˘ ·ÚÔ‡-
Û·˜ ÌÂϤÙ˘ ηٷ‰ÂÈÎÓ‡ÂÈ ·‡ÍËÛË ÙˆÓ ·È‰È-
ÎÒÓ ·Ù˘¯ËÌ¿ÙˆÓ ÙËÓ ÙÚÈÂÙ›· 2000-2003, fiˆ˜
Û˘Ó¿ÁÂÙ·È ·fi ÙËÓ ÚÔۤϢÛË ÛÙ· Â͈ÙÂÚÈο
È·ÙÚ›· ÙÔ˘ ¡ÔÛÔÎÔÌ›Ԣ ¶·›‰ˆÓ ¶·ÙÚÒÓ. ∏
·‡ÍËÛË ·˘Ù‹ ÔÊ›ÏÂÙ·È Î˘Ú›ˆ˜ ÛÙËÓ ·‡ÍËÛË
ÙˆÓ ·Ù˘¯ËÌ¿ÙˆÓ Ô˘ Û˘Ì‚·›ÓÔ˘Ó Ì¤Û· ÛÙÔ
Û›ÙÈ, ηıÒ˜ Î·È Û ·‡ÍËÛË ÙˆÓ ÙÚÔ¯·›ˆÓ.
∞ÓÙ›ÛÙÔȯ·, ‰È·ÈÛÙÒıËΠ̛ˆÛË ÙˆÓ ·Ù˘¯Ë-
Ì¿ÙˆÓ Ô˘ Û˘Ì‚·›ÓÔ˘Ó Ì¤Û· ‹ Á‡Úˆ ·fi Ù·
Û¯ÔÏ›· (·fi 17,8% Û 11,3%), ηıÒ˜ Î·È ÌÈ-
ÎÚ‹ ˘Ô¯ÒÚËÛË ·˘ÙÒÓ Ô˘ Û˘Ì‚·›ÓÔ˘Ó ÛÙȘ
·È‰ÈΤ˜ ¯·Ú¤˜ (·fi 14,8% Û 12,4%). ∂›Ó·È Èı·Ófi Ë ·Ú·ÙËÚÔ‡ÌÂÓË Ì›ˆÛË, Â¿Ó ‰ÂÓ ÔÊ›-
ÏÂÙ·È Û ÂӉ¯fiÌÂÓË ·Ó¿ÚÎÂÈ· ‰Â‰Ô̤ӈÓ
ÙˆÓ ·Ú¯Â›ˆÓ ÙÔ˘ ¡ÔÛÔÎÔÌ›Ԣ ¶·›‰ˆÓ, Ó· ›-
Ó·È ·ÔÙ¤ÏÂÛÌ· ÙˆÓ ÚÔÏËÙÈÎÒÓ Ì¤ÙÚˆÓ Ô˘
¤¯Ô˘Ó ÏËÊı› Ù· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ· ÛÙË ¯ÒÚ·
Ì·˜, fiˆ˜ ÁÈ· ·Ú¿‰ÂÈÁÌ· Ë Â¤ÎÙ·ÛË ÙÔ˘ ̤ÙÚÔ˘ ÙˆÓ Û¯ÔÏÈÎÒÓ ÙÚÔ¯ÔÓfiÌˆÓ ‹ Ê˘Ï¿ÎˆÓ, ηıÒ˜ Î·È Ë Â˘·ÈÛıËÙÔÔ›ËÛË ÙˆÓ ÂÎ·È‰Â˘ÙÈÎÒÓ Î·È ÙˆÓ ‰ËÌÔÙÈÎÒÓ ·Ú¯ÒÓ ˆ˜ ÚÔ˜ ÙËÓ ·ÛÊ¿ÏÂÈ· ÛÙ· Û¯ÔÏ›·. ŸÛÔÓ ·ÊÔÚ¿ ÛÙ· ·Ù˘¯‹Ì·Ù· Ô˘ Ï·Ì‚¿ÓÔ˘Ó ¯ÒÚ· ÛÙȘ ·È‰ÈΤ˜ ¯·-
Ú¤˜, ·Ú¿ ÙË ÌÈÎÚ‹ ÙÔ˘˜ Ì›ˆÛË, ·Ú·Ì¤ÓÔ˘Ó
Û˘¯Ó¿ Î·È Ì¿ÏÈÛÙ· ÙÚÈÏ¿ÛÈ· ·fi ÙÔ 4,4% Ô˘
¤¯ÂÈ ÂÎÙÈÌËı› ÁÈ· ÙÔ Û‡ÓÔÏÔ Ù˘ ¯ÒÚ·˜ (10).
∆·˘Ùfi¯ÚÔÓ·, Û ȉȷ›ÙÂÚ· ˘„ËÏ¿ ›‰·
ηٷÁÚ¿ÊÔÓÙ·È Ù· ÔÛÔÛÙ¿ ÙˆÓ ·Ù˘¯ËÌ¿ÙˆÓ
ÛÙÔ Û›ÙÈ ‹ fiÔ˘ ÂÌϤÎÔÓÙ·È ÌÂÙ·ÊÔÚÈο ̤۷, Ù· ÚÒÙ· ÂÍ·ÈÙ›·˜ Ù˘ Û˘¯ÓfiÙËÙ¿˜ ÙÔ˘˜ Î·È Ù· ‰Â‡ÙÂÚ· ÂÍ·ÈÙ›·˜ Ù˘ ‚·Ú‡ÙËÙ·˜ Î·È ÙˆÓ Û˘ÓÂÂÈÒÓ ÙÔ˘˜ (11). ™ÙËÓ ÚÒÙË ÂÚ›ÙˆÛË, ÂÎÙfi˜ ·fi ÙÔ˘˜ ·ÓÙÈÎÂÈÌÂÓÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ Ô˘ ÂÌϤÎÔÓÙ·È (12), ÛÙfi¯Ô˜ ÙˆÓ Ì¤ÙÚˆÓ
ÚfiÏ˄˘ ı· Ú¤ÂÈ Ó· Â›Ó·È Î˘Ú›ˆ˜ Ë ÂÓË̤ڈÛË ÙˆÓ ÁÔÓ¤ˆÓ. ™ÙË ‰Â‡ÙÂÚË, fiÔ˘ ÂÌϤ-
·˜) (13) ›ÙÂ Â›Ó·È Ô‰ËÁÔ› Ô¯ËÌ¿ÙˆÓ (.¯. ÎÚ¿ÓË, ÚÔÛٷ٢ÙÈο ¿ÎÚˆÓ), ÂÓÒ ˘¿Ú¯Ô˘Ó ηÈ
ÂÚÈıÒÚÈ· ‚ÂÏÙ›ˆÛ˘ ÙˆÓ Û˘ÓıËÎÒÓ Û Â˙Ô‰ÚfiÌÈ· Î·È ‰ÚfiÌÔ˘˜ (14). ™ÙËÓ ·ÚÔ‡Û· ÌÂϤÙË ·Ú·ÙËÚ‹ıËΠÌÈ·
¿ÓÈÛË Î·Ù·ÓÔÌ‹ ÙˆÓ ·Ù˘¯ËÌ¿ÙˆÓ ·Ó¿ÌÂÛ· ÛÙ·
‰‡Ô ʇϷ. ªÂ ‚¿ÛË Ù· ‰Â‰Ô̤ӷ Ô˘ ÂÍÂÙ¿ÛÙËηÓ, Ù· ·ÁfiÚÈ· Ê·›ÓÂÙ·È Ó· ÙÚ·˘Ì·Ù›˙ÔÓÙ·È
Û˘¯ÓfiÙÂÚ· ·fi Ù· ÎÔÚ›ÙÛÈ· Û ·Ó·ÏÔÁ›· 1,6/1, Ë ÔÔ›· Â›Ó·È Û¯Â‰fiÓ Ù·˘ÙfiÛËÌË Ì ÂΛÓË Ô˘
·Ó·Ê¤ÚÂÙ·È ·fi ¿ÏÏÔ˘˜ ÂÚ¢ÓËÙ¤˜ (15) ‹ ·fi ›ÛË̘ ·Ó·ÊÔÚ¤˜ (16). ∏ ‰È·ÊÔÚ¿ ·˘Ù‹ ÛÂ
ÌÂÁ¿ÏÔ ÔÛÔÛÙfi ÔÊ›ÏÂÙ·È ÛÙËÓ È‰ÈÔÛ˘ÁÎÚ·Û›·, ·ÏÏ¿ Î·È ÛÙÔÓ ÙÚfiÔ Î·È ÙÔ Â›‰Ô˜ ÂΉ‹ÏˆÛ˘ Ù˘ Û˘ÌÂÚÈÊÔÚ¿˜ ÙˆÓ ‰‡Ô Ê‡ÏˆÓ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ·È¯ÓȉÈÔ‡ ‹ ÙˆÓ ˘fiÏÔÈˆÓ ‰Ú·-
Ó˜ ¤Ú¢Ó˜ (7) Ë Û˘¯ÓfiÙËÙ· ÙÚ·˘Ì·ÙÈÛÌÔ‡ ÛÙÔ
ÛÙ· ·ÁfiÚÈ· Û ۯ¤ÛË
Ì ٷ ÎÔÚ›ÙÛÈ· (22,1% Û ۯ¤ÛË Ì 16,6%) Î·È Ù· ÙÚ·‡Ì·Ù· ·˘Ù¿ ·ÎÔÏÔ˘ıÔ‡Ó Âԯȷ΋ ηٷ-
ÓÔÌ‹ Ì ·È¯Ì‹ (35,1% ÛÙËÓ ·ÚÔ‡Û· ÌÂϤÙË)
ηٿ ÙÔ˘˜ ·ÚÈÓÔ‡˜ Ì‹Ó˜ (17).
ŸÛÔÓ ·ÊÔÚ¿ ÛÙ· ÎÔÈÓˆÓÈÎÔ-‰ËÌÔÁÚ·ÊÈο
¯·Ú·ÎÙËÚÈÛÙÈο, ·Ú·ÙËÚ‹ıËΠfiÙÈ Ù· ÂÚÈÛÛfiÙÂÚ· ·È‰È¿ ¤¯Ô˘Ó ÁÔÓ›˜ ¯·ÌËÏÔ‡ ‹ ̤ÙÚÈÔ˘
ÌÔÚʈÙÈÎÔ‡ ÂȤ‰Ô˘, ÌËÙ¤Ú˜ ÂÚÁ·˙fiÌÂÓ˜ Î·È ÙÔ˘Ï¿¯ÈÛÙÔÓ ¤Ó·Ó ·‰ÂÏÊfi ‹ ·‰ÂÏÊ‹ ÛÙËÓ ÔÈÎÔÁ¤ÓÂÈ·. ∞ÓÙ›ÛÙÔȯ· ˘„ËÏ¿ ÔÛÔÛÙ¿ ¤¯Ô˘Ó ·Ú·ÙËÚËı› Î·È Û ¿ÏϘ ¤Ú¢Ó˜ ηٿ ÙÔ ·ÚÂÏıfiÓ (18-20). ¢ÂÓ Â›Ó·È fï˜ ÁÓˆÛÙfi ·Ó Ù· ¯·Ú·ÎÙËÚÈÛÙÈο ·˘Ù¿ ·ÔÙÂÏÔ‡Ó ÚˆÙÔÁÂÓ›˜ ·Èٛ˜, ÔfiÙÂ Ô Û¯ÔÏÈ·ÛÌfi˜ Á›ÓÂÙ·È ¿ÓÙ· Ì ÂÈʇϷÍË.
∞fi Ù· ›‰Ë ÙˆÓ ·Ù˘¯ËÌ¿ÙˆÓ Ô˘ ÌÂÏÂÙ‹ıËηÓ, ÔÈ ÙÒÛÂȘ ·ÔÙÂÏÔ‡Ó ÙËÓ ÚÒÙË ·ÈÙ›· ÙÚ·˘Ì·ÙÈÛÌÔ‡ (66,7%) Î·È ·ÔÙÂÏÔ‡Ó ÙË ‰Â‡ÙÂÚË ·ÈÙ›· ı·Ó¿ÙÔ˘ ·fi ·Ù˘¯‹Ì·Ù· ÛÙ· ·È‰È¿ (11). ∏ ηٷÓÔÌ‹ ·˘ÙÒÓ ÙˆÓ ·Ù˘¯ËÌ¿ÙˆÓ Î·ıÒ˜ Î·È ÔÈ ·Èٛ˜ ÙÔ˘˜ ¤¯Ô˘Ó ··Û¯ÔÏ‹ÛÂÈ ÙÔ˘˜ ÂÚ¢ÓËÙ¤˜, fi¯È ÌfiÓÔ ‰ÈfiÙÈ ·ÔÙÂÏÔ‡Ó ÙÔÓ Î‡ÚÈÔ ÏfiÁÔ ÓÔÛËÏ›·˜ ÙˆÓ ·È‰ÈÒÓ, ·ÏÏ¿ Î·È ÁÈ·Ù› Û˘¯Ó¿ Â›Ó·È ÙÔ ·ÔÙ¤ÏÂÛÌ· Û˘ÁÎ·Ï˘Ì̤Ó˘
¶·È‰È·ÙÚÈ΋ 2006;69:278-285
̤ÙÚˆÓ, ‚ÂÏÙ›ˆÛË ÙˆÓ Ì¤ÙÚˆÓ ·ÛÊ·Ï›·˜ ÙˆÓ
ÂÛˆÙÂÚÈÎÒÓ Î·È Â͈ÙÂÚÈÎÒÓ ¯ÒÚˆÓ Î.¿.).
∆¤ÏÔ˜, Ú¤ÂÈ Ó· ÂÈÛËÌ·Óı› fiÙÈ ÔÈ ÂÚÈÔ-
ÚÈÛÌÔ› Ô˘ Ù›ıÂÓÙ·È Û ·Ó¿ÏÔÁ˜ ÂÚÈÁÚ·ÊÈΤ˜
ÌÂϤÙ˜
ÊÒÓ, fiˆ˜ Î·È ÛÙËÓ ·ÚÔ‡Û·, Â›Ó·È ‰Â‰Ô̤ÓÔÈ
Î·È Ú¤ÂÈ Ó· Ï·Ì‚¿ÓÔÓÙ·È ˘’ fi„ÈÓ ÚÈÓ ÁÂÓÈ΢ÙÔ‡Ó ÔÔÈ·‰‹ÔÙÂ Û˘ÌÂÚ¿ÛÌ·Ù·. ∏
·Ô˘Û›· Ì·ÚÙ‡ÚˆÓ ÂÚÈÔÚ›˙ÂÈ ÙË ÛËÌ·ÓÙÈÎfiÙËÙ· ÙÔ˘ ¤ÏÂÁ¯Ô˘ Î·È ÂÈÛ¿ÁÂÈ Û‡Á¯˘ÛË ÛÙËÓ ·Ó¿-
Ï˘ÛË. ∞ÎfiÌË, ¤Ó· ¿ÏÏÔ Úfi‚ÏËÌ· Ù˘ ·ÚÔ‡-
Û·˜ ¤Ú¢ӷ˜ Â›Ó·È Ë ÂÎÙ›ÌËÛË ÙÔ˘ ηٿÏÏËÏÔ˘
·ÚÔÓÔÌ·ÛÙ‹ ÁÈ· ÙÔÓ ˘ÔÏÔÁÈÛÌfi ÙˆÓ ‰ÂÈÎÙÒÓ
›وÛ˘, ·ÊÔ‡
¤¯Ô˘Ó ÂÈÛËÌ¿ÓÂÈ
›ӷÈ: ÙÔ Ê‡ÏÔ ÙÔ˘ ·È‰ÈÔ‡, Ë ËÏÈΛ· ÙÔ˘, ÙÔ ÌÔÚʈÙÈÎfi Â›Â‰Ô ÙˆÓ ÁÔÓ¤ˆÓ, Ë ··Û¯fiÏËÛË Ù˘ ÌËÙ¤Ú·˜ ‹ ·ÎfiÌË Î·È Ë ¯ÚÔÓÈ΋ ÛÙÈÁÌ‹ Î·È Ë ÂÔ¯‹ ÙÔ˘ ·Ù˘¯‹Ì·ÙÔ˜. ¶ÂÚÈÁÚ·ÊÈΤ˜ ÌÂϤÙ˜, fiˆ˜ Ë Û˘ÁÎÂÎÚÈ̤ÓË, ÌÔÚÔ‡Ó Ó· ˘Ô‰Â›ÍÔ˘Ó ·‰Ú¿ Ù¤ÙÔÈÔ˘ Ù‡Ô˘ ·Ú¿ÁÔÓÙ˜, ˘Ô‰ÂÈÎÓ‡ÔÓÙ·˜ Ù·˘Ùfi¯ÚÔÓ· Î·È ÙÔ˘˜ ÛÙfi¯Ô˘˜ Ô˘ ı· Ú¤ÂÈ Ó· ¤¯Ô˘Ó Ù· Ù˘¯fiÓ Ì¤ÙÚ· ÚfiÏ˄˘. øÛÙfiÛÔ, Ë ·ÔÛ·Ê‹ÓÈÛË ÙÔ˘ ÚfiÏÔ˘ ÙÔ˘˜, fiˆ˜ Î·È ÙÔ Â›Â‰Ô Û˘ÓÂÈÛÊÔÚ¿˜ ÙÔ˘ οı ·Ú¿ÁÔÓÙ· ÛÙÔ Û‡ÓıÂÙÔ ı¤Ì· Ô˘ ϤÁÂÙ·È ·È‰ÈÎfi ·Ù‡¯ËÌ·, ı· ÚÔ·„Ô˘Ó ÌfiÓÔ Ì¤Û· ·fi ÚÔÛÂÎÙÈο ۯ‰ȷṲ̂Ó˜ ÂȉËÌÈÔÏÔÁÈΤ˜ ¤Ú¢Ó˜, ÔÈ Ôԛ˜ Â›Ó·È ··Ú·›ÙËÙ˜.
∂˘¯·ÚÈÛٛ˜
ÁÈ·
ÏfiÁÔ ·˘Ùfi ‰ÂÓ ¤ÁÈÓ ٤ÙÔÈ· ÂÎÙ›ÌËÛË.
ÛÙÔȯ›· ‰Â›¯ÓÔ˘Ó fiÙÈ ÛÙËÓ ∂ÏÏ¿‰·, Ô ‰Â›ÎÙ˘ ı·Ó·ÙËÊfiÚˆÓ ·È‰ÈÎÒÓ ·Ù˘¯ËÌ¿ÙˆÓ ¤¯ÂÈ ‰È·ÌÔÚʈı› Û 7,8 ·È‰È¿ ÁÈ· οı 100.000 ηÙÔ›ÎÔ˘˜. ∞ÓÙ›ÛÙÔȯÔÈ ‰Â›ÎÙ˜ Â›Ó·È 0,27 ÁÈ· ÙȘ ÙÒÛÂȘ, 4,14 ÁÈ· Ù· ÙÚÔ¯·›· ‰˘ÛÙ˘¯‹Ì·Ù·, 0,14 ÁÈ· Ù· ÂÁη‡Ì·Ù· ηÈ
0,15 ÁÈ· ÙȘ ‰ËÏËÙËÚÈ¿ÛÂȘ, Ô˘ Â›Ó·È ·fi ÙÔ˘˜ ˘„ËÏfiÙÂÚÔ˘˜ ÛÙËÓ ∂˘Úˆ·˚΋ ŒÓˆÛË (21). √È ·ÚÈıÌÔ› ·˘ÙÔ› ı· Ú¤ÂÈ Ó· ·ÔÙÂϤÛÔ˘Ó ·ÓÙÈΛÌÂÓÔ ÚÔ‚ÏËÌ·ÙÈÛÌÔ‡, ·ÏÏ¿ Ù·˘Ùfi¯ÚÔÓ·
ηٷ‰ÂÈÎÓ‡Ô˘Ó fiÙÈ ˘¿Ú¯Ô˘Ó Î·È ÌÂÁ¿Ï· ÂÚÈıÒÚÈ· ‚ÂÏÙ›ˆÛ˘, Û˘ÁÎÚÈÓfiÌÂÓÔÈ Ì ·˘Ù¿ ¿Ï-
ÏˆÓ ¯ˆÚÒÓ. ∏ ʇÛË ÙÔ˘ ·È‰ÈÎÔ‡ ·Ù˘¯‹Ì·ÙÔ˜
‰‡ÛÎÔÏ· ÂÈÙÚ¤ÂÈ ÙËÓ ÂÍ·ÁˆÁ‹ ÂȉÈÎÒÓ Û˘-
ÌÂÚ·ÛÌ¿ÙˆÓ. ∞ÔÙÂÏ›, fï˜, ÁÂÓÈÎfiÙÂÚË Â-
Ô›ıËÛË (22,23) fiÙÈ Û ÌÂÁ¿ÏÔ ‚·ıÌfi Ù· ·È‰È-
ο ·Ù˘¯‹Ì·Ù· ÌÔÚÔ‡Ó Ó· ÚÔ‚ÏÂÊıÔ‡Ó, Û¯ÂÙ›˙ÔÓÙ·È Ì ·Ú¿ÁÔÓÙ˜ ÙÔ˘ ÂÚÈ‚¿ÏÏÔÓÙÔ˜
ÙˆÓ ·È‰ÈÒÓ ·Ó¿ÏÔÁÔ˘˜ Ì ·˘ÙÔ‡˜
µÈ‚ÏÈÔÁÚ·Ê›·
1.Vincenten J, Michalsen A. Priorities for child safety in the European Union: agenda for action. Inj Control Saf Promot 2002;9:1-8.
2.Centers for Disease Control and Prevention (CDC). Medical expenditures attributable to injuriesUnited States, 2000. MMWR Morb Mortal Wkly Rep 2004;53:1-4.
3.Cohen LR, Runyan CW, Downs SM, Bowling JM. Pediatric injury prevention counseling priorities. Pediatrics 1997;99:704-710.
4.Petridou E. Childhood injuries in the European Union: can epidemiology contribute to their control? Acta Paediatr 2000;89:1244-1249.
5.Dessypris N, Petridou E, Skalkidis Y, Moustaki M, Koutselinis A, Trichopoulos D. Countrywide estimation of the burden of injuries in Greece: a limited resources approach. J Cancer Epidemiol Prev 2002;7:123-129.
6.Rivara FP. Pediatric injury control in 1999: where do we go from here? Pediatrics 1999;103:883-888.
7.§·Ú›Ô˘
·È‰È¿: ÂȉËÌÈÔÏÔÁ›· Î·È ÚfiÏË„Ë. 13Ô ¶·ÓÂÏÏ‹ÓÈÔ ™˘Ó¤‰ÚÈÔ ∂ÏÏËÓÈ΋˜ ∂Ù·ÈÚ›·˜ ∫ÔÈÓˆÓÈ΋˜ ¶·È‰È·ÙÚÈ΋˜ Î·È ¶ÚÔ·ÁˆÁ‹˜ Ù˘ ÀÁ›·˜. ¶¿ÙÚ·: 1820 ª·˝Ô˘ 2001. 8.ªÔ˘ÛÙ¿ÎË ª, ¢ÂÛ‡Ú˘ ¡, ¶›ÙÛÔ˜ ¡, ¶ÂÙÚ›‰Ô˘ ∂. √È Î·ÎÒÛÂȘ ÙÔ˘ ÁfiÓ·ÙÔ˜ ÛÙ· ·È‰È¿ Û ‰Ú·ÛÙËÚÈfiÙËÙ˜ ÂχıÂÚÔ˘ ¯ÚfiÓÔ˘. 13Ô ¶·ÓÂÏÏ‹ÓÈÔ ™˘Ó¤‰ÚÈÔ ∂ÏÏËÓÈ΋˜ ∂Ù·ÈÚ›·˜ ∫ÔÈÓˆÓÈ΋˜ ¶·È‰È·ÙÚÈ΋˜ Î·È ¶ÚÔ·ÁˆÁ‹˜ Ù˘ ÀÁ›·˜. ¶¿ÙÚ·: 18-20 ª·˝Ô˘ 2001.
9.∫ÔÓÙ¤ µ, ¶ÂÙÚ›‰Ô˘ ∂. ¶·È‰Èο ·È¯Ó›‰È·, fiÛÔ
›ӷÈ; 13Ô ¶·ÓÂÏÏ‹ÓÈÔ ™˘Ó¤‰ÚÈÔ ∂ÏÏËÓÈ΋˜ ∂Ù·ÈÚ›·˜ ∫ÔÈÓˆÓÈ΋˜ ¶·È‰È·ÙÚÈ΋˜ Î·È ¶ÚÔ·ÁˆÁ‹˜ Ù˘ ÀÁ›·˜. ¶¿ÙÚ·: 18-20 ª·˝Ô˘ 2001.
10. Petridou E, Sibert J, Dedoukou X, Skalkidis I, Trichopoulos D. Injuries in public and private playgrounds: the relative contribution of structural, equipment and human factors. Acta Paediatr 2002;91:691-697.
11.WHO Regional Office for Europe. Valent F, d’Anna L, Tamburini G, Barbone F. Burden of disease attributable to selected environmental factors and injuries among Europe’s children and adolescents. Environmental Burden of Disease Series No. 8, 2004. http://www.who.int/entity/quantifying_ehimpacts/ publications/en/ebd8web.pdf
12.Belechri M, Petridou E, Trichopoulos D. Bunk versus conventional beds: a comparative assessment of fall injury risk. J. Epidemiol Community Health 2002;56:413-417.
13.Petridou E, Skalkidou A, Lescohier I, Trichopoulos D. Car restraints and seating position for prevention of motor vehicle injuries in Greece. Arch Dis Child 1998;78:335-339.
14.Moustaki M, Petridou E, Trichopoulos D. Person, time and place coordinates of pedestrian injuries: a study in Athens. Acta Paediatr 2001;90:558-562.
15.Chan CC, Cheng JC, Wong TW, Chow CB, Luis BP, Cheung WL et al. An international comparison of childhood injuries in Hong Kong. Inj Prev 2000; 6:20-23.
16. UNICEF. Innocenti Research Centre. Innocenti report Card No 2, Florence, Italy: Feb 2001.
17.Lyons RA, Delahunty AM, Kraus D, Heaven M, McCabe M, Allen H et al. Children’s fractures: a population based study. Inj Prev 1999;5:129-132.
18.American Academy of Pediatrics (AAP), Committee on Injury and Poison Prevention. Falls from heights: windows, roofs, and balconies. Pediatrics 2001;107:1188-1191.
19.Petridou E, Trichopoulos D, Mera E, Papadatos Y, Papazoglou K, Marantos A et al. Risk factors for childhood burn injuries: a case-control study from Greece. Burns 1998;24:123-128.
20. Jordan EA, Duggan AK, Hardy JB. Injuries in children of adolescent mothers: home safety education associated with decreased injury risk. Pediatrics 1993;91:481-487.
21.European Child Safety Alliance of the European Consumer Safety Association (ECOSA). Priorities for child safety in the European Union: Agenda for action. Amsterdam, 2004 June: Website: www.childsafetyeurope.org
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23.WHO Regional Office for Europe’s Health Evidence Network (HEN). How can injuries in children and older people be prevented? 2004 October: Webpage: http://www.who.dk/HEN/Syntheses/injuries/20041 107_1
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A’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋
¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ
“∞Á›· ™ÔÊ›·”
AÏÏËÏÔÁÚ·Ê›·:
∞ÓÙˆÓ›· ¢·ÛÙ·Ì¿ÓË
∫·ÏÏÈÁ¿ 5
∆.∫. 152 37, ºÈÏÔı¤Ë
∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 19-05-2006
∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 24-05-2006
∏ Û˘¯ÓfiÙËÙ· ÙˆÓ ÊÔÚ¤ˆÓ Ù˘ Û˘ÁÁÂÓÔ‡˜
ÛÙËÓ ∂ÏÏ¿‰· Ì ÙËÓ ÂÊ·ÚÌÔÁ‹ ÌÔÚÈ·ÎÒÓ Ù¯ÓÈÎÒÓ
∞. ¢·ÛÙ·Ì¿ÓË, ª. ¢Ú·ÎÔÔ‡ÏÔ˘, ∞. ™ÂÚÙ‰¿ÎË, °.¶. ÃÚÔ‡ÛÔ˜, ∞. ¢¿ÎÔ˘ - µÔ˘ÙÂÙ¿ÎË
¶ÂÚ›ÏË„Ë
∂ÈÛ·ÁˆÁ‹: ∏ ‰È·Ù·Ú·¯‹ Ù˘ ÛÙÂÚÂÔÂȉÔÁ¤ÓÂÛ˘ Ô˘ ÚÔηÏÂ›Ù·È ·fi ÌÂÙ·ÏÏ¿ÍÂȘ ÙÔ˘ ÁÔÓȉ›Ô˘ Ù˘ 21-˘‰ÚÔÍ˘Ï¿Û˘ (CYP21), ·ÔÙÂÏ› ÙÔ Û˘¯ÓfiÙÂÚÔ ·›ÙÈÔ Ù˘ Û˘ÁÁÂÓÔ‡˜ ˘ÂÚÏ·Û›·˜ ÂÈÓÂÊÚȉ›ˆÓ (™À∂). ¢Â‰Ô̤ӷ ÁÈ· ÙË Û˘¯ÓfiÙËÙ· ÙÔ˘ ÁÔÓȉ›Ô˘, ‚·ÛÈṲ̂ӷ Û ÌÔÚȷΤ˜ Ù¯ÓÈΤ˜, Â›Ó·È ÂÏ¿¯ÈÛÙ·. ™ÎÔfi˜ Ù˘ ÌÂϤÙ˘ ‹Ù·Ó Ë ·Ó·˙‹ÙËÛË ÙÔ˘ ›‰Ô˘˜ Î·È Ù˘ Û˘¯ÓfiÙËÙ·˜ ÙˆÓ ‰È·Ù·Ú·¯ÒÓ ÙÔ˘ ÁÔÓȉ›Ô˘ CYP21 ÛÙÔÓ ÂÏÏËÓÈÎfi ÏËı˘ÛÌfi.
ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ: ¢Â›ÁÌ·Ù· ·›Ì·ÙÔ˜ ÓÂÔÁÓÒÓ ÂÏ‹ÊıËÛ·Ó ·fi Ì·ÈÂ˘Ù‹ÚÈ· fiÏ˘ Ù˘ ¯ÒÚ·˜. ∏ ·Ó¿Ï˘ÛË ÙÔ˘ DNA ¤ÁÈÓ Û 381 ‰Â›ÁÌ·Ù· (762 ·ÏÏËÏfiÌÔÚÊ·). ∏ ̤ıÔ‰Ô˜ ÙÔ˘ ÂÎÏÂÎÙÈÎÔ‡ ÔÏÏ·Ï·ÛÈ·ÛÌÔ‡ ÙÌ‹Ì·ÙÔ˜ Û˘ÁÎÂÎÚÈ̤ÓÔ˘ ·ÏÏËÏfiÌÔÚÊÔ˘ (allele specific PCR) ÂÊ·ÚÌfiÛÙËΠÁÈ· ÙËÓ ·Ó›¯Ó¢ÛË 12 ÌÔÚÈ·ÎÒÓ ‰È·Ù·Ú·¯ÒÓ ÙÔ˘ ÁÔÓȉ›Ô˘ CYP21: ÔÌfi˙˘ÁÔ˜ ·¿ÏÂÈ„Ë/ÌÂÙ·ÙÚÔ‹ ÙÔ˘ ÁÔÓȉ›Ô˘ CYP21, P30L Î·È W22X (ÂÍÒÓÈÔ 1), I2splice (ÈÓÙÚfiÓÈÔ 2), 8bpdelE3 (ÂÍÒÓÈÔ 3), I172N (ÂÍÒÓÈÔ 4), ClusterE6 (ÂÍÒÓÈÔ 6), V281L Î·È F306+T (ÂÍÒÓÈÔ 7), Q318X Î·È R356W (ÂÍÒÓÈÔ 8), P453S (ÂÍÒÓÈÔ 10). ∂ÓÓ¤· ·fi ·˘Ù¤˜ ÙȘ ÌÂÙ·ÏÏ¿ÍÂȘ ·ÓÙÈÛÙÔÈ¯Ô‡Ó ÛÙÔ 94% ÙˆÓ ‰È·Ù·Ú·¯ÒÓ ÙÔ˘ ÁÔÓȉ›Ô˘ CYP21 Û ÏËı˘ÛÌfi ∂ÏÏ‹ÓˆÓ ·Û¯fiÓÙˆÓ, Û‡Ìʈӷ Ì ·Ï·ÈfiÙÂÚË ÌÂϤÙË. ∞ÔÙÂϤÛÌ·Ù·: ∞ÓȯÓ‡ıËÎ·Ó ÌÂÙ·ÏÏ¿ÍÂȘ Û 39 ·ÏÏËÏfiÌÔÚÊ· (5,12%): ‰ÂηÙÚ›· Ù˘ ÎÏ·ÛÈ΋˜ ÌÔÚÊ‹˜ Ù˘ ™À∂ (Q318X, 1,3%; I172N, 0,14%; I2splice, 0,14%, W22X, 0,14%) Î·È 26 Ù˘ ÌË ÎÏ·ÛÈ΋˜ ÌÔÚÊ‹˜ (V281L, 3,28%; P453S, 0,98%; P30L, 0,28%). ¶ÔÛÔÛÙfi 10,2% ÙÔ˘ ‰Â›ÁÌ·ÙÔ˜ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ ‹Ù·Ó ÊÔÚ›˜ ÙˆÓ ‰È·Ù·Ú·¯ÒÓ Ù˘ 21-˘‰ÚÔÍ˘Ï¿Û˘. ∏ Û˘¯ÓfiÙËÙ· ÙˆÓ ÂÙÂÚÔ˙˘ÁˆÙÒÓ Ù˘ ÎÏ·ÛÈ΋˜ ÌÔÚÊ‹˜ Ù˘ ÓfiÛÔ˘ Â›Ó·È 3,4% Î·È Ù˘ ÌË ÎÏ·ÛÈ΋˜ ÌÔÚÊ‹˜ 6,8%. ÀÔÏÔÁ›ÛÙËÎÂ Ë Û˘¯ÓfiÙËÙ· Ù˘ ÎÏ·ÛÈ΋˜ ÌÔÚÊ‹˜ ·Ó¿ÚÎÂÈ·˜ Ù˘ 21-˘‰ÚÔÍ˘Ï¿Û˘ Û 1:3.436 Î·È Ë Û˘¯ÓfiÙËÙ· Ù˘ ÌË ÎÏ·ÛÈ΋˜ ÌÔÚÊ‹˜ Û 1:859. ∏ ˘ÔÏÔÁÈÛı›۷ Û˘¯ÓfiÙËÙ· ÙÔ˘ ÁÔÓȉ›Ô˘ Â›Ó·È 0,051. ™˘ÌÂÚ¿ÛÌ·Ù·: ∏ Û˘¯ÓfiÙËÙ· Ù˘ ÓfiÛÔ˘ Î·È ÙˆÓ ÂÙÂÚÔ˙˘ÁˆÙÒÓ Â›Ó·È Ôχ ÌÂÁ·Ï‡ÙÂÚË ÛÙÔÓ ÂÏÏËÓÈÎfi ÏËı˘ÛÌfi ¤Ó·ÓÙÈ ÙˆÓ ∫ÈÓ¤˙ˆÓ Î·È ÙˆÓ ¡ÂÔ˙ËÏ·Ó‰ÒÓ, ÔÈ ÔÔ›ÔÈ ¤¯Ô˘Ó Ú·ÁÌ·ÙÔÔÈ‹ÛÂÈ ÌÂϤÙ˜ Ì ·Ó¿ÏÔÁ˜ ÌÔÚȷΤ˜ Ù¯ÓÈΤ˜. ∏ ·ÚÔ‡Û· ¤Ú¢ӷ ÂȂ‚·›ˆÛ ÙÔ ÁÂÁÔÓfi˜ fiÙÈ ÚÔËÁÔ‡ÌÂÓ· ‰Â‰Ô̤ӷ ÂϤÁ¯ˆÓ ‰È·ÏÔÁ‹˜ ÓÂÔÁÓÒÓ (screening), ‚·ÛÈṲ̂ӷ Û ÔÚÌÔÓÈΤ˜ ÂÍÂÙ¿ÛÂȘ, ˘ÔÂÎÙÈÌÔ‡Ó ÙË Û˘¯ÓfiÙËÙ· Ù˘ ™À∂. ∂ÈϤÔÓ, ÚÔ·ÙÂÈ ÙÔ Û˘Ì¤Ú·ÛÌ· fiÙÈ Ë Û˘¯ÓfiÙËÙ· ÙÔ˘ ÁÔÓȉ›Ô˘ ÔÈΛÏÏÂÈ ÛËÌ·ÓÙÈο ÛÙÔ˘˜ ‰È¿ÊÔÚÔ˘˜ ÏËı˘ÛÌÔ‡˜ Î·È ‰ÂÓ ÌÔÚ› Ó· ˘ÔÏÔÁÈÛÙ› ˘ÔıÂÙÈο ·fi ‰Â‰Ô̤ӷ ¿ÏÏˆÓ ÏËı˘ÛÌÈ·ÎÒÓ ÔÌ¿‰ˆÓ. √ ηıÔÚÈÛÌfi˜ ÙÔ˘ ›‰Ô˘˜ Î·È Ù˘ Û˘¯ÓfiÙËÙ·˜ ÙˆÓ ‰È·ÊfiÚˆÓ ÌÂÙ·ÏÏ¿ÍÂˆÓ ÙÔ˘ CYP21 ÁÔÓȉ›Ô˘ fiˆ˜ ·ÚÔ˘ÛÈ¿ÛÙËηÓ, Â›Ó·È È‰È·›ÙÂÚ· ¯Ú‹ÛÈÌÔ˜: ·) ÁÈ· ÙËÓ ·Ó·˙‹ÙËÛË ÂÙÂÚÔ˙˘ÁˆÙÒÓ Ù˘ ÓfiÛÔ˘ ÛÙÔÓ ÁÂÓÈÎfi ÏËı˘ÛÌfi Î·È ÙË ¯ÔÚ‹ÁËÛË ÁÂÓÂÙÈÎÒÓ Û˘Ì‚Ô˘ÏÒÓ, ‚) ÁÈ· ÙËÓ ¤ÁηÈÚË ·Ó·ÁÓÒÚÈÛË ÙˆÓ ‡ÔÙˆÓ ÁÈ· ÙË ‰È·Ù·Ú·¯‹ ÂÌ‚Ú‡ˆÓ ηٿ ÙÔÓ ÚÔÁÂÓÓËÙÈÎfi ¤ÏÂÁ¯Ô Î·È Á) ÁÈ· ÙË ‰ËÌÈÔ˘ÚÁ›· ‚¿Û˘ ‰Â‰ÔÌ¤ÓˆÓ ÚÔÎÂÈ̤ÓÔ˘ Ó· ÂÊ·ÚÌÔÛÙ› ÚfiÁÚ·ÌÌ· screening ÛÙÔÓ ÂÏÏËÓÈÎfi
Detection of congenital adrenal hyperplasia carrier frequency in Greece by molecular methods
A. Dastamani, M. Dracopoulou, A. Sertedaki, G.P. Chroussos, A. Dakou - Voutetakis
Department of Endocrinology-Metabolism, 1st Pediatric Clinic, University of Athens, “Agia Sofia” Children’s Hospital
Abstract
Background: Defective steroidogenesis caused by mutations of the 21-hydroxylase gene (CYP21), is the most common cause of congenital adrenal hyperplasia (CAH), covering about 95% of cases of CAH.
Data on the gene frequency based on molecular studies are scarce. The aim of this study was to determine the nature of CYP21 gene defects, their frequencies and their geographic distribution in the Hellenic population, which are of theoretical and practical importance for genetic counseling.
Methods: Blood samples from unrelated neonates were obtained from maternity hospitals around Greece. DNA analysis was carried out on 762 chromosomes. Allele-specific PCR was employed to identify 12 molecular defects of the CYP21 gene: homozygous deletion/conversion, P30L and W22X (exon 1), I2splice (intron 2), 8bpdE3 (exon 3), I172N (exon 4), ClusterE6 (exon 6), V281L and F306+T (exon 7), Q318X and R356W (exon 8), P453S (exon 10). It had previously been shown that these mutations account for 94% of CYP21 defects in the Greek patient population.
Results: Thirty-nine mutant alleles (5.12%) were detected, all in the heterozygous state. Thirteen mutant alleles (1.7%) carried mutations associated with the classical (C) form of 21-hydroxylase deficiency; 10 Q318X (1.3%), 1 I172N (0.14%), 1 π2splice (0.14%), 1 W22X (0.14%). Twenty-six mutant alleles (3.28%) carried mutations concordant with the non-classical (NC) form of the disease; 17 V281L (2.23%), 7 P453S (0.98%) and 2 P30L (0.28%). In this study, 10.2% of the subjects were found to be carriers of molecular defects of 21-hydroxylase deficiency. The heterozygote frequency for C form was 3.4% and for NC forms 6.8%. It was estimated in the Hellenic population the incidence of the NC form is 1:859 and of the C form 1:3,436. The calculated gene frequency is 0.051.
Conclusions: The carrier frequency is higher in Greek population than in Chinese and New Zealand populations who also underwent direct genetic testing of the CYP21 gene. It was confirmed that previous findings based on hormonal newborn screening appear inaccurate and underestimate the frequency of CAH. Gene frequency varies significantly in different populations and cannot be extrapolated from the data of some population groups only. The findings of this study can be applied: a) to detect heterozygotes in the general population, a prerequisite for genetic counseling, b) for early recognition of the affected fetus during prenatal testing, important for prevention of virilization of the female fetus, and c) to create a data base for establishing a neonatal screening programme in the Greek population.
Key words: Congenital adrenal hyperplasia, 21-hydroxylase, mutations, carrier frequency.
∂ÈÛ·ÁˆÁ‹
∏ Û˘ÁÁÂÓ‹˜ ˘ÂÚÏ·Û›· ÂÈÓÂÊÚȉ›ˆÓ (™À∂) ·ÔÙÂÏ› ÎÏËÚÔÓÔÌÈ΋ ‰È·Ù·Ú·¯‹ Ù˘
ÂÈÓÂÊÚȉȷ΋˜ ÛÙÂÚÂÔÂȉÔÁ¤ÓÂÛ˘, Ô˘ ÔÊ›ÏÂÙ·È Û ·Ó¿ÚÎÂÈ· ÂÓfi˜ ·fi Ù· 5 ¤Ó˙˘Ì· Ô˘ Û˘ÌÌÂÙ¤¯Ô˘Ó ÛÙË Û‡ÓıÂÛË Ù˘ ÎÔÚÙÈ˙fiÏ˘ ·fi ¯ÔÏËÛÙÂÚfiÏË. ¶ÂÚ·ÈÙ¤Úˆ ı· Á›ÓÂÈ ·Ó·ÊÔÚ¿ ÛÙËÓ ·Ó¿ÚÎÂÈ· Ù˘ 21-˘‰ÚÔÍ˘Ï¿Û˘, ·ÊÔ‡ Ë ÏÂÈÔÓfiÙËÙ· ÙˆÓ ÂÚÈÙÒÛÂˆÓ ™À∂ (90-95%) ÚÔηÏÂ›Ù·È ·fi ·Ó¿ÚÎÂÈ· Ù˘ 21-˘‰ÚÔÍ˘Ï¿Û˘, ÂÓfi˜ ÌÈÎÚÔÛˆÌÈ·ÎÔ‡ ΢ÙÔ¯ÚÒÌ·ÙÔ˜ ƒ450, Ô˘ ηٷχÂÈ ÙËÓ ·ÓÙ›‰Ú·ÛË ÌÂÙ·ÙÚÔ‹˜ Ù˘ 17-√∏ ÚÔÁÂÛÙÂÚfiÓ˘ (17√∏P) Û 11-‰ÂÔ͢ÎÔÚÙÈ˙fiÏË. ∏ ÂÓ˙˘ÌÈ΋ ·˘Ù‹ ‰È·Ù·Ú·¯‹, ˆ˜ ·Ó·Ì¤ÓÂÙ·È, ¤¯ÂÈ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙË ÌÂȈ̤ÓË ·Ú·ÁˆÁ‹ ÎÔÚÙÈ˙fiÏ˘, Ë ÔÔ›· Ô‰ËÁ› - ̤ۈ ÙÔ˘ Ì˯·ÓÈÛÌÔ‡ ·ÚÓËÙÈ-
ÙÔ˘ÚÁ›· ÙˆÓ ÂÈÓÂÊÚȉ›ˆÓ. √È Úfi‰ÚÔ̘ Ô˘Û›Â˜ Ô˘ ·ıÚÔ›˙ÔÓÙ·È, ÏfiÁˆ ÙÔ˘ ˘¿Ú¯ÔÓÙÔ˜ ÂÓ˙˘ÌÈÎÔ‡ ÊÚ·ÁÌÔ‡, ÚÔˆıÔ‡ÓÙ·È ÚÔ˜ ·Ú·ÁˆÁ‹ ·Ó‰ÚÔÁfiÓˆÓ (∂ÈÎfiÓ· 1). ø˜ ÂÎ ÙÔ‡ÙÔ˘, ˘¿Ú¯ÂÈ ‰ÈÙÙ‹ ÔÚÌÔÓÈ΋ ·Ú¤ÎÎÏÈÛË: ·Ó¿Ú-
ÎÂÈ· ÎÔÚÙÈ˙fiÏ˘ Î·È ˘ÂÚ·Ó‰ÚÔÁÔÓ·ÈÌ›· (1).
√ ‚·ıÌfi˜ Ù˘ ÂÓ˙˘ÌÈ΋˜ ·Ó¿ÚÎÂÈ·˜ Ù˘ 21-˘‰ÚÔÍ˘Ï¿Û˘ ηıÔÚ›˙ÂÈ ÙË ‚·Ú‡ÙËÙ· Ù˘
ÎÏÈÓÈ΋˜ ¤ÎÊÚ·Û˘ Ù˘ ™À∂. ¶Ï‹Ú˘ ·ÒÏÂÈ·
Ù˘ ‰Ú·ÛÙÈÎfiÙËÙ·˜ Ù˘ 21-˘‰ÚÔÍ˘Ï¿Û˘ Ô‰ËÁ› Û ‚·ÚÈ¿ ÎÏÈÓÈ΋ ÂÈÎfiÓ· (ÎÏ·ÛÈ΋ ÌÔÚÊ‹),
ÂÓÒ Ë ÌÂÚÈ΋ ·Ó¿ÚÎÂÈ· ¤¯ÂÈ ËÈfiÙÂÚË ÎÏÈÓÈ΋ ¤ÎÊÚ·ÛË (ÌË ÎÏ·ÛÈ΋ ÌÔÚÊ‹). ∏ ÎÏ·ÛÈ΋ ÌÔÚÊ‹ Ù˘ ÓfiÛÔ˘ Ù·ÍÈÓÔÌÂ›Ù·È ÛÙȘ ·ÎfiÏÔ˘ı˜ ‰‡Ô ˘ÔÔÌ¿‰Â˜: ·) ªÔÚÊ‹ Ì ·ÒÏÂÈ· ¿Ï·ÙÔ˜ (salt wastingSW). ∞ÔÙÂÏ› ‚·ÚÈ¿ ÂÓ˙˘ÌÈ΋ ‰È·Ù·Ú·¯‹ Ô˘
Correspondence: Antonia Dastamani 5, ∫alliga Str. 152 37, Filothei
Date of submission: 19-05-2006
Date of approval: 24-05-2006
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P-450scc
¢5-ÚÂÁÓÂÓÔÏfiÓËP-450c17
3‚-HSD
HSD
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ƒ-450c21
ƒ-450c17
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(18-√∏)
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18-OH-ÎÔÚÙÈÎÔÛÙÂÚfiÓË
(18-ÔÍÂȉ¿ÛË)
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ƒ-450c21
¢ÂÛÌÔÏ¿ÛË
AÓ‰ÚÔÛÙÂÓ‰ÈfiÓË17‚-HSD∆ÂÛÙÔÛÙÂÚfiÓË
ƒ-450c17
¢ÂÛÌÔÏ¿ÛË
¢ÂÛÔ͢ÎÔÚÙÈ˙fiÏË
ƒ-450c17
∫ÔÚÙÈ˙fiÏË
‰È·ÁÓˆÛÙ› ¤ÁηÈÚ·, Ô‰ËÁ› Û ·Ê˘‰¿ÙˆÛË, ÔͤˆÛË, Ô˘Ú·ÈÌ›·, ηٷÏËÍ›· Î·È ÙÂÏÈο ÛÂ
ı¿Ó·ÙÔ ÙȘ ÚÒÙ˜ ‚‰ÔÌ¿‰Â˜ Ù˘ ˙ˆ‹˜. ™ÙȘ
ÂÚÈÙÒÛÂȘ ·˘Ù¤˜, Ù· ÌÂÓ ı‹Ï· ÎÈÓ‰˘Ó‡ԢÓ
Ó· ¯·Ú·ÎÙËÚÈÛÙÔ‡Ó ˆ˜ ¿ÚÚÂÓ· ηٿ ÙË Á¤ÓÓË-
ÛË, ÂÓÒ ÙfiÛÔ Ù· ·ÁfiÚÈ· fiÛÔ Î·È Ù· ÎÔÚ›ÙÛÈ·
ÎÈÓ‰˘ÓÂ‡Ô˘Ó ·fi ·ÈÊÓ›‰ÈÔ ı¿Ó·ÙÔ (1).
‚) ªÔÚÊ‹ Ì ·Ï‹ ·ÚÚÂÓÔÔ›ËÛË (simple virilizing - SV). ™ÙË ÌÔÚÊ‹ ·˘Ù‹ ‰È·ÈÛÙÒÓÂÙ·È
ÔÈΛÏÔ˘ ‚·ıÌÔ‡ ·ÚÚÂÓÔÔ›ËÛË ÙˆÓ ıËϤˆÓ
Î·È ÚÒÈÌË ‹‚Ë, Ì ÂÈÙ¿¯˘ÓÛË ÙfiÛÔ Ù˘ Ûˆ-
Ì·ÙÈ΋˜ ·Ó¿Ù˘Í˘ fiÛÔ Î·È Ù˘ ÛÎÂÏÂÙÈ΋˜
ˆÚ›Ì·ÓÛ˘ Î·È ÛÙ· ‰‡Ô ʇϷ, ÂÓÒ ‰ÂÓ ·Ú·ÙË-
ÚÔ‡ÓÙ·È ËÏÂÎÙÚÔÏ˘ÙÈΤ˜ ‰È·Ù·Ú·¯¤˜ (1).
∏ ÌË ÎÏ·ÛÈ΋ ÌÔÚÊ‹ (non classical - NC)
Ù˘ ™À∂ ¤¯ÂÈ ÔÈΛÏË ÎÏÈÓÈ΋ ¤ÎÊÚ·ÛË Î·È Â-
ÚÈÏ·Ì‚¿ÓÂÈ ÚÒÈÌË ÙÚ›¯ˆÛË ÙÔ˘ ÂÊË‚·›Ô˘, ˘ÂÚÙÚ›¯ˆÛË, ·ÎÌ‹ Î·È ‰È·Ù·Ú·¯¤˜ Ù˘ ÂÌÌ‹ÓÔ˘ Ú‡Ûˆ˜ Î·È Ù˘ ÁÔÓÈÌfiÙËÙ·˜ (1).
3‚-
ƒ-450
∞ڈ̷ٿÛË
√ÈÛÙÚfiÓË√ÈÛÙÚ·‰ÈfiÏË
·fi 1:100 ¤ˆ˜ 1:1.000, ·Ó¿ÏÔÁ· Ì ÙËÓ ÏËı˘ÛÌȷ΋ ÔÌ¿‰· (2). ∏ ÌË ÎÏ·ÛÈ΋ ÌÔÚÊ‹ Ù˘ ™À∂ ·ÔÙÂÏ› ÙÔ Û˘¯ÓfiÙÂÚÔ, ÁÂÓÂÙÈÎÒ˜
ηıÔÚÈ˙fiÌÂÓÔ, ÌÂÙ·‚ÔÏÈÎfi ÓfiÛËÌ· ÛÙÔÓ ¿ÓıÚˆÔ (3).
∆Ô ¤Ó˙˘ÌÔ Ù˘ 21-˘‰ÚÔÍ˘Ï¿Û˘ Â›Ó·È ¤Ó· ÌÈÎÚÔÛˆÌÈ·Îfi ΢Ùfi¯ÚˆÌ· ƒ450 (ƒ450c21), Ô˘ Έ‰ÈÎÔÔÈÂ›Ù·È ·fi ÙÔ ÁÔÓ›‰ÈÔ CYP21 (1). ∆Ô ÁÔÓ›‰ÈÔ ·˘Ùfi ‰ڿ˙ÂÙ·È ÛÙÔ ‚Ú·¯‡ ÛΤÏÔ˜ ÙÔ˘ ¯ÚˆÌÔÛÒÌ·ÙÔ˜ 6 (6p21,3) Î·È Û ÁÂÈÙÓ›·ÛË Ì ٷ ÁÔÓ›‰È· ÙÔ˘ Û˘ÌÏËÚÒÌ·ÙÔ˜, ÛÙËÓ ÂÚÈÔ¯‹ ÙÔ˘ Ì›˙ÔÓÔ˜ Û˘ÛÙ‹Ì·ÙÔ˜ ÈÛÙÔÛ˘Ì‚·ÙfiÙËÙ·˜. ™Â ·fiÛÙ·ÛË 30kb ·fi ÙÔ ÁÔÓ›‰ÈÔ ·˘Ùfi ÂÓÙÔ›˙ÂÙ·È ÙÔ „¢‰ÔÁÔÓ›‰ÈÔ (CYP21ƒ). ∆· ‰‡Ô ·˘Ù¿ ÁÔÓ›‰È· ¤¯Ô˘Ó Ì‹ÎÔ˜ 3kb ÙÔ Î·ı¤Ó·. ¶·Ú¿ ÙËÓ ˘„ËÏ‹ ÔÌÔÏÔÁ›· ÙˆÓ ‰‡Ô ·˘ÙÒÓ ÁÔÓȉ›ˆÓ (98%), ÙÔ „¢‰ÔÁÔÓ›‰ÈÔ Â›Ó·È ÙÂÏ›ˆ˜ ·ÓÂÓÂÚÁfi. √È ÌÂÙ·ÏÏ¿ÍÂȘ Ô˘ Ô‰ËÁÔ‡Ó Û ·Ó·Ú΋ ·Ú·ÁˆÁ‹ ÙÔ˘ ÂÓ˙‡ÌÔ˘ 21-˘‰ÚÔÍ˘Ï¿ÛË Â›Ó·È ·ÔÙ¤ÏÂÛÌ· ‰‡Ô Ù‡ˆÓ ·Ó·Û˘Ó‰˘·ÛÌÒÓ ÌÂٷ͇ ÙˆÓ ‰‡Ô ·˘ÙÒÓ ÁÔÓȉ›ˆÓ (CYP21-CYP21P): ·) ¿ÓÈÛÔ˜ ÂȯȷÛÌfi˜ ηٿ ÙË Ì›ˆÛË, Ô˘ Ô‰ËÁ› Û ϋÚË ·¿ÏÂÈ„Ë ÙÔ˘ CYP21 (deletion) Î·È ‚) Ê·ÈÓfiÌÂÓ· ÌÂÙ·ÙÚÔ‹˜ (conversion) Ô˘ ÚÔηÏÔ‡Ó ÙË ÌÂÙ·ÊÔÚ¿ ·ÓÂÓÂÚÁÒÓ ÌÂÙ·ÏÏ¿ÍÂˆÓ ·fi ÙÔ CYP21P ÛÙÔ CYP21, fiÔ˘ ηı›ÛÙ·ÓÙ·È ÊıÔÚÔÔȤ˜ (1). √È ÁÓˆÛÙ¤˜ ‰È·Ù·Ú·¯¤˜ ÙÔ˘ ÁÔÓȉ›Ô˘ CYP21 Ô˘ Ô‰ËÁÔ‡Ó ÛÙȘ ‰È¿ÊÔÚ˜ ÌÔÚʤ˜ Ù˘ ·Ó¿ÚÎÂÈ·˜ Ù˘ 21-˘‰ÚÔÍ˘Ï¿Û˘ Â›Ó·È ··Ï›„ÂȘ/
·ÏÏËÏÔ˘¯ÈÒÓ ÙÔ˘ CYP21P („¢‰ÔÁÔÓȉ›Ô˘). ™Â οıÂ
‰Â›ÁÌ· ·Ó·˙ËÙ‹ıËÎ·Ó ÔÈ ·ÎfiÏÔ˘ı˜ 12 ÌÔÚȷΤ˜ ‰È·Ù·-
Ú·¯¤˜ ÙÔ˘ ÁÔÓȉ›Ô˘ CYP21: ·) √Ìfi˙˘ÁÔ˜ ·¿ÏÂÈ„Ë/ÌÂ-
Ù·ÙÚÔ‹ ÙÔ˘ ÁÔÓȉ›Ô˘ CYP21, ‚) ÌÂÙ¿ÏÏ·ÍË P30L ηÈ
W22X ÛÙÔ ÚÒÙÔ ÂÍÒÓÈÔ, Á) ÌÂÙ¿ÏÏ·ÍË π2splice ÛÙÔ ‰Â‡-
ÙÂÚÔ ÂÛÒÓÈÔ, ‰) ·¿ÏÂÈ„Ë 8 ‚¿ÛÂˆÓ 8bpdE3 ÛÙÔ ÙÚ›ÙÔ
ÂÍÒÓÈÔ, Â) ÌÂÙ¿ÏÏ·ÍË π172¡ ÛÙÔ Ù¤Ù·ÚÙÔ ÂÍÒÓÈÔ, ÛÙ)
Û‡ÌÏÂÁÌ· 3 ÌÂÙ·ÏÏ¿ÍÂˆÓ Cluster E6 ÛÙÔ ¤ÎÙÔ ÂÍÒÓÈÔ, ˙) ÌÂÙ¿ÏÏ·ÍË V281L Î·È F306+∆ ÛÙÔ ¤‚‰ÔÌÔ ÂÍÒÓÈÔ, Ë)
ÌÂÙ¿ÏÏ·ÍË Q318X Î·È R356W ÛÙÔ fiÁ‰ÔÔ ÂÍÒÓÈÔ, ı) ÌÂ-
Ù¿ÏÏ·ÍË P453S ÛÙÔ ‰¤Î·ÙÔ ÂÍÒÓÈÔ. √È ÂÓÓ¤· ·fi ·˘Ù¤˜ ÙȘ ÌÔÚȷΤ˜ ‰È·Ù·Ú·¯¤˜ Ô˘ ÌÂÏÂÙ‹ıËηÓ, ·ÓÙÈÚÔÛˆ-
Â‡Ô˘Ó ÙÔ 94% ÙˆÓ ‰È·Ù·Ú·¯ÒÓ ÙÔ˘ ÁÔÓȉ›Ô˘ CYP21 ÛÂ
ÏËı˘ÛÌfi ∂ÏÏ‹ÓˆÓ ·Û¯fiÓÙˆÓ, Û‡Ìʈӷ Ì ÚÔËÁÔ‡ÌÂÓË ÌÂϤÙË ÙÔ˘ ÂÚÁ·ÛÙËÚ›Ô˘ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ (5). ∂Ê·ÚÌfiÛÙËÎÂ Ë ‰ÔÎÈÌ·Û›· x2 ˆ˜ ÎÚÈÙ‹ÚÈÔ “ηϋ˜ ÂÊ·ÚÌÔÁ‹˜” (goodness of fit) Î·È ÛÙË Û˘Ó¤¯ÂÈ· Ë Â͛ۈÛË Hardy-Weinberg, ÚÔÎÂÈ̤ÓÔ˘ Ó· ˘ÔÏÔÁÈÛÙ› Ë Û˘¯ÓfiÙËÙ· ÙÔ˘ ÁÔÓȉ›Ô˘ Î·È Ù˘ ÓfiÛÔ˘ (11).
∞ÔÙÂϤÛÌ·Ù·
∆ÚÈ¿ÓÙ· ÂÓÓ¤· ·fi Ù· 762 ·ÏÏËÏfiÌÔÚÊ·
¤ÊÂÚ·Ó ÌÂÙ¿ÏÏ·ÍË ÙÔ˘ ÁÔÓȉ›Ô˘ CYP21 (5,12%), fiÏ· Û ÂÙÂÚfi˙˘ÁË Î·Ù¿ÛÙ·ÛË. ∞fi Ù·
39 ·˘Ù¿ ·ÏÏËÏfiÌÔÚÊ·, 13 (1,7%) ¤ÊÂÚ·Ó ÌÂ-
Ù·ÏÏ¿ÍÂȘ Ô˘ Û¯ÂÙ›˙ÔÓÙ·È Ì ÙËÓ ÎÏ·ÛÈ΋
ÌÔÚÊ‹ Ù˘ ™À∂: 10 Q318X (1,3%), 1 I172N (0,13%), 1 I2splice (0,13%), 1 W22X (0,13%) Î·È 26 (3,4%) ¤ÊÂÚ·Ó ÌÂÙ·ÏÏ¿ÍÂȘ Ù˘ ÌË ÎÏ·-
ÛÈ΋˜ ÌÔÚÊ‹˜ Ù˘ ÓfiÛÔ˘: 17 V281L (2,23%), 7 P453S (0,92%), 2 P30L (0,26%). √È ÌÂÙ·ÏÏ¿ÍÂȘ Ô˘ ·ÓȯÓ‡ıËÎ·Ó Î·È ÔÈ ·ÓÙ›ÛÙÔȯ˜ Û˘¯ÓfiÙËÙ¤˜ ÙÔ˘˜ Ê·›ÓÔÓÙ·È ÛÙÔ ¶›Ó·Î· 1. ™ÙÔÓ
¶›Ó·Î· 2 ·Ó·ÁÚ¿ÊÔÓÙ·È Ë Û˘¯ÓfiÙËÙ· ÙˆÓ ÂÙÂ-
ÚÔ˙˘ÁˆÙÒÓ ÛÙȘ ‰È¿ÊÔÚ˜ ÂÚÈÔ¯¤˜ Ù˘ ∂ÏÏ¿‰·˜, ηıÒ˜ Î·È ÙÔ Â›‰Ô˜ ÙˆÓ ÌÂÙ·ÏÏ¿ÍÂˆÓ Ô˘
·ÓȯÓ‡ıËÎ·Ó ÛÙȘ ÂÚÈÔ¯¤˜ ·˘Ù¤˜.
™˘ÓÂÒ˜ ÔÛÔÛÙfi 10,2% ÙˆÓ ÓÂÔÁÓÒÓ Ù˘
·ÚÔ‡Û·˜ ÌÂϤÙ˘ ‹Ù·Ó ÊÔÚ›˜ ÙˆÓ ‰È·Ù·Ú·-
¯ÒÓ Ù˘ 21-˘‰ÚÔÍ˘Ï¿Û˘. ªÂ ÙËÓ ÂÊ·ÚÌÔÁ‹ Ù˘ ‰ÔÎÈÌ·Û›·˜ ¯2, ‰È·ÈÛÙÒıËÎÂ Û˘Ìʈӛ· ÌÂٷ͇ Ù˘ ·Ú·ÙËÚÔ‡ÌÂÓ˘ (10,2%) Î·È Ù˘ ·Ó·ÌÂÓfiÌÂÓ˘ (9,7%) Û˘¯ÓfiÙËÙ·˜ ÙˆÓ ÂÙÂÚÔ˙˘ÁˆÙÒÓ. ∏ Û˘¯ÓfiÙËÙ· ÙˆÓ ÂÙÂÚÔ˙˘ÁˆÙÒÓ Ù˘
ÎÏ·ÛÈ΋˜ ÌÔÚÊ‹˜ Ù˘ ÓfiÛÔ˘ ‹Ù·Ó 3,4% Î·È Ù˘ ÌË ÎÏ·ÛÈ΋˜ ÌÔÚÊ‹˜ 6,8%. ªÂ ÙËÓ ÂÊ·ÚÌÔÁ‹ Ù˘ Â͛ۈÛ˘ Hardy-Weinberg ˘ÔÏÔÁ›˙ÂÙ·È fiÙÈ Ë Û˘¯ÓfiÙËÙ· Ù˘ ÎÏ·ÛÈ΋˜ ÌÔÚÊ‹˜ ·Ó¤Ú¯ÂÙ·È Û 1:3.436 Î·È Ù˘ ÌË ÎÏ·ÛÈ΋˜ ÌÔÚÊ‹˜ Û 1:859. ∏ ˘ÔÏÔÁÈÛı›۷ Û˘¯ÓfiÙËÙ· ÙÔ˘ ÁÔÓȉ›Ô˘ (gene frequency) Â›Ó·È 0.051 (¶›Ó·Î·˜ 3). ™˘˙‹ÙËÛË
™ÙËÓ ·ÚÔ‡Û· ÌÂϤÙË ·Ó·Ï‡ıËΠ¤Ó· Ù˘¯·›Ô Î·È ·ÓÙÈÚÔÛˆÂ˘ÙÈÎfi ‰Â›ÁÌ· ÓÂÔÁÓÒÓ ·fi ‰È¿ÊÔÚ˜ ÂÚÈÔ¯¤˜ Ù˘ ∂ÏÏ¿‰·˜. ∞Ó·˙ËÙ‹ıËÎ·Ó 12 ÌÔÚȷΤ˜ ‰È·Ù·Ú·¯¤˜ ÙÔ˘ ÁÔÓȉ›Ô˘ CYP21 Û 381 ÓÂÔÁÓ¿, ÚÔÎÂÈ̤ÓÔ˘ Ó· ηıÔÚÈÛÙ› Ë Û˘¯ÓfiÙËÙ· ÙˆÓ ÂÙÂÚÔ˙˘ÁˆÙÒÓ Ù˘ ™À∂
Ù˘ 21-˘‰ÚÔÍ˘Ï¿Û˘
ÚÔ·„ÂÈ ·fi
ÂÊ·ÚÌÔÁ‹ ÌÔÚÈ·ÎÒÓ Ù¯ÓÈÎÒÓ, Ô‰ËÁ› ÛÙÔ Û˘Ì¤Ú·ÛÌ· fiÙÈ Ë Û˘¯ÓfiÙËÙ· Ù˘ ÓfiÛÔ˘ Î·È ÙˆÓ ÂÙÂÚÔ˙˘ÁˆÙÒÓ Â›Ó·È Ôχ ÌÂÁ·Ï‡ÙÂÚË ÛÙÔÓ ÂÏÏËÓÈÎfi ÏËı˘ÛÌfi ¤Ó·ÓÙÈ ÙˆÓ ‰‡Ô ·˘ÙÒÓ ÏËı˘ÛÌÒÓ. ∏ ‰È·ÊÔÚ¿ ·˘Ù‹ ÌÔÚ› Ó· ÔÊ›ÏÂÙ·È Û ̛· ÛÂÈÚ¿ ·Ú·ÁfiÓÙˆÓ, fiˆ˜ de novo ÌÂÙ·ÏÏ¿ÍÂȘ ‹ ÙÔ Ê·ÈÓfiÌÂÓÔ ÙÔ˘ È‰Ú˘Ù‹ (founder effect) Û ÔÚÈṲ̂Ó˜ ÁˆÁÚ·ÊÈΤ˜ ÂÚÈÔ¯¤˜ (1). ∏ Û˘¯ÓfiÙËÙ· ÙˆÓ ÂÙÂÚÔ˙˘ÁˆÙÒÓ ÛÙÔÓ ÂÏÏËÓÈÎfi ÏËı˘ÛÌfi Â›Ó·È ·Ó¿ÏÔÁË Ì ·˘Ù‹ Ù˘ ∞˘ÛÙÚ›·˜ (13) Î·È Ù˘ ÚÒËÓ °ÈÔ˘ÁÎÔÛÏ·‚›·˜ (13), Ô˘ ·Ó·Ê¤ÚÂÙ·È Û ÚfiÛÊ·ÙË ÌÂϤÙË. ∏ Û‡ÁÎÚÈÛË fï˜ ÙˆÓ ‰Â‰ÔÌ¤ÓˆÓ Ù˘ ÌÂϤÙ˘ ÙÔ˘ ÂÏÏËÓÈÎÔ‡ ÏËı˘ÛÌÔ‡ Ì ٷ ·ÔÙÂϤÛÌ·Ù· Ù˘ ÌÂϤÙ˘ ·˘Ù‹˜ ÚÔÛÎÚÔ‡ÂÈ Û ‰È·ÊÔÚ¤˜, ÙfiÛÔ ÛÙËÓ ÂÈÏÔÁ‹ ÙÔ˘ ˘ÏÈÎÔ‡ fiÛÔ Î·È ÛÙÔ Ì¤ÁÂıÔ˜ ÙÔ˘ ‰Â›ÁÌ·ÙÔ˜. ™ÙË ÙÂÏÂ˘Ù·›· ·˘Ù‹ ÌÂϤÙË, Ë ·Ó·˙‹ÙËÛË ÊÔÚ¤ˆÓ ¤ÁÈÓ Û 100 ÂÓ‹ÏÈη ¿ÙÔÌ·, Ô˘ ·Ú·ÎÔÏÔ˘ıÔ‡ÓÙ·Ó Û οÔÈÔ ÓÔÛÔÎÔÌ›Ô, ÂÓÒ ÛÙËÓ ·ÚÔ‡Û· ÌÂϤÙË Ô ·ÚÈıÌfi˜ ÙˆÓ ‰ÂÈÁÌ¿ÙˆÓ ‹Ù·Ó ÌÂÁ·Ï‡ÙÂÚÔ˜ Î·È Ë ‰ÂÈÁÌ·ÙÔÏË„›· Ù˘¯·›· Î·È ·ÊÔÚ¿ Û ÓÂÔÁÓÈÎfi ÏËı˘ÛÌfi.
¶›Ó·Î·˜ 1. ªÂÙ·ÏÏ¿ÍÂȘ ÙÔ˘ ÁÔÓȉ›Ô˘ CYP21 Ô˘ ·ÓȯÓ‡ıËÎ·Ó ªÂÙ·ÏÏ¿ÍÂȘ Ô˘ ·ÓȯÓ‡ıËηÓ∞ÏÏËÏfiÌÔÚÊ· (n)%∫ÏÈÓÈ΋ ÌÔÚÊ‹ V281L172,23NC P453S70,92NC P30L20,26NC Q318X101,3C I172N10,13C I2splice10,13C W22X10,13C
¶ÂÚÈÔ¯‹∞ÚÈıÌfi˜ ∞ÚÈıÌfi˜ ™˘¯ÓfiÙËÙ· ∂›‰Ô˜
‰ÂÈÁÌ¿ÙˆÓÂÙÂÚÔ˙˘ÁˆÙÒÓÂÙÂÚÔ˙˘ÁˆÙÒÓÌÂÙ·ÏÏ¿ÍÂˆÓ £Ú¿ÎË20420%V281L ª·Î‰ÔÓ›·33618,2%Q318X, V281L, P453S, I172N ¡. ∞ÈÁ·›Ô˘11218,2%V281L ◊ÂÈÚÔ˜13215,4%V281L £ÂÛÛ·Ï›·35411,4%V281L, P453S, I2splice ™ÙÂÚ¿ ∂ÏÏ¿‰·3239,3%Q318X, P453S ∞ı‹Ó·155138,4%Q318X, P453S, V281L, P30L, W22X ∫Ú‹ÙË2627,6%V281L ¶ÂÏÔfiÓÓËÛÔ˜5036%V281L, P30L ¡. πÔÓ›Ô˘7---
∏ Û˘¯ÓfiÙËÙ· Ù˘ ·Ó¿ÚÎÂÈ·˜ Ù˘ 21˘‰ÚÔÍ˘Ï¿Û˘ ·ÁÎÔÛÌ›ˆ˜ (¶›Ó·Î·˜ 4), fiˆ˜
ÚÔ·ÙÂÈ ·fi Ù· ÓÂÔÁÓÈο ÚÔÁÚ¿ÌÌ·Ù· ‰È·ÏÔÁ‹˜ (screening) Ô˘ ‚·Û›˙ÔÓÙ·È ÛÙË Ì¤ÙÚËÛË Ù˘ 17√∏ ÚÔÁÂÛÙÂÚfiÓ˘, ÁÈ· ÙËÓ ÎÏ·ÛÈ΋
ÌÔÚÊ‹ Ù˘ ÓfiÛÔ˘ Î˘Ì·›ÓÂÙ·È ·fi 1:5.000 ¤ˆ˜ 1:23.000 ÛÙȘ ‰È¿ÊÔÚ˜ ÏËı˘ÛÌȷΤ˜ ÔÌ¿‰Â˜ (14). ™ÙËÓ ∂˘ÚÒË, Ë Û˘¯ÓfiÙËÙ· Ù˘ ™À∂ ·fi ·Ó¿ÚÎÂÈ· Ù˘ 21-˘‰ÚÔÍ˘Ï¿Û˘ Û ÓÂÔÁÓÈÎfi
ÏËı˘ÛÌfi Î˘Ì·›ÓÂÙ·È ·fi 1:11.000 ¤ˆ˜ 1:15.000 (14). ∞fi Ù· ‰Â‰Ô̤ӷ ·˘Ù¿ ηÈ
ÂÊ·ÚÌfi˙ÔÓÙ·˜ ÙÔÓ ÓfiÌÔ Hardy-Weinberg, ÚÔ·ÙÂÈ fiÙÈ Ë Û˘¯ÓfiÙËÙ· ÙˆÓ ÂÙÂÚÔ˙˘ÁˆÙÒÓ
Ù˘ ™À∂ Â›Ó·È 1,7% ·ÁÎÔÛÌ›ˆ˜ (8), ·ÚÈıÌfi˜
Ô˘ ˘ÔÏ›ÂÙ·È Î·Ù¿ Ôχ ÙÔ˘ Ú·ÁÌ·ÙÈÎÔ‡.
∏ ·ÚÔ‡Û· ÌÂϤÙË Î·Ù·‰ÂÈÎÓ‡ÂÈ fiÙÈ Ë Û˘¯ÓfiÙËÙ· ÙˆÓ ÂÙÂÚÔ˙˘ÁˆÙÒÓ ÁÈ· ÙËÓ ÎÏ·ÛÈ΋ (3,4%) Î·È ÌË ÎÏ·ÛÈ΋ ÌÔÚÊ‹ (6,8%) Ù˘ ™À∂
Â›Ó·È Ôχ ÌÂÁ·Ï‡ÙÂÚË ·fi ÙË Û˘¯ÓfiÙËÙ· Ô˘
ÚÔ·ÙÂÈ ·fi Ù· ‰Â‰Ô̤ӷ ÙˆÓ ÚÔÁÚ·ÌÌ¿ÙˆÓ screening. ∞Ó¿ÏÔÁÔ Â›Ó·È ÙÔ Û˘Ì¤Ú·ÛÌ·
Ô˘ ¤¯ÂÈ ÂÍ·¯ı› ·fi ÙË ÌÂϤÙË ÛÙË ¡¤· ∑Ë-
Ï·Ó‰›· (9) Î·È ÛÙËÓ ∞˘ÛÙÚ›· (13). ∏ ‰È·ÊÔÚ¿ ·˘Ù‹ ÌÔÚ› Ó· Û¯ÂÙ›˙ÂÙ·È Ì ÙÔ ÁÂÁÔÓfi˜ fiÙÈ
ÂÚ›Ô˘ ÙÔ 14% ÙˆÓ ÂÚÈÙÒÛÂˆÓ Ù˘ ÎÏ·ÛÈ-
΋˜ÌÔÚÊ‹˜ Î·È ÙÔ 87% ÙˆÓ ÓÂÔÁÓÒÓ Ì ÌË ÎÏ·ÛÈ΋ ÌÔÚÊ‹ Ù˘ ™À∂ ‰ÂÓ ·ÓȯÓ‡ÔÓÙ·È
ÔÚÌÔÓÈÎfi ¤ÏÂÁ¯Ô Ô˘ Ú·ÁÌ·ÙÔÔÈÂ›Ù·È ÙȘ ÚÒÙ˜ Ë̤Ú˜ ˙ˆ‹˜ (15).
Ù· ÚÔÁÚ¿ÌÌ·Ù·
screening ÛÙ· ÓÂÔÁÓ¿ ¤‰ÂÈÍ·Ó ÌÂÁ·Ï‡ÙÂÚË Û˘-
Ì ÙȘ ¤Ú¢Ó˜ (case surveys) Ô˘ η٤ÁÚ·Ê·Ó Ù· ÂÚÈÛÙ·ÙÈο
‰Â‰Ô̤ӷ (8), ·Ô‰ÂÈÎÓ‡ÔÓÙ·È ·Ó·ÎÚÈ‚‹ Î·È ˘ÔÂÎÙÈÌÔ‡Ó ÛËÌ·ÓÙÈο ÙË Û˘¯ÓfiÙËÙ· ÙˆÓ ÂÙÂÚÔ˙˘ÁˆÙÒÓ Ù˘ ™À∂. ™˘ÌÂÚ·ÛÌ·ÙÈο, Ô ÌÔÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ ·Ô‰ÂÈÎÓ‡ÂÙ·È ·ÎÚÈ‚¤ÛÙÂÚË Ì¤ıÔ‰Ô˜ ÁÈ· ÙËÓ ·Ó›¯Ó¢ÛË ÙˆÓ ÂÙÂÚÔ˙˘ÁˆÙÒÓ Ù˘ ·Ó¿ÚÎÂÈ·˜ Ù˘ 21˘‰ÚÔÍ˘Ï¿Û˘ ÛÙÔÓ ÁÂÓÈÎfi ÏËı˘ÛÌfi. ¶·Ú¿ Ù·‡Ù·, Ë ÂÊ·ÚÌÔÁ‹ ÙÔ˘ ÌÔÚÈ·ÎÔ‡ ÂϤÁ¯Ô˘ Û ÌÂÁ·Ï‡ÙÂÚ˜ ÏËı˘ÛÌȷΤ˜ ÔÌ¿‰Â˜ Â›Ó·È ‰‡ÛÎÔÏË, ‰ÈfiÙÈ Â›Ó·È È‰È·›ÙÂÚ· ‰··ÓËÚ‹ Î·È ¯ÚÔÓÔ‚fiÚ·. ∂ÈϤÔÓ, Ë ·ÚÔ‡Û· ÌÂϤÙË ‰Â›¯ÓÂÈ
fiÙÈ Ë Û˘¯ÓfiÙËÙ· ÙˆÓ ÌÂÙ·ÏÏ¿ÍÂˆÓ ÙÔ˘ CYP21
ÁÔÓȉ›Ô˘ ÔÈΛÏÏÂÈ ÛËÌ·ÓÙÈο ÛÙÔ˘˜ ‰È¿ÊÔÚÔ˘˜
ÏËı˘ÛÌÔ‡˜ Î·È ‰ÂÓ ÌÔÚ› Ó· ˘ÔÏÔÁÈÛÙ›
·fi ‰Â‰Ô̤ӷ ¿ÏÏˆÓ ÏËı˘ÛÌÈ·ÎÒÓ ÔÌ¿‰ˆÓ. ™ÙÔÓ ÂÏÏËÓÈÎfi ÏËı˘ÛÌfi, Ë Ï¤ÔÓ Û˘¯Ó‹
¶›Ó·Î·˜ 3. ™˘¯ÓfiÙËÙ˜ ÂÙÂÚÔ˙˘ÁˆÙÒÓ, ÓfiÛÔ˘ Î·È ÁÔÓȉ›Ô˘ Û ‰È¿ÊÔÚ˜ ÏËı˘ÛÌȷΤ˜ ÔÌ¿‰Â˜
¶ÏËı˘ÛÌÔ› / ∞ÚÈıÌfi˜ ‰ÂÈÁÌ¿ÙˆÓ/ ∞ÚÈıÌfi˜ ÌÂÙ·ÏϿ͈ә˘¯ÓfiÙËÙ· ÂÙÂÚÔ˙˘ÁˆÙÒÓ™˘¯ÓfiÙËÙ· ÓfiÛÔ˘™˘¯ÓfiÙËÙ· ÁÔÓȉ›Ô˘
Ô˘ ÌÂÏÂÙ‹ıËηә‡ÓÔÏÔªÔÚʤ˜™‡ÓÔÏÔªÔÚʤ˜™‡ÓÔÏÔ
∫›Ó· / 1000 / 40,9%0,9% C1:49.3821:49.382 C0,0045
¡¤· ∑ËÏ·Ó‰›· / 603 / 94,8%2,8% C1:1.729 1:5.032 C0,024 2,0% NC1:10.100 NC
∞˘ÛÙÚ›· / 100 / 139%5% C1:4931:2.500 C0,045 4% NC1:1.111 NC
. °ÈÔ˘ÁÎÔÛÏ·‚›· / 100 / 1310%6% C1:4001:2.500 C0,05 4% NC1:1.111 NC
∂ÏÏ¿‰· / 381 / 1210,2%3,4% C1:3821:3.436 C0,051 6,8% NC1:859 NC
ÚÔÁÚ¿ÌÌ·Ù· screening ÓÂÔÁÓÒÓ
¶ÂÚÈÔ¯‹™˘¯ÓfiÙËÙ·∞ÚÈıÌfi˜ ÂÍÂÙ·Ûı¤ÓوӌΉÔÛË
AÏ¿Ûη, Yupik Eskimos1:2821.1311982 (16)
°·ÏÏ›·, La Reunion1:2.14114.9871987 (8)
IÙ·Ï›·1:18.000420.9601996 (17)
IÛÚ·‹Ï1:28.462113.8461997 (18)
™·Ô˘‰È΋ ∞Ú·‚›·1:14.24056.9581997 (18)
π·ˆÓ›·1:18.0004.500.0001997 (19)
™Ô˘Ë‰›·1:9.8001.500.0001998 (20)
∏¶∞, T¤Í·˜1:16.0081.900.0001998 (15)
N¤· ZËÏ·Ó‰›·1:23.344536.9151995 (21)
°·ÏÏ›·, Lille1:13.000408.1381999 (22)
B¤ÏÁÈÔ1:25.000206.1081997 (23)
∏¶∞, Wisconsin1:11.000149.6841997 (24)
∂Ï‚ÂÙ›·1:10.749333.2212002 (25)
√ÏÏ·Ó‰›·1:11.764176.6842001 (26)
ÌÔÚÊ‹ Ù˘ ÓfiÛÔ˘ Â›Ó·È Ë Q318X, ·ÎÔÏÔ˘ıÔ‡-
ÌÂÓË ·fi ÙËÓ P30L, I2splice Î·È W22X. ∏ ÌÂ-
Ù¿ÏÏ·ÍË V281L ·ÔÙÂÏ› ÙË Û˘¯ÓfiÙÂÚË ÌÔÚÈ·-
΋ ‰È·Ù·Ú·¯‹ Ù˘ ÌË ÎÏ·ÛÈ΋˜ ÌÔÚÊ‹˜ Ù˘
™À∂, ‰Â‰Ô̤ÓÔ Ô˘ ÂȂ‚·ÈÒÓÂÙ·È ÙfiÛÔ ·fi
¿ÏÏË ÌÂϤÙË Û ÏËı˘ÛÌfi ∂ÏÏ‹ÓˆÓ ·Û¯fi-
ÓÙˆÓ (5), fiÛÔ Î·È ·fi ÌÂϤÙ˜ Ô˘ Ú·ÁÌ·ÙÔ-
ÔÈ‹ıËÎ·Ó ‰ÈÂıÓÒ˜ (1). ∏ ˘„ËÏfiÙÂÚË Û˘¯Ófi-
ÙËÙ· ÂÙÂÚÔ˙˘ÁˆÙÒÓ ·Ú·ÙËÚ‹ıËΠÛÙȘ Â-
ÚÈÔ¯¤˜ £Ú¿Î˘, ª·Î‰ÔÓ›·˜ Î·È ¡‹ÛˆÓ ∞ÈÁ·›Ô˘, Ë ¯·ÌËÏfiÙÂÚË ÛÙËÓ ÂÚÈÔ¯‹ Ù˘ ¶ÂÏÔÔÓ-
Ó‹ÛÔ˘, ÂÓÒ ‰ÂÓ ·ÓȯÓ‡ıËÎ·Ó ÂÙÂÚÔ˙˘ÁÒÙ˜
ÛÙ· πfiÓÈ· ¡ËÛÈ¿. ∆· ‰Â‰Ô̤ӷ Ù˘ ·ÚÔ‡Û·˜ ¤Ú¢ӷ˜ ÚÔ-
ÛʤÚÔÓÙ·È ÁÈ· ÙË ‰ËÌÈÔ˘ÚÁ›· ‚¿Û˘ ‰Â‰ÔÌ¤ÓˆÓ ÁÈ· ÙËÓ ÂÊ·ÚÌÔÁ‹ ÚÔÁÚ¿ÌÌ·ÙÔ˜ screening ÛÙÔÓ ÂÏÏËÓÈÎfi ÏËı˘ÛÌfi, ÚÔÎÂÈ̤ÓÔ˘ Ó· ÌÂȈı› Ë ÓÔÛËÚfiÙËÙ· Î·È Ë ıÓËÛÈÌfiÙËÙ· Ô˘ Û¯ÂÙ›˙ÂÙ·È Ì ÙËÓ ÎÏ·ÛÈ΋ ÌÔÚÊ‹ Ù˘ ÓfiÛÔ˘. ∂ÈϤÔÓ, ı· ‰È¢ÎÔχÓÔ˘Ó ÙÔÓ ·Ú¯ÈÎfi ‰È·ÁÓˆÛÙÈÎfi ÚÔÛ·Ó·ÙÔÏÈÛÌfi ÛÙË ÌÔÚȷ΋ ‰ÈÂÚ‡ÓËÛË ÂÓfi˜ ¿Û¯ÔÓÙÔ˜ ‹ Ì›·˜ ÔÈÎÔÁ¤ÓÂÈ·˜, Ì ÂÎΛÓËÛË ·fi
Û˘Ì‚Ô˘ÏÒÓ.
π‰È·›ÙÂÚ· ¢¯·ÚÈÛÙԇ̠ÙÔ˘˜ Û˘Ó·‰¤ÏÊÔ˘˜ È·ÙÚÔ‡˜ Ô˘ Û˘Ó¤‚·Ï·Ó ÛËÌ·ÓÙÈο ÛÙËÓ ˘ÏÔÔ›ËÛË ·˘Ù‹˜ Ù˘ ÌÂϤÙ˘, ÁÈ· ÙË Û˘ÏÏÔÁ‹ ÙˆÓ ‰ÂÈÁÌ¿ÙˆÓ ·fi ÙÔÓ ÔÌÊ¿ÏÈÔ ÏÒÚÔ ÙˆÓ ÓÂÔÁÓÒÓ: °. ∞ÓÙˆÓ¿Î˘ (¶·ÓÂÈÛÙËÌÈ·Îfi ¡ÔÛÔÎÔÌÂ›Ô ƒ›Ô˘), °. ¢·ÛÙ·Ì¿ÓË (¡ÔÛÔÎÔÌÂ›Ô “∞ÏÂÍ¿Ó‰Ú·”), ¢È·Ì·ÓÙfiÔ˘ÏÔ˜ (ª·È¢ÙÈ΋ ∫ÏÈÓÈ΋ “πÔÎÚ¿ÙÂÈÔ” ∞ÁÚÈÓ›Ô˘), ∂. ¢Ô˘Ú¿ÙÛÔ˜ (ª·ÈÂ˘Ù‹ÚÈÔ “§ËÙÒ”), ∫·ÙÛ¿ÓÔ˘ (ª·È¢ÙÈ΋ ∫ÏÈÓÈ΋ “ªËÙ¤Ú·” §·Ì›·˜), ∏. ∫·ÙÛ›Î˘ (“πÔÎÚ¿ÙÂÈÔ” £ÂÛÛ·ÏÔӛ΢), º. ∫Ô˘‚‡‰Ë (ª·È¢ÙÈ΋ ∫ÏÈÓÈ΋ “ÕÁÈÔ˜ ∂Ï¢ı¤ÚÈÔ˜”), °. §È¿ÏÈÔ˜ (¶·ÓÂÈÛÙËÌÈ·Îfi ¡ÔÛÔÎÔÌÂ›Ô §¿ÚÈÛ·˜), ™. ™ËÊ¿Î˘ (¶∂¶∞°¡∏ ∫Ú‹Ù˘), £. ™Ù¤ÊÔ˜ (¶·ÓÂÈÛÙËÌÈ·Îfi ¡ÔÛÔÎÔÌÂ›Ô πˆ·ÓÓ›ÓˆÓ), π. ∆ÚÈ·ÓÙ·Ê˘ÏÏ›‰Ë˜ (¶·ÓÂÈÛÙËÌÈ·Îfi ¡ÔÛÔÎÔÌÂ›Ô ∞ÏÂÍ·Ó‰ÚÔ‡ÔÏ˘), π. ÷Ù˙‹˜ (ª·ÈÂ˘Ù‹ÚÈÔ “ª·Ú›Î· ∏ÏÈ¿‰Ë”).
µÈ‚ÏÈÔÁÚ·Ê›·
1.White PC, Speiser PW. Congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Endocr Rev 2000;21:245-291.
2.Miller WL. Genetics, diagnosis, and management of 21-hydroxylase deficiency. J Clin Endocrinol Metab 1994;78:241-246.
3.Speiser PW, Dupont B, Rubinstein P, Piazza A, Kastelan A, New MI. High frequency of nonclassical steroid 21-hydroxylase deficiency. Am J Hum Genet 1985;37:650-667.
4.Wedell A. Molecular genetics of congenital adrenal hyperplasia (21-hydroxylase deficiency): implica-
tions for diagnosis, prognosis and treatment. Acta Paediatr 1998;87:159-164.
5. Dracopoulou-Vabouli M, Maniati-Christidi M, Dacou-Voutetakis C. The spectrum of molecular defects of the CYP21 gene in the Hellenic population: variable concordance between genotype and phenotype in the different forms of congenital adrenal hyperplasia. J Clin Endocrinol Metab 2001;86:2845-2848.
6.Dacou-Voutetakis C, Dracopoulou M. High incidence of molecular defects of the CYP21 gene in patients with premature adrenarche. J Clin Endocrinol Metab 1999;84:1570-1574.
7.New MI, Lorenzen F, Lerner AJ, Kohn B, Oberfield SE, Pollack MS et al. Genotyping steroid 21-hydroxylase deficiency: hormonal reference data. J Clin Endocrinol Metab 1983;57:320-326.
8.Pang SY, Wallace MA, Hofman L, Thuline HC, Dorche C, Lyon IC et al. Worldwide experience in newborn screening for classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Pediatrics 1988;81:866-874.
9.Fitness J, Dixit N, Webster D, Torresani T, Pergolizzi R, Speiser PW et al. Genotyping of CYP21, linked chromosome 6p markers, and a sex-specific gene in neonatal screening for congenital adrenal hyperplasia. J Clin Endocrinol Metab 1999;84:960-966.
10.Wedell A, Luthman H. Steroid 21-hydroxylase deficiency: two additional mutations in salt-wasting disease and rapid screening of disease-causing mutations. Hum Mol Genet 1993;2:499-504.
11.Stern C. The Hardy-Weinberg law. Science 1943;97: 137-138.
12.Lee HH, Kuo JM, Chao HT, Lee YJ, Chang JG, Tsai CH et al. Carrier analysis and prenatal diagnosis of congenital adrenal hyperplasia caused by 21-hydroxylase deficiency in Chinese. J Clin Endocrinol Metab 2000;85:597-600.
13.Baumgartner-Parzer SM, Nowotny P, Heinze G, Waldhausl W, Vierhapper H. Carrier frequency of congenital adrenal hyperplasia (21-hydroxylase deficiency) in a middle European population. J Clin Endocrinol Metab 2005;90:775-778.
14.Pang S, Shook MK. Current status of neonatal screening for congenital adrenal hyperplasia. Curr Opin Pediatr 1997;9:419-423.
15.Therrell BL Jr, Berenbaum SA, Manter-Kapanke V, Simmank J, Korman K, Prentice L et al. Results of screening 1.9 million Texas newborns for 21-hy-
droxylase-deficient congenital adrenal hyperplasia. Pediatrics 1998;101:583-590.
16.Pang S, Murphey W, Levine LS, Spence DA, Leon A, LaFranchi S et al. A pilot newborn screening for congenital adrenal hyperplasia in Alaska. J Clin Endocrinol Metab 1982;55:413-420.
17.Balsamo A, Cacciari E, Piazzi S, Cassio A, Bozza D, Pirazzoli P et al. Congenital adrenal hyperplasia: neonatal mass screening compared with clinical diagnosis only in the Emilia-Romagna region of Italy, 1980-1995. Pediatrics 1996;98:362-367.
18.Sack J, Front H, Kaiserman I, Schreiber M. 21-Hydroxylase deficiency: screening and incidence in Israel. Horm Res 1997;48:115-119.
19.Tajima T, Fujieda K, Nakae J, Toyoura T, Shimozawa K, Kusuda S et al. Molecular basis of nonclassical steroid 21-hydroxylase deficiency detected by neonatal mass screening in Japan. J Clin Endocrinol Metab 1997;82:2350-2356.
20.Thil’en A, Nordenstrom A, Hagenfeldt L, von Dobeln U, Guthenberg C, Larsson A. Benefits of neonatal screening for congenital adrenal hyperplasia (21hydroxylase deficiency) in Sweden. Pediatrics 1998;101:E11.
21.Cutfield WS, Webster D. Newborn screening for congenital adrenal hyperplasia in New Zealand. J Pediatr 1995;126:118-121.
22.Cartigny-Maciejewski M, Guilley N, Vanderbecken S, Gonde S, Stuckens C, Ponte C et al. Neonatal screening of congenital adrenal hyperplasia due to 21-hydroxylase deficiency: Lille experience 19801996. Arch Pediatr 1999;6:151-158.
23.Van der Kamp HJ, Wit JM. Neonatal screening for congenital adrenal hyperplasia. Eur J Endocrinol 2004;151 (Suppl 3):U71-U75.
24.Allen DB, Hoffman GL, Fitzpatrick P, Laessig R, Maby S, Slyper A. Improved precision of newborn screening for congenital adrenal hyperplasia using weight-adjusted criteria for 17-hydroxyprogesterone levels. J Pediatr 1997;130:128-133.
25.Steigert M, Schoenle EJ, Biason-Lauber A, Torresani T. High reliability of neonatal screening for congenital adrenal hyperplasia in Switzerland. J Clin Endocrinol Metab 2002;87:4106-4110.
26.Van der Kamp HJ, Noordam K, Elvers B, Van Baarle M, Otten BJ, Verkerk PH. Newborn screening for congenital adrenal hyperplasia in the Netherlands. Pediatrics 2001;108:1320-1324.
∂ÚÁ·ÛÙ‹ÚÈÔ π·ÙÚÈ΋˜
°ÂÓÂÙÈ΋˜ ¶·ÓÂÈÛÙËÌ›Ô˘
∞ıËÓÒÓ, ¡ÔÛÔÎÔÌ›Ô
¶·›‰ˆÓ “∞Á›· ™ÔÊ›·”, ∞ı‹Ó·
AÏÏËÏÔÁÚ·Ê›·:
AÁÁÂÏÈ΋ ∫ÔÏȷϤÍË
∂ÚÁ·ÛÙ‹ÚÈÔ π·ÙÚÈ΋˜
°ÂÓÂÙÈ΋˜ ¶·ÓÂÈÛÙËÌ›Ô˘
∞ıËÓÒÓ
¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ
“∞Á›· ™ÔÊ›·”
∆.∫. 115 27, °Ô˘‰›, ∞ı‹Ó·
E-mail: akolial@cc.uoa.gr
∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 18-01-2006
∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 17-04-2006
ÀÂÚ¿ÚÈıÌ· ¯ÚˆÌÔÛˆÌÈο ıÚ·‡ÛÌ·Ù·
ÚÔÂÚ¯fiÌÂÓ· ·fi ÙÔ ¯ÚˆÌfiۈ̷ 15: Û˘Û¯¤ÙÈÛË Ê·ÈÓÔÙ‡Ô˘ - ÁÔÓÔÙ‡Ô˘
∞. ∫ÔÏȷϤÍË, ™. ∫›ÙÛÈÔ˘, Œ. ºÚ˘Û›Ú·, Ã. ™ÔÊÔÎÏ¤Ô˘˜, ∂. ∫Ô˘‚›‰Ë, ∫. ™·Ï·‚Ô‡Ú·, ∞. ª·‡ÚÔ˘
¶ÂÚ›ÏË„Ë
∂ÈÛ·ÁˆÁ‹: ∆· ˘ÂÚ¿ÚÈıÌ· ¯ÚˆÌÔÛˆÌÈο ıÚ·‡ÛÌ·Ù· (SMCs) ·ÔÙÂÏÔ‡Ó Û¿ÓȘ ¯ÚˆÌÔÛˆÌÈΤ˜ ·ÓˆÌ·Ï›Â˜ Î·È ÚÔηÏÔ‡Ó ÌÂÚÈ΋ ÙÚÈۈ̛· ÂÚÈÔ¯ÒÓ ÙÔ˘ ÁÔÓȉÈÒÌ·ÙÔ˜ Ì ¯·Ú·ÎÙËÚÈÛÙÈΤ˜ Ê·ÈÓÔÙ˘ÈΤ˜ ÂÈÙÒÛÂȘ. ŸÛ· ÚÔ¤Ú¯ÔÓÙ·È ·fi ÙÔ ¯ÚˆÌfiۈ̷ 15 ·ÔÙÂÏÔ‡Ó ÙÔ 50% ÙˆÓ ÂÚÈÙÒÛÂˆÓ Î·È ¤¯Ô˘Ó Èı·ÓÒ˜ ÙË ÌÂÁ·Ï‡ÙÂÚË ÛËÌ·Û›·, ‰ÈfiÙÈ ÛÙÔ ¯ÚˆÌfiۈ̷ ·˘Ùfi ÂÓÙÔ›˙ÂÙ·È Ë ÎÚ›ÛÈÌË ÂÚÈÔ¯‹ ÙˆÓ Û˘Ó‰ÚfiÌˆÓ Prader-Willi/Angelman (PWS/AS), ÛÙËÓ ÔÔ›· ·Ú·ÙËÚÂ›Ù·È ÁÔÓȉȷ΋ ·ÔÙ‡ˆÛË.
ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ: ¶·ÚÔ˘ÛÈ¿˙ÔÓÙ·È Ù· Â˘Ú‹Ì·Ù· ·fi ÙË ‰ÈÂÚ‡ÓËÛË 16 ÂÚÈÙÒÛÂˆÓ ÌÂ
SMC(15)s. ∏ ·Ú¯È΋ ÂÓÙfiÈÛË ¤ÁÈÓ ÌÂ
·Ó¿Ï˘ÛË Û 14 ·È‰È¿ Î·È Û 2 ‰Â›ÁÌ·Ù· ·ÌÓÈ·ÎÔ‡ ˘ÁÚÔ‡ Î·È Ë ¯ÚˆÌÔÛˆÌÈ΋
ÚԤϢÛË Ù·˘ÙÔÔÈ‹ıËΠ̠ÊıÔÚ›˙ÔÓÙ· in situ ˘‚ÚȉÈÛÌfi. ™Â fiϘ ÙȘ ÂÚÈÙÒÛÂȘ ÌÂÏÂÙ‹ıËÎÂ Ô Î·Ú˘fiÙ˘Ô˜ ÙˆÓ ÁÔÓ¤ˆÓ. ∞ÔÙÂϤÛÌ·Ù·: ™Â 15/16 ÂÚÈÙÒÛÂȘ (93,75%) ÙÔ SMC(15) ‚Ú¤ıËΠ۠ÔÛÔÛÙfi 100% ÙˆÓ ÌÂÙ·Ê¿ÛÂˆÓ Î·È ÌfiÓÔ Û ̛· (6,25%) ‹Ù·Ó ̈۷˚Îfi. ™Â 14 ÂÚÈÙÒÛÂȘ (87,5%) ÙÔ SMC(15) ‹Ù·Ó de novo Î·È Û 2 (12,5%) ÎÏËÚÔÓÔÌÈÎfi. ªÈÎÚ¿ SMC(15)s ·ÓȯÓ‡ÙËÎ·Ó Û 9/16 ÂÚÈÙÒÛÂȘ. ∂Ù¿ ÂÚÈÛÙ·ÙÈο Ì ÌÂÁ¿ÏÔ˘ ÌÂÁ¤ıÔ˘˜ SMC(15) ÌÂÏÂÙ‹ıËÎ·Ó Ì ÙÔÓ ·ÓȯÓÂ˘Ù‹ SNRPN/15qtel ÁÈ· ÙËÓ ÎÚ›ÛÈÌË ÂÚÈÔ¯‹ ÙˆÓ Û˘Ó‰ÚfiÌˆÓ PWS/AS. ™Â ‰‡Ô ·fi ·˘Ù¿ Ô˘ ÂÚÈ›¯·Ó ÙËÓ ÎÚ›ÛÈÌË ÂÚÈÔ¯‹, ·ÎÔÏÔ‡ıËÛ ÌÔÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜, Ô ÔÔ›Ô˜ ÂȂ‚·›ˆÛ ÙËÓ ·ÚÔ˘Û›· 3 ·ÏÏËÏfiÌÔÚÊˆÓ Ù˘ ÎÚ›ÛÈÌ˘ ÂÚÈÔ¯‹˜ PWS/AS Î·È ¤‰ÂÈÍ fiÙÈ ÙÔ SMC(15) ‹Ù·Ó ÌËÙÚÈ΋˜ ÚÔ¤Ï¢Û˘. ªÂϤÙË, fï˜, ÙÔ˘ ÚÔÙ‡Ô˘ ÌÂı˘Ï›ˆÛ˘ ÛÙÔ˘˜ ·ÛıÂÓ›˜ ·˘ÙÔ‡˜ ·¤ÎÏÂÈÛ ÙË ‰È¿ÁÓˆÛË Û˘Ó‰ÚfiÌˆÓ PWS/AS. ™˘ÌÂÚ¿ÛÌ·Ù·: ∆· Â˘Ú‹Ì·Ù· Ù˘ ÌÂϤÙ˘ ÂÈÛËÌ·›ÓÔ˘Ó ÙË ÛËÌ·Û›· ÙÔ˘ ÔÏÔÎÏËڈ̤ÓÔ˘ ÁÂÓÂÙÈÎÔ‡ ÂϤÁ¯Ô˘ ÙˆÓ SMC(15)s ÁÈ· ÙË Û˘Û¯¤ÙÈÛË ÁÔÓÔÙ‡Ô˘ - Ê·ÈÓÔÙ‡Ô˘ Î·È ÙË ÁÂÓÂÙÈ΋ ηıÔ‰‹ÁËÛË ÙˆÓ ÔÈÎÔÁÂÓÂÈÒÓ.
§¤ÍÂȘ ÎÏÂȉȿ: ÀÂÚ¿ÚÈıÌÔ
Supernumerary marker chromosomes derived from chromosome 15: phenotype - genotype correlation
Laboratory of Medical Genetics, University of Athens, “Aghia Sofia” Children’s Hospital
Correspondence: Aggeliki Kolialexi Laboratory of Medical Genetics, University of Athens “Aghia Sofia” Children’s Hospital 115 27, Goudi, Athens
E-mail: akolial@cc.uoa.gr
Date of submission: 18-01-2006
Date of approval: 17-04-2006
A. Kolialexi, S. Kitsiou, H. Fryssira, C. Sofocleous, E. Kouvidi, K. Salavoura, A. Mavrou
Abstract
Background: Supernumerary marker chromosomes (SMCs) are rare chromosomal abnormalities which cause partial trisomy of genomic regions with characteristic phenotypic effects. Those derived from chromosome 15 account for 50% of cases and are of great importance because this chromosome includes the critical region for the Prader-Willi/Angelman syndromes (PWS/AS).
Methods: The results of investigation of 16 cases of SMC(15) are presented. The initial identification was made by conventional cytogenetics analysis in 14 children and 2 amniotic fluid samples, and their chromosomal origin was determined by fluorescence in situ hybridisation (FISH). Parental karyotypes were studied in all cases.
Results: In 15/16 (93.75%) cases the SMC(15) was found in 100% of metaphases and in one (6.25%) it was mosaic. In 14/16 (87.5%) cases the SMC(15) was de novo while in the remaining two (12.5%) it was familial. Small SMC(15)s were found in 9/16 cases. Seven of 16 cases with large SMC(15)s were studied for the PWS/AS critical region with the SNRPN/15qtel probe. In two which contained the critical region,
subsequent molecular study confirmed the presence of 3 alleles of the PWS/AS critical region and showed that SMC(15) was maternally-derived. The study of the methylation pattern in these patients, however, excluded the diagnosis of PWS/AS.
Conclusions: This study illustrates the significance of genetic investigation of SMC(15)s for genotypephenotype correlation and genetic counseling.
Key words: Supernumerary marker chromosome, chromosome 15.
™˘ÓÙÔÌÔÁڷʛ˜
SMCsÀÂÚ¿ÚÈıÌ· ¯ÚˆÌÔÛˆÌÈο ıÚ·‡ÛÌ·Ù· FISHºıÔÚ›˙ˆÓ in situ ˘‚ÚȉÈÛÌfi˜ PWS/AS™‡Ó‰ÚÔÌ· Prader-Willi/Angelman UPDMÔÓÔÁÔÓÂ˚΋ ‰Èۈ̛· WCPAÓȯÓÂ˘Ù‹˜ ÁÈ· ÔÏfiÎÏËÚÔ ÙÔ ¯ÚˆÌfiۈ̷ Ì ÙË Ì¤ıÔ‰Ô ‚·Ê‹˜ D¡RP¶ÔÏ˘ÌÔÚÊÈÎo› ‰ÈÓÔ˘ÎÏÂÔÙȉÈÎÔ› ‰Â›ÎÙ˜ MSPCRT¯ÓÈ΋ ·Ï˘ÛȉˆÙ‹˜ ·ÓÙ›‰Ú·Û˘ ÔÏ˘ÌÂÚ¿Û˘ ÁÈ· ÙÔÓ ¤ÏÂÁ¯Ô ÙÔ˘ ÚÔÙ‡Ô˘
ÌÂı˘Ï›ˆÛ˘
EÈÛ·ÁˆÁ‹
∆· ˘ÂÚ¿ÚÈıÌ· ¯ÚˆÌÔÛˆÌÈο ıÚ·‡ÛÌ·Ù· (supernumerary marker chromosomes - SMCs)
·ÔÙÂÏÔ‡Ó ÂÙÂÚÔÁÂÓ‹ ÔÌ¿‰· ·ÚÈıÌËÙÈÎÒÓ
¯ÚˆÌÔÛˆÌÈÎÒÓ ·ÓˆÌ·ÏÈÒÓ Ô˘ ¯·Ú·ÎÙËÚ›˙ÔÓÙ·È ·fi ÌÂÚÈ΋ ÙÚÈۈ̛· ÔÚÈÛÌ¤ÓˆÓ ÂÚÈÔ¯ÒÓ ÙÔ˘ ÁÔÓȉÈÒÌ·ÙÔ˜ (1). ∏ Û˘¯ÓfiÙËÙ¿ ÙÔ˘˜
Î˘Ì·›ÓÂÙ·È ·fi 0,14 ¤ˆ˜ 0,72 ·Ó¿ 1000 ÁÂÓÓ‹ÛÂȘ, ÂÓÒ ÛÙÔÓ ÚÔÁÂÓÓËÙÈÎfi ¤ÏÂÁ¯Ô Â›Ó·È 0,06-0,1%, ÔÛÔÛÙfi Ô˘ ·ÓÙÈÛÙÔȯ› ÛÙÔ 5% ÙˆÓ ÂÌ‚Ú‡ˆÓ Ô˘ ÂϤÁ¯ÔÓÙ·È (1,2).
∏ ÎÏ·ÛÈ΋ ΢ÙÙ·ÚÔÁÂÓÂÙÈ΋ Ô˘ ÛÙËÚ›˙ÂÙ·È ÛÙËÓ ·Ó¿Ï˘ÛË Ù·ÈÓÈÒÓ ÛÙ· ¯ÚˆÌÔÛÒÌ·Ù· ‰ÂÓ Â·ÚΛ ÁÈ· ÙËÓ Ù·˘ÙÔÔ›ËÛË Ù‹˜ ¯ÚˆÌÔ-
ÛˆÌÈ΋˜ ÚÔ¤Ï¢Û˘ ÙˆÓ SMCs, ÂÂȉ‹ Û˘Ó‹ıˆ˜ Â›Ó·È Ôχ ÌÈÎÚÔ‡ ÌÂÁ¤ıÔ˘˜ Î·È ‰ÂÓ ¤¯Ô˘Ó ‰È·ÁÓˆÛÙÈΤ˜ Ù·Èӛ˜. ∏ ·Ó¿Ù˘ÍË Ù˘ Ù¯ÓÈ΋˜ ÙÔ˘ ÊıÔÚ›˙ÔÓÙÔ˜ in situ ˘‚ÚȉÈÛÌÔ‡ (FISH) ¤‰ˆÛ Ӥ˜ ‰˘Ó·ÙfiÙËÙ˜ ÛÙËÓ ÂÚÁ·ÛÙËÚȷ΋ ‰ÈÂÚ‡ÓËÛË ÙˆÓ SMCs Î·È ÛÙË Û˘Û¯¤ÙÈÛ‹ ÙÔ˘˜ Ì ¤Ó·Ó Û˘ÁÎÂÎÚÈ̤ÓÔ ÎÏÈÓÈÎfi Ê·ÈÓfiÙ˘Ô (2,3).
√È Ê·ÈÓÔÙ˘ÈΤ˜ ÂÈÙÒÛÂȘ ·fi ÙËÓ ·-
ÚÔ˘Û›· ÙˆÓ SMCs Â›Ó·È ÔÈΛϘ Î·È ÂÍ·ÚÙÒÓÙ·È Î˘Ú›ˆ˜ ·fi ÙË ¯ÚˆÌÔÛˆÌÈ΋ ÙÔ˘˜ ÚԤϢÛË, ÙÔ Ì¤ÁÂıfi˜ ÙÔ˘˜ Î·È ÙËÓ ·ÚÔ˘Û›· ÙÔ˘˜
ÁÈ· ÙÔ ÙÔ 70% ÙˆÓ ÌÈÎÚÒÓ SMCs Ô˘ ÚÔ¤Ú¯ÔÓÙ·È ·fi ÌË ·ÎÚÔÎÂÓÙÚÈο ¯ÚˆÌÔÛÒÌ·Ù·. ªÂ-
Á·Ï‡ÙÂÚ· SMCs, fï˜, ·Ó¿ÏÔÁ· Ì ÙË ¯ÚˆÌÔÛˆÌÈ΋ ÙÔ˘˜ ÚԤϢÛË, ÌÔÚ› Ó· ÚÔηϤÛÔ˘Ó ÛÔ‚·ÚÔ‡ ‚·ıÌÔ‡ ÓÔËÙÈ΋ ˘ÛÙ¤ÚËÛË Î·È
Û˘ÁÁÂÓ›˜ ·ÓˆÌ·Ï›Â˜ (5,6). ™Â ÂÚÈÙÒÛÂȘ Ô˘ ÙÔ SMC ·ÓȯÓ‡ÂÙ·È ÛÙÔÓ ÚÔÁÂÓÓËÙÈÎfi
·fi ÙÔ˘˜ ÁÔÓ›˜, Ô Î›Ó‰˘ÓÔ˜ ·ıÔÏÔÁÈÎÔ‡ Ê·ÈÓÔÙ‡Ô˘ ›-
Ì ÙÔÓ ÁÔÓ¤·. ∂¿Ó, fï˜, ÙÔ ¯ÚˆÌÔÛˆÌÈÎfi ıÚ·‡ÛÌ· Â›Ó·È de novo, ÙfiÙÂ Ô Î›Ó‰˘ÓÔ˜ Ó· Û˘Óԉ‡ÂÙ·È ·fi ·ıÔÏÔÁÈÎfi Ê·ÈÓfiÙ˘Ô ˘ÔÏÔÁ›˙ÂÙ·È Û 13%, fiˆ˜ ÚÔ·ÙÂÈ ·fi ÌÂϤÙË 377.000 ·ÌÓÈÔ·Ú·ÎÂÓÙ‹ÛÂˆÓ (7).
¶ÔÛÔÛÙfi 50% ÙˆÓ ˘ÂÚ¿ÚÈıÌˆÓ ¯ÚˆÌÔÛˆÌÈÎÒÓ ıÚ·˘ÛÌ¿ÙˆÓ ·ÔÙÂÏÔ‡Ó ÙÌ‹Ì·Ù·
ÙÔ˘ ¯ÚˆÌÔÛÒÌ·ÙÔ˜ 15 Î·È ÔÈ Ê·ÈÓÔÙ˘ÈΤ˜
ÙÔ˘˜ ÂÈÙÒÛÂȘ ÂÍ·ÚÙÒÓÙ·È Î˘Ú›ˆ˜ ·fi ÙËÓ
‡·ÚÍË ‹ fi¯È Ù˘ ÎÚ›ÛÈÌ˘ ÂÚÈÔ¯‹˜ ÙˆÓ Û˘Ó‰ÚfiÌˆÓ Prader-Willi/Angelman (PWS/AS) (2). ªÈÎÚ¿ SMC(15), de novo ‹ ÔÈÎÔÁÂÓ‹, Ù· ÔÔ›·
‰ÂÓ ÂÚȤ¯Ô˘Ó ¢¯ÚˆÌ·Ù›ÓË Ô‡Ù ÙËÓ ÎÚ›ÛÈÌË
ÂÚÈÔ¯‹ PWS/AS, ‰ÂÓ Û¯ÂÙ›˙ÔÓÙ·È ¿ÌÂÛ· ÌÂ
·ıÔÏÔÁÈÎfi Ê·ÈÓfiÙ˘Ô (6). ªÂÁ·Ï‡ÙÂÚ·
SMC(15) Ô˘ ÂÚȤ¯Ô˘Ó ÙËÓ ÎÚ›ÛÈÌË ÂÚÈÔ¯‹ PWS/AS, Â›Ó·È Û¯Â‰fiÓ ¿ÓÙ· de novo, ÌËÙÚÈ΋˜ ÚÔ¤Ï¢Û˘ Î·È Û˘Óԉ‡ÔÓÙ·È ·fi ÛÔ‚·ÚÔ‡ ‚·ıÌÔ‡ ÓÔËÙÈ΋ ˘ÛÙ¤ÚËÛË Î·È Û˘ÁÁÂÓ›˜ ·ÓˆÌ·Ï›Â˜ (6).
¶·Ú¿ ÙÔ ÁÂÁÔÓfi˜ fiÙÈ ÂÏÏ›ÌÌ·Ù· ÛÙËÓ ÂÚÈÔ¯‹ q11-q13 ÙÔ˘ ¯ÚˆÌÔÛÒÌ·ÙÔ˜ 15 ·ÔÙÂÏÔ‡Ó ÙËÓ ÈÔ Û˘¯Ó‹ ·ÈÙ›· ÂΉ‹ÏˆÛ˘ Û˘Ó‰ÚfiÌˆÓ PWS (70%) Î·È AS (60%), ÔÛÔÛÙfi 2530% ÙˆÓ ·ÛıÂÓÒÓ Ì PWS Î·È 3-5% Ì AS ÂÌÊ·Ó›˙Ô˘Ó ÌËÙÚÈ΋ ‹ ·ÙÚÈ΋, ·ÓÙ›ÛÙÔȯ·, ÌÔÓÔÁÔÓÂ˚΋ ‰Èۈ̛· (UPD) Î·È Ôχ ÌÈÎÚfiÙÂÚ· ÔÛÔÛÙ¿ ÙˆÓ Û˘Ó‰ÚfiÌˆÓ PWS/AS (1-5%)
ÔÊ›ÏÔÓÙ·È Û ÌÂÙ·ÏÏ¿ÍÂȘ ÛÙÔ Î¤ÓÙÚÔ ÁÔÓȉȷ΋˜ ·ÔÙ‡ˆÛ˘. ™Â fiϘ ·˘Ù¤˜ ÙȘ ÂÚÈÙÒ-
ÛÂȘ, Ë Ù¯ÓÈ΋ MSPCR Ô˘ ÌÂÏÂÙ¿ ÙÔ ÚfiÙ˘-
Ô ÌÂı˘Ï›ˆÛ˘ ÙÔ˘ ÂÍˆÓ›Ô˘ 1 ÙÔ˘ ÁÔÓȉ›Ô˘
›‰ÈÔ ÈÛ¯‡ÂÈ Î·È
SNRPN ÛÙËÓ ÎÚ›ÛÈÌË ÁÈ· ÙÔ PW/AS ÂÚÈÔ¯‹,
¶·È‰È·ÙÚÈ΋ 2006;69:294-300
̤۷ Û ̛· ÓËÛ›‰· CpG, Ë ÔÔ›· - ˆ˜ ·ÔÙ¤-
ÏÂÛÌ· ·ÏÏËÏÂȉڿÛÂˆÓ ÌÂٷ͇ ÙˆÓ Î¤ÓÙÚˆÓ
ÁÔÓȉȷ΋˜ ·ÔÙ‡ˆÛ˘ ÙÔ˘ ÌËÙÚÈÎÔ‡ Î·È ·-
ÙÚÈÎÔ‡ ·ÏÏËÏÔÌfiÚÊÔ˘ - Ê·›ÓÂÙ·È Ó· Â›Ó·È ÌÂ-
ı˘ÏȈ̤ÓË ÛÙÔ ÌËÙÚÈÎfi Î·È ÌË ÌÂı˘ÏȈ̤ÓË
ÛÙÔ ·ÙÚÈÎfi. ø˜ ÌË ÌÂı˘ÏȈ̤ÓË Ë ÂÚÈÔ¯‹
·˘Ù‹ ÛÙÔ ·ÙÚÈÎfi ·ÏÏËÏfiÌÔÚÊÔ ‰È·ÙËÚ›ٷÈ
ÂÓÂÚÁ‹ Î·È ÈÛÙ‡ÂÙ·È fiÙÈ Ú˘ıÌ›˙ÂÈ ÙËÓ ¤ÎÊÚ·-
ÛË fiÏˆÓ Û¯Â‰fiÓ ÙˆÓ ÁÔÓȉ›ˆÓ Ù˘ ÎÚ›ÛÈÌ˘ ÂÚÈÔ¯‹˜. ™Â ÂÚ›ÙˆÛË ·ÚÔ˘Û›·˜ ¯ÚˆÌÔÛˆÌÈÎÔ‡ ıÚ·‡ÛÌ·ÙÔ˜ Ô˘ ÂÚÈÏ·Ì‚¿ÓÂÈ ÙËÓ ÂÚÈÔ¯‹ 15q11-q13, ˘¿Ú¯ÂÈ Èı·ÓfiÙËÙ·
Ô›ËÛ˘ ÙÔ˘ Ê˘ÛÈÔÏÔÁÈÎÔ‡ ÚÔÙ‡Ô˘
Ì˯·ÓÈÛÌÔ‡
PWS,
ÙÒÛÂȘ Â›Ó·È ·ÔÙ¤ÏÂÛÌ· ÌÔÓÔÁÔÓÂ˚΋˜ ‰Èۈ̛·˜ (6-11). ™ÙËÓ ·ÚÔ‡Û· ÌÂϤÙË
Â˘Ú‹Ì·Ù· Ù˘ ÁÂÓÂÙÈ΋˜ ·Ó¿Ï˘Û˘ 16 ÂÚÈÙÒÛÂˆÓ SMCs Ô˘ ÚÔ¤Ú¯ÔÓÙ·È
Ìfiۈ̷ 15 Î·È Á›ÓÂÙ·È ÚÔÛ¿ıÂÈ· Û‡Ó‰ÂÛ˘ ÙÔ˘ ÁÔÓÔÙ‡Ô˘ Ì ÙÔÓ Ê·ÈÓfiÙ˘Ô.
ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ
™ÙËÓ ·ÚÔ‡Û· ÌÂϤÙË ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È 14 ·È‰È¿
ËÏÈΛ·˜ 4 ÌËÓÒÓ ¤ˆ˜ 10 ÂÙÒÓ Î·È 2 ‰Â›ÁÌ·Ù· ·ÌÓÈ·ÎÔ‡ ˘ÁÚÔ‡ ·fi ÙÔ ‰Â‡ÙÂÚÔ ÙÚ›ÌËÓÔ Ù˘ ·ËÛ˘, ÛÙÔÓ Î·Ú˘fiÙ˘Ô ÙˆÓ ÔÔ›ˆÓ ‰È·ÈÛÙÒıËΠ˘ÂÚ¿ÚÈıÌÔ ¯Úˆ-
ÌÔÛˆÌÈÎfi ıÚ·‡ÛÌ·. √È Ì¤ıÔ‰ÔÈ Ô˘ ¯ÚËÛÈÌÔÔÈ‹ıËÎ·Ó ÁÈ· ÙË ÌÂϤÙË
ÂÚÈÂÏ¿Ì‚·Ó·Ó: ·) ∫˘ÙÙ·ÚÔÁÂÓÂÙÈ΋ ·Ó¿Ï˘ÛË: ∏ ÌÂϤÙË ÙÔ˘ ηڢÔÙ‡Ô˘ ¤ÁÈÓ Û ÌÂÙ·Ê·ÛÈο ¯ÚˆÌÔÛÒÌ·Ù· ÌÂÙ¿ ·fi ηÏÏȤÚÁÂÈ· ÏÂÌÊÔ΢ÙÙ¿ÚˆÓ ÂÚÈÊÂÚÈÎÔ‡ ·›Ì·ÙÔ˜ ‹ ΢ÙÙ¿ÚˆÓ ·ÌÓÈ·ÎÔ‡ ˘ÁÚÔ‡ Î·È Û‹Ì·ÓÛË Ì ٷÈӛ˜ G (12). ™Â fiϘ ÙȘ ÂÚÈÙÒÛÂȘ ÌÂÏÂÙ‹ıËÎÂ Ô Î·Ú˘fiÙ˘Ô˜ ÙˆÓ ÁÔÓ¤ˆÓ. ‚) ºıÔÚ›˙ˆÓ in situ ˘‚ÚȉÈÛÌfi˜ (FISH): ∏ Ù·˘ÙÔÔ›ËÛË Ù˘ ¯ÚˆÌÔÛˆÌÈ΋˜ ÚÔ¤Ï¢Û˘ ÙÔ˘ ˘ÂÚ¿ÚÈıÌÔ˘ ¯ÚˆÌÔÛˆÌÈÎÔ‡ ıÚ·‡ÛÌ·ÙÔ˜
FISH Û ÌÂÙ·Ê·ÛÈο ¯ÚˆÌÔÛÒÌ·Ù·. ™ÙË ÌÂϤÙË ¯ÚËÛÈÌÔÔÈ‹ıËηÓ: i) Ô ·ÓȯÓÂ˘Ù‹˜ ÁÈ· ÔÏfiÎÏËÚÔ ÙÔ
Ìfiۈ̷ 15 Ì ÙË Ì¤ıÔ‰Ô ‚·Ê‹˜ ÙˆÓ ¯ÚˆÌÔÛˆÌ¿ÙˆÓ (whole chromosome paints - WCP) (Cambio) Î·È ii) Ô ·ÓȯÓÂ˘Ù‹˜ SNRPN/15qtel, Ô˘ ÂÚÈÏ·Ì‚¿ÓÂÈ ÙÔ ÁÔÓ›‰ÈÔ SNRPN ÛÙËÓ Ù·ÈÓ›· 15q11-q13 Î·È ÙÔÓ ÙÂÏÔÌÂÚȉȷÎfi ‰Â›ÎÙË ÙˆÓ Ì·ÎÚÒÓ ÛÎÂÏÒÓ ÙÔ˘ ¯ÚˆÌÔÛÒÌ·ÙÔ˜ 15 (15qtel). Á) ªÔÚÈ·Îfi˜
DNA ·fi Ù· ·È‰È¿ Î·È ÙÔ˘˜ ÁÔÓ›˜ ÙÔ˘˜ ¤ÁÈÓ Ì ÙÔ ÂÌÔÚÈο ‰È·ı¤ÛÈÌÔ Qiamp Blood Mini Kit (Qiagen). ∏ Ù·˘ÙÔÔ›ËÛË Ù˘ ÁÔÓÂ˚΋˜ ÚÔ¤Ï¢Û˘ ÙÔ˘ SMC Î·È Ë ‰ÈÂÚ‡ÓËÛË Èı·Ó‹˜ UPD ¤ÁÈÓ Ì ·Ó¿Ï˘ÛË 8 D¡RPs, ¤ÍÈ (D15S543, D15S11, D15S10, D15S113, GABRB3, D15S97) ·fi ÙËÓ ÎÚ›ÛÈÌË ÂÚÈÔ¯‹ PWS/AS Î·È ‰‡Ô (CYP19, D15S87) ·fi ÙËÓ ÙÂÏÔÌÂÚȉȷ΋ ÂÚÈÔ¯‹ ÙÔ˘ ¯ÚˆÌÔÛÒÌ·ÙÔ˜ 15 Î·È Ù· ·ÔÙÂϤÛÌ·Ù· ·Ó·Ï‡ıËÎ·Ó ÛÙÔÓ ·˘ÙfiÌ·ÙÔ ·Ó·Ï˘Ù‹ Open GeneTM System Ù˘Visible Genetics. √ ¤ÏÂÁ¯Ô˜ ÙÔ˘ ÚÔÙ‡Ô˘ ÌÂı˘Ï›ˆÛ˘ Ù˘ ÎÚ›ÛÈÌ˘ ÂÚÈÔ¯‹˜ ÙÔ˘ PWS/AS ¤ÁÈÓ Ì ÌÂϤÙË ÙÔ˘ ÂÍˆÓ›Ô˘ 1 ÛÙÔ ÁÔÓ›‰ÈÔ SNRPN Ì ÙËÓ ÂȉÈ΋ Ù¯ÓÈ΋ ·Ï˘ÛȉˆÙ‹˜ ·ÓÙ›‰Ú·Û˘ ÔÏ˘ÌÂÚ¿Û˘ (MSPCR) ÌÂÙ¿ ·fi ¯ËÌÈ΋ ÙÚÔÔÔ›ËÛË ÙÔ˘ DNA Ì ÙÔ ÂÌÔÚÈο ‰È·ı¤ÛÈÌÔ CpG GenomeTM Modification Kit (Q-BIOgene).
∞ÔÙÂϤÛÌ·Ù·
™Â fiϘ ÙȘ ÂÚÈÙÒÛÂȘ, ÙÔ SMC ÂÓÙÔ›ÛÙËΠ·Ú¯Èο Ì ÎÏ·ÛÈ΋ ΢ÙÙ·ÚÔÁÂÓÂÙÈ΋ ·Ó¿Ï˘ÛË (¶›Ó·Î·˜ 1, ∂ÈÎfiÓ· 1). ™Â 15 ·fi ÙȘ 16 ÂÚÈÙÒÛÂȘ (93,75%) ÙÔ SMC ˘‹Ú¯Â ÛÙÔ 100% ÙˆÓ ÌÂÙ·Ê¿ÛÂˆÓ Ô˘ ÌÂÏÂÙ‹ıËÎ·Ó Î·È ÌfiÓÔ ÛÙÔÓ ·ÛıÂÓ‹ ¡Ô 1 ‹Ù·Ó Û ̈۷˚Îfi, Û ÔÛÔÛÙfi 45%. √ ηڢfiÙ˘Ô˜ ÙˆÓ ÁÔÓ¤ˆÓ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈÎfi˜ Û 14/16 ÂÚÈÙÒÛÂȘ (87,5%). ™ÙÔÓ ·Ù¤Ú· ÙÔ˘ ·ÛıÂÓ‹ ¡Ô 1 ·ÓȯÓ‡ÙËΠ۠ÔÛÔÛÙfi 6% ÙÔ ›‰ÈÔ SMC Ô˘ ‚Ú¤ıËΠÛÙÔ ·È‰› Û 45% ÙˆÓ ÌÂÙ·Ê¿ÛÂˆÓ (ηڢfiÙ˘Ô˜ ·Ù¤Ú· ·ÛıÂÓ‹ ¡Ô 1: 46,XY[94]/47,XY+marker[6]).
∂›Û˘, ÛÙË ÌËÙ¤Ú· ÙÔ˘ ÂÌ‚Ú‡Ô˘ ¡Ô 16 ÙÔ SMC ˘‹Ú¯Â Û fiϘ ÙȘ ÌÂÙ·Ê¿ÛÂȘ Ô˘ ·Ó·Ï‡ıËÎ·Ó (47,ÃÃ+marker[100]).
ªÂÙ¿ ÙË ÌÂϤÙË Ì ÙË Ì¤ıÔ‰Ô ‚·Ê‹˜ ÔÏfiÎÏËÚˆÓ ÙˆÓ ¯ÚˆÌÔۈ̿وÓ, ¯ÚËÛÈÌÔÔÈÒÓÙ·˜ DNA ·ÓȯÓÂ˘Ù‹ ÁÈ· ÙÔ ¯ÚˆÌfiۈ̷ 15, ‰È·ÈÛÙÒıËΠfiÙÈ ÙÔ SMC Û fiϘ ÙȘ ÂÚÈÙÒÛÂȘ ‹Ù·Ó ÙÌ‹Ì· ÙÔ˘ ¯ÚˆÌÔÛÒÌ·ÙÔ˜ 15 (∂ÈÎfiÓ· 2).
∂ÈÎfiÓ· 1. ªÂÙ·Ê¿ÛÂȘ ·fi ÙÔ˘˜ ·ÛıÂÓ›˜ ¡Ô 1 (·ÚÈÛÙÂÚ¿) Ì ÌÈÎÚÔ‡ ÌÂÁ¤ıÔ˘˜ SMC(15) Î·È ¡Ô 14 (‰ÂÍÈ¿) Ì ÌÂÁ¿ÏÔ˘ ÌÂÁ¤ıÔ˘˜ SMC(15) (Ù·Èӛ˜ G).
¡Ô∫˘ÙÙ·ÚÔÁÂÓÂÙÈ΋ ·Ó¿Ï˘ÛËFISH
1 46,XX[55]/47,XX+mar[45] ÔÈÎÔÁÂÓ¤˜*ish der (15)(wcp15+)
2 47,XX+mar[100] de novo ish der (15)(wcp15+)
3 47,XY+mar[100] de novo ish der (15)(wcp15+)
4 47,XÀ+mar[100] de novo ish der (15)(wcp15+)
5 47,XÀ+mar[100] de novo ish der (15)(wcp15+)
6 47,XX+mar[100] de novo ish der (15)(wcp15+)
7 47,XX+mar[100] de novo ish der (15)(wcp15+)
8 47,XY+mar[100] de novo ish der (15)(wcp15+)
9 47,XX+mar[100] de novo ish der (15)(wcp15+) 1047,XX+mar[100] de novo ish der(15)(wcp15+),ish(15q11.2q13) (SNRPNx3,15qtelx2) 1147,XX+mar[100] de novo ish der(15)(wcp15+),ish(15q11.2q13) (SNRPNx2,15qtelx2)
1247,XX+mar[100] de novo ish der(15)(wcp15+),ish(15q11.2q13) (SNRPNx2,15qtelx2)
1347,XX+mar[100] de novo ish der(15)(wcp15+),ish(15q11.2q13) (SNRPNx2,15qtelx2) 1447,XX+mar[100] de novo ish der(15)(wcp15+),ish(15q11.2q13) (SNRPNx3,15qtelx2) 1547,XY+mar[100] de novo ish der(15)(wcp15+),ish(15q11.2q13) (SNRPNx2,15qtelx2) 1647,XX+mar[100] ÔÈÎÔÁÂÓ¤˜**ish der(15)(wcp15+),ish(15q11.2q13) (SNRPNx2,15qtelx2)
* ∫·Ú˘fiÙ˘Ô˜ ·Ù¤Ú·: 46,XY[94]/47,XY+marker[6]
** K·Ú˘fiÙ˘Ô˜ ÌËÙ¤Ú·˜: 47,XX+marker[100]
™ÙȘ ÂÚÈÙÒÛÂȘ ¡Ô 10 ¤ˆ˜ ¡Ô 16 Ì ÌÂ-
Á¿ÏÔ˘ ÌÂÁ¤ıÔ˘˜ SMC(15), ÌÂϤÙË Ì ÙÔÓ ·ÓÈ-
¯ÓÂ˘Ù‹ SNRPN/15qtel ÁÈ· ¤ÏÂÁ¯Ô ‡·Ú͢ Ù˘
ÎÚ›ÛÈÌ˘ ÂÚÈÔ¯‹˜ PWS/AS, ·ÔÎ¿Ï˘„ 3 Û‹Ì·Ù· ˘‚ÚȉÈÛÌÔ‡ ÁÈ· ÙÔ ÁÔÓ›‰ÈÔ SNRPN ÛÙÔ˘˜
·ÛıÂÓ›˜ ¡Ô 10 Î·È ¡Ô 14. ™Â η̛· ·fi ÙȘ
ÂÚÈÙÒÛÂȘ Ô˘ ÌÂÏÂÙ‹ıËηÓ, ‰ÂÓ ·ÓȯÓ‡ÙËÎ·Ó ÛÙÔ SMC(15) Û‹Ì·Ù· ˘‚ÚȉÈÛÌÔ‡ ÁÈ· ÙÔ
ÙÂÏÔÌÂÚ›‰ÈÔ ÙÔ˘ ¯ÚˆÌÔÛÒÌ·ÙÔ˜ 15 (15qtel)
(EÈÎfiÓ· 3). ™ÙÔ˘˜ ·ÛıÂÓ›˜ ¡Ô 10 Î·È ¡Ô 14, Ë ·Ó¿Ï˘ÛË DNRPs ÂȂ‚·›ˆÛ ÙËÓ ·ÚÔ˘Û›·
ÙÚÈÒÓ ·ÏÏËÏfiÌÔÚÊˆÓ Î·È ¤‰ÂÈÍ fiÙÈ ÙÔ SMC ‹Ù·Ó ÌËÙÚÈ΋˜ ÚÔ¤Ï¢Û˘ (∂ÈÎfiÓ· 4). ŒÏÂÁ¯Ô˜ ÙÔ˘ ÚÔÙ‡Ô˘ ÌÂı˘Ï›ˆÛ˘ ÛÙÔ˘˜ ‰‡Ô ·˘ÙÔ‡˜ ·ÛıÂÓ›˜ Ì ªSPCR ¤‰ÂÈÍÂ Ê˘ÛÈÔÏÔÁÈÎfi ÚfiÙ˘Ô ÌÂı˘Ï›ˆÛ˘, ·ÔÎÏ›ÔÓÙ·˜ ÙËÓ Èı·ÓfiÙËÙ· Û˘Ó‰ÚfiÌÔ˘ PWS Î·È ÛÙ· ‰‡Ô ·È‰È¿ (∂ÈÎfiÓ· 5).
∂ÈÎfiÓ· 2. ªÂÙ¿Ê·ÛË ·fi ÙËÓ ·ÛıÂÓ‹ ¡Ô 10 ÌÂÙ¿ ·fi ÌÂϤÙË Ì FISH (̤ıÔ‰Ô˜ ‚·Ê‹˜ ÔÏfiÎÏËÚÔ˘ ÙÔ˘ ¯ÚˆÌÔÛÒÌ·ÙÔ˜ 15). ∆Ô SMC(15) Î·È Ù· ‰‡Ô Ê˘ÛÈÔÏÔÁÈο ¯ÚˆÌÔÛÒÌ·Ù· 15 ¤¯Ô˘Ó ‚·Ê› Ì ÙÔÓ ·ÓȯÓÂ˘Ù‹ (Ú¿ÛÈÓÔ ¯ÚÒÌ·). ∂ÈÎfiÓ· 3. ªÂÙ¿Ê·ÛË ·fi ÙËÓ ·ÛıÂÓ‹ ¡Ô 14 ÌÂÙ¿ ·fi ÌÂϤÙË Ì FISH Ì ÙÔ˘˜ ·ÓȯÓÂ˘Ù¤˜ SNRPN (ÎfiÎÎÈÓÔ Û‹Ì· ) Î·È 15qtel (Ú¿ÛÈÓÔ Û‹Ì·).
∂ÈÎfiÓ· 4. ∞ÔÙÂϤÛÌ·Ù· ·Ó¿Ï˘Û˘ DNRP Ù˘ ÔÏ˘ÌÔÚÊÈ΋˜ ı¤Û˘ D15S97 ÛÙËÓ ·ÛıÂÓ‹ ¡Ô 10. ¶·Ú·ÙËÚÂ›Ù·È Ë ·ÚÔ˘Û›· ÙÚÈÒÓ ·ÏÏËÏfiÌÔÚÊˆÓ (‰‡Ô ÌËÙÚÈ΋˜ Î·È ¤Ó· ·ÙÚÈ΋˜ ÚÔ¤Ï¢Û˘) ÛÙÔ ·È‰› (F: ·Ù¤Ú·˜, C: ·È‰›, M: ÌËÙ¤Ú·).
∂ÈÎfiÓ· 5. ∞ÔÙÂϤÛÌ·Ù· Ù˘ ÌÂϤÙ˘ Ì MSPCR. C3: º˘ÛÈÔÏÔÁÈÎfi˜ Ì¿ÚÙ˘Ú·˜: ·ÚÔ˘Û›· ‰‡Ô ÚÔ˚fiÓÙˆÓ, ÂÓfi˜ ·fi ÙÔ ÌËÙÚÈ΋˜ ÚÔ¤Ï¢Û˘ ·ÏÏËÏfiÌÔÚÊÔ (ÌÂÁ¤ıÔ˘˜ 174bp) Î·È ÂÓfi˜ ·fi ÙÔ ·ÙÚÈ΋˜ ÚÔ¤Ï¢Û˘ ·ÏÏËÏfiÌÔÚÊÔ (ÌÂÁ¤ıÔ˘˜ 100bp) (Ê˘ÛÈÔÏÔÁÈÎfi ÚfiÙ˘Ô ÌÂı˘Ï›ˆÛ˘), C1: M¿ÚÙ˘Ú·˜ Ì PWS: ·ÚÔ˘Û›· ÌfiÓÔ ÚÔ˚fiÓÙÔ˜ ·fi ÙÔ ÌËÙÚÈÎfi ·ÏÏËÏfiÌÔÚÊÔ (ÌÂÁ¤ıÔ˘˜ 174bp), C2: M¿ÚÙ˘Ú·˜ Ì AS: ·ÚÔ˘Û›· ÌfiÓÔ ÚÔ˚fiÓÙÔ˜ ·fi ÙÔ ·ÙÚÈÎfi ·ÏÏËÏfiÌÔÚÊÔ (ÌÂÁ¤ıÔ˘˜ 100bp), 5 Î·È 6: ∞ÛıÂÓ‹˜ ¡Ô 10 (Ê˘ÛÈÔÏÔÁÈÎfi ÚfiÙ˘Ô ÌÂı˘Ï›ˆÛ˘), 7 Î·È 8: ∞ÛıÂÓ‹˜ ¡Ô 14 (Ê˘ÛÈÔÏÔÁÈÎfi ÚfiÙ˘Ô ÌÂı˘Ï›ˆÛ˘).
΋ ÌÂϤÙË 16 ÂÚÈÙÒÛÂˆÓ SMCs Ô˘ ‰È·ÁÓÒÛÙËÎ·Ó Ì ÎÏ·ÛÈ΋ ΢ÙÙ·ÚÔÁÂÓÂÙÈ΋ ·Ó¿Ï˘ÛË
ÛÙÔ ∂ÚÁ·ÛÙ‹ÚÈÔ π·ÙÚÈ΋˜ °ÂÓÂÙÈ΋˜ ÙÔ˘ ¶·ÓÂ-
ÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ Î·Ù¿ ÙËÓ ÂÓÙ·ÂÙ›· 2000-
2005. ™Â fiϘ ÙȘ ÂÚÈÙÒÛÂȘ, Ë Ù·˘ÙÔÔ›ËÛË
Ù˘ ¯ÚˆÌÔÛˆÌÈ΋˜ ÙÔ˘˜ ÚÔ¤Ï¢Û˘ ¤ÁÈÓ ÌÂ
ÙËÓ Ù¯ÓÈ΋ FISH. ∂›Ó·È ÂӉȷʤÚÔÓ ÙÔ ÁÂÁÔÓfi˜
fiÙÈ Û 10 ·fi ÙȘ 16 ÂÚÈÙÒÛÂȘ Ù˘ ÌÂϤÙ˘, Ë
ËÏÈΛ· Ù˘ ÌËÙ¤Ú·˜ ‹Ù·Ó ÌÂÁ·Ï‡ÙÂÚË ÙˆÓ 35
ÂÙÒÓ, ÂÔ̤ӈ˜ Â›Ó·È Èı·Ófi fiÙÈ Ë ÂÌÊ¿ÓÈÛË
ÙˆÓ SMC(15)s ÔÊ›ÏÂÙ·È Û ·ÙÂÏ‹ ‰È·¯ˆÚÈÛÌfi
ÙˆÓ ¯ÚˆÌÔÛˆÌ¿ÙˆÓ ÛÙË Ì›ˆÛË π, Ô˘ ¤¯ÂÈ Û˘Ó-
‰Âı› Ì ·˘ÍË̤ÓË ËÏÈΛ· Ù˘ ÌËÙ¤Ú·˜ (3,13).
¢Âη٤ÛÛÂÚ· SMC(15)s (¡Ô 2 ¤ˆ˜ ¡Ô 15)
‹Ù·Ó de novo, ÂÓÒ Û ‰‡Ô ÂÚÈÙÒÛÂȘ ‹Ù·Ó ÔÈÎÔÁÂÓ‹. ™˘ÁÎÂÎÚÈ̤ӷ, fiÛÔÓ ·ÊÔÚ¿ ÛÙËÓ
·ÛıÂÓ‹ ¡Ô 1, Ë ÔÔ›· ·Ú·¤ÌÊıËΠÏfiÁˆ ÌÈ-
ÎÚÔ‡ ‚·ıÌÔ‡ ÓÔËÙÈ΋˜ ˘ÛÙ¤ÚËÛ˘ Î·È ‰È·Ù·Ú·¯ÒÓ ÙÔ˘ ÏfiÁÔ˘, ÙÔ SMC(15) ‰È·ÈÛÙÒıËΠ۠ÌÈÎÚfi ÔÛÔÛÙfi (6%) Î·È ÛÙÔÓ Ê·ÈÓÔÙ˘Èο
Ê˘ÛÈÔÏÔÁÈÎfi ·Ù¤Ú· Ù˘. ∂Ô̤ӈ˜, Ô ‹ÈÔ˜ ·ıÔÏÔÁÈÎfi˜ Ê·ÈÓfiÙ˘Ô˜ Ù˘ ·ÛıÂÓÔ‡˜ Èı·ÓÒ˜ Ó· ÔÊ›ÏÂÙ·È ÛÙÔ ÌÂÁ·Ï‡ÙÂÚÔ
ÙÔ˘ SMC(15) (45%) Û ۯ¤ÛË Ì ÙÔÓ Ê˘ÛÈÔÏÔÁÈο Ê·ÈÓÔÙ˘ÈÎfi ·Ù¤Ú· Ù˘. ™ÙÔ ¤Ì‚Ú˘Ô ¡Ô
16 Ì SMC(15) ÛÙÔ 100% ÙˆÓ ÌÂÙ·Ê¿ÛÂˆÓ Ô˘ ÌÂÏÂÙ‹ıËηÓ, ·fi ÙËÓ ·Ó¿Ï˘ÛË ÙÔ˘ ηڢÔÙ‡Ô˘ ÙˆÓ ÁÔÓ¤ˆÓ ‰È·ÈÛÙÒıËΠfiÙÈ Ë ÌËÙ¤Ú· ¤ÊÂÚ ÙÔ ›‰ÈÔ SMC(15) Û fiϘ ÙȘ ÌÂÙ·Ê¿ÛÂȘ Ô˘ ÌÂÏÂÙ‹ıËηÓ. √È ÁÔÓ›˜
Ê·È-
ÓÂÔÁÓfi.
·ÛıÂÓ›˜ No 2 ¤ˆ˜ ¡Ô 9 ·ÓȯÓ‡ÙËΠÌÈÎÚÔ‡ ÌÂÁ¤ıÔ˘˜ SMC(15). ∆·
·˘Ù¿,
Î·È ÔÈ ÂÚÈÙÒÛÂȘ Ô˘ ·Ó·Ê¤ÚÔÓÙ·È ÛÙË ‚È‚ÏÈÔÁÚ·Ê›·, ·ÚÔ˘Û›·˙·Ó ‹ÈÔ ÎÏÈÓÈÎfi Ê·ÈÓfiÙ˘Ô ¯ˆÚ›˜ ‰˘ÛÌÔÚÊÈο ¯·Ú·ÎÙËÚÈÛÙÈο, Ì ÌÂÙÚ›Ô˘ ‚·ıÌÔ‡ ÓÔËÙÈ΋ ˘ÛÙ¤ÚËÛË Î·È Ì·ıËÛȷο ÚÔ‚Ï‹Ì·Ù·. ∞˘Ùfi Èı·ÓÒ˜ ÔÊ›ÏÂÙ·È ÛÙËÓ ‡·ÚÍË ÛÙÔ SMC ›Ù ÌË ÏÂÈÙÔ˘ÚÁÈÎÒÓ ÁÔÓȉ›ˆÓ, ›Ù ÁÔÓȉ›ˆÓ Ô˘ ‰ÂÓ ÌÂÙ·ÁÚ¿ÊÔÓÙ·È (6). ™‡Ìʈӷ Ì ‚È‚ÏÈÔÁÚ·ÊÈο ‰Â‰Ô̤ӷ, ÌÂÁ¿ÏÔ˘ ÌÂÁ¤ıÔ˘˜ SMC(15)s Ô˘ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ÙÔ ÁÔÓ›‰ÈÔ SNRPN, ·Ú·ÙËÚÔ‡ÓÙ·È Û ÔÛÔÛÙfi 35% ÙˆÓ ·ÛıÂÓÒÓ Ì PWS, ÂÓÒ Ë ‰È·›ÛÙˆÛ‹ ÙÔ˘˜ ÛÙÔÓ ÚÔÁÂÓÓËÙÈÎfi ¤ÏÂÁ¯Ô ¤¯ÂÈ Î›Ó‰˘ÓÔ 12% ÂÌÊ¿ÓÈÛ˘ ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ÏfiÁˆ UPD Ô˘ ÚÔ·ÙÂÈ ·fi ÙËÓ Ù˘¯·›· ·‰Ú·ÓÔÔ›ËÛË ÙÔ˘ ·ÙÚÈÎÔ‡ ·ÏÏËÏfiÌÔÚÊÔ˘ (6-11). °È· ÙÔÓ ÏfiÁÔ ·˘Ùfi, Ù· ÌÂÁ¿ÏÔ˘ ÌÂÁ¤ıÔ˘˜ SMC(15)s ÛÙ· ÂÚÈÛÙ·ÙÈο ¡Ô 10 ¤ˆ˜ ¡Ô 16 Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ ‰ÈÂÚ¢ӋıËÎ·Ó ÂÚ·ÈÙ¤Úˆ Ì FISH, Ì ÙÔÓ ·ÓȯÓÂ˘Ù‹ SNRPN/15qtel, ÛÙËÓ ÎÚ›ÛÈÌË ÂÚÈÔ¯‹ ÙˆÓ Û˘Ó‰ÚfiÌˆÓ PWS/AS. ∞fi ÙÔ˘˜ ·ÛıÂÓ›˜ ·˘ÙÔ‡˜, ÔÈ ¡Ô 10 Î·È ¡Ô 14 ›¯·Ó ·ÚfiÌÔÈ· ÎÏÈÓÈ΋ Û˘Ìو̷ÙÔÏÔÁ›·, ˘ÔÙÔÓ›· Î·È Û·ÛÌÔ‡˜ Î·È ·Ú·¤ÌÊıËÎ·Ó ÁÈ· ΢ÙÙ·ÚÔÁÂÓÂÙÈÎfi ¤ÏÂÁ¯Ô Û ËÏÈΛ· 8 Î·È 8ó ÌËÓÒÓ, ·ÓÙ›ÛÙÔȯ·. ∏ ·ÚÔ˘Û›· ÙÚÈÒÓ ·ÓÙÈÁÚ¿ÊˆÓ Ù˘ ÎÚ›ÛÈÌ˘ ÂÚÈÔ¯‹˜ PWS/AS ÛÙ· ‰‡Ô ·˘Ù¿ ·È‰È¿ ÂȂ‚·ÈÒıËÎÂ Î·È Ì ·Ó¿Ï˘ÛË DNRPs. ∂ÈϤÔÓ, Ë ÌÂϤÙË ¤‰ÂÈÍ fiÙÈ ÙÔ SMC(15) ‹Ù·Ó Î·È ÛÙȘ ‰‡Ô ÂÚÈÙÒÛÂȘ ÌËÙÚÈ΋˜ ÚÔ¤Ï¢Û˘,
·ÏÏ¿ ÌÂϤÙË ÙÔ˘ ÚÔÙ‡Ô˘ ÌÂı˘Ï›ˆÛ˘ ·¤-
ÎÏÂÈÛ ÙËÓ Èı·ÓfiÙËÙ· ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ PWS.
™ÙËÓ ÂÚ›ÙˆÛË ¡Ô 15 ÙÔ ÌÂÁ¿ÏÔ SMC(15)
‹Ù·Ó de novo, ·ÓȯÓ‡ÙËΠÛÙÔÓ ÚÔÁÂÓÓËÙÈÎfi
¤ÏÂÁ¯Ô Î·È ‰ÂÓ ÂÚÈ›¯Â ÙËÓ ÎÚ›ÛÈÌË ÂÚÈÔ¯‹
ÙˆÓ Û˘Ó‰ÚfiÌˆÓ PW/AS. ∂Âȉ‹, fï˜, ‰ÂÓ
‹Ù·Ó ‰˘Ó·Ùfi Ó· ·ÔÎÏÂÈÛÙ› οÔÈÔ˜ ·ıÔÏÔ-
ÁÈÎfi˜ Ê·ÈÓfiÙ˘Ô˜ Î·È Ó· ÚÔÛ‰ÈÔÚÈÛÙ› Ë ‚·Ú‡ÙËÙ¿ ÙÔ˘, ÔÈ ÁÔÓ›˜ ·ÔÊ¿ÛÈÛ·Ó ÙË ‰È·ÎÔ‹ Ù˘ ·ËÛ˘.
π‰È·›ÙÂÚÔ ÂӉȷʤÚÔÓ ·ÚÔ˘ÛÈ¿˙ÂÈ Ë ·ÛıÂÓ‹˜ ¡Ô 11, Ë ÔÔ›· ·Ú·¤ÌÊıËΠÁÈ· ΢ÙÙ·ÚÔÁÂÓÂÙÈÎfi ¤ÏÂÁ¯Ô Û ËÏÈΛ· 2 ÂÙÒÓ ÏfiÁˆ ‹ÈˆÓ ‰˘ÛÌÔÚÊÈÎÒÓ ¯·Ú·ÎÙËÚÈÛÙÈÎÒÓ, fiˆ˜ ÌÈÎÚ‹
̇ÙË, ‚ÏÂÊ·ÚfiÙˆÛË, Ì·ÎÚ‡ Ê›ÏÙÚÔ Î·È Û˘-
ÌÙÒÌ·Ù· ȉÈÔ·ıÔ‡˜ ·ÚıÚ›Ùȉ·˜ Ì ÂȉÂÈÓÔ‡-
ÌÂÓË ÎÏÈÓÈ΋ ÂÈÎfiÓ·. ¶·Ú¿ ÙÔ ÁÂÁÔÓfi˜ fiÙÈ Ë ·È-
ÙÈÔÏÔÁ›· Ù˘ ȉÈÔ·ıÔ‡˜ ·ÚıÚ›Ùȉ·˜ ·Ú·Ì¤ÓÂÈ
¿ÁÓˆÛÙË, Û ۿÓȘ ÂÚÈÙÒÛÂȘ Ë ÓfiÛÔ˜ ¤¯ÂÈ
Û˘Ó‰Âı› Ì ¯ÚˆÌÔÛˆÌÈΤ˜ ·ÓˆÌ·Ï›Â˜, fiˆ˜
ÂÏÏ›ÌÌ·Ù· ÙÔ˘ Ì·ÎÚÔ‡ ÛΤÏÔ˘˜ ÙÔ˘ ¯ÚˆÌÔÛÒÌ·ÙÔ˜ 22 (22q-). øÛÙfiÛÔ, fiÛÔ Â›Ì·ÛÙ Û ı¤ÛË
Ó· ÁÓˆÚ›˙Ô˘ÌÂ, Â›Ó·È Ë ÚÒÙË ÊÔÚ¿ Ô˘ Û¯ÂÙ›˙ÂÙ·È Ì ÙËÓ ·ÚÔ˘Û›· SMC(15) (14-19).
™˘ÌÂÚ·ÛÌ·ÙÈο, Ë ·Ó›¯Ó¢ÛË de novo ‹ oÈÎÔÁÂÓÔ‡˜ SMC(15) ··ÈÙ› ÂÚ·ÈÙ¤Úˆ ‰ÈÂÚ‡ÓËÛË Ì ÌÔÚÈ·Îfi ΢ÙÙ·ÚÔÁÂÓÂÙÈÎfi Î·È ÌÔÚÈ·Îfi
¤ÏÂÁ¯Ô, Ì ÛÎÔfi Ó· ÚÔÛ‰ÈÔÚÈÛÙ› Ë ÁÔÓȉȷ΋ Û‡ÛÙ·ÛË ÙÔ˘ ˘ÂÚ¿ÚÈıÌÔ˘ ¯ÚˆÌÔÛÒÌ·ÙÔ˜, Ó· Ù·˘ÙÔÔÈËı› Ë ÁÔÓÂ˚΋ ÚԤϢÛË Î·È Ó· ·ÔÎÏÂÈÛÙ› Ë ÌÔÓÔÁÔÓÂ˚΋ ‰Èۈ̛· (UPD). √ ÔÏÔÎÏËڈ̤ÓÔ˜ ·˘Ùfi˜ ÁÂÓÂÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ Â›Ó·È ··Ú·›ÙËÙÔ˜ ÁÈ· ÙÔÓ Û˘Û¯ÂÙÈÛÌfi ÁÔÓÔÙ‡Ô˘ -
Ê·ÈÓÔÙ‡Ô˘, ·ÏÏ¿ Î·È ÁÈ· ÙË ÁÂÓÂÙÈ΋ ηıÔ‰‹ÁËÛË ÙˆÓ ÔÈÎÔÁÂÓÂÈÒÓ.
BÈ‚ÏÈÔÁÚ·Ê›·
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Chronic arthritis associated with chromosome deletion 22q11.2 syndrome. J Rheumatol 2002;29: 2648-2650.
19.Czako M, Riegel M, Morava E, Schinzel A, Kosztolanyi
G. Patient with rheumatoid arthritis and MCA/MR syndrome due to unbalanced der(18) transmission of a paternal translocation t(18;20) (p11.1;p11.1). Am J Med Genet 2002;108:226-228.
ABSTRACT
Child abuse registration, fetal growth, and preterm birth: a population based study
N. Spencer, A. Wallace, R. Sundrum, C. Bacchus, S. Logan
Objectives: To study the relation of intra-uterine growth and gestational age with child protection registration in a 20 year whole population birth cohort.
Setting: West Sussex area of England.
Study design: Retrospective whole population birth cohort. Outcomes: Child protection registration; individual categories of registration-sexual abuse, physical abuse, emotional abuse, and neglect. Population and participants: 119,771 infants born in West Sussex between January 1983 and December 2001 with complete data including birth weight, gestational age, maternal age, and postcode.
Results: In all categories of registration a linear trend was noted such that the lower the birth weight z score the higher the likelihood of child protection registration. Similar trends were noted for gestational age. All these trends were robust to adjustment for maternal age and socioeconomic status.
Conclusions: The results of this study suggest that lower levels of fetal growth and shorter gestational duration are associated with increased likelihood of child protection registration in all categories including sexual abuse independent of maternal age or socioeconomic status. This study does not permit comment on whether poor fetal growth or preterm birth predispose to child abuse and neglect or the association arises because they share a common pathway.
J Epidemiol Community Health, 2006;60(4):337-340.
Editorial commentary on next page
N. Spencer
The opportunity to study the association of child abuse with fetal growth and preterm birth arose out of our work with this unique population-based child health data set that combines high quality information on all children born with addresses in the West Sussex region of England and child protection registration data for the same region (1). Our review of the published literature suggested that the association was disputed and a previous review of casecontrol studies had concluded that the association was spurious (2). However, two population-based studies (3,4) reported an association with low birth weight after adjustment for confounding variables.
Previously reported studies had a number of limitations. They studied all forms of abuse combined, which precluded any analysis of how different forms of abuse might differ in their association with pregnancy outcomes. In addition, the studies that analysed the association of abuse with low birth weight (<2500 g) failed to distinguish between low birth weight resulting from impaired fetal growth or short gestation.
The West Sussex data set, collected over 19 years, was large enough to allow us to overcome some of these limitations. The paper reports on the association of fetal growth, expressed as birth weight z scores categorised into five groups, and gestational age, categorised into three groups, with registration for child abuse and neglect in four categories, physical, emotional, sexual, and neglect. The data set also enabled us to adjust for maternal age and an areabased measure of socioeconomic status, both of which are known to be associated with pregnancy outcomes and risk of abuse.
The results showed that, for all categories of abuse, there was a linear trend such that the lower the category of fetal growth and gestation the higher the risk of abuse. Neglect showed the strongest linear trend by both fetal growth and gestation and sexual abuse the weakest (1).
What can we conclude from these results? There can be little doubt that impaired fetal growth and short gestation are associated with subsequent risk of child protection registration independent of maternal age or socioeconomic status. It also seems that the risk of registration increases with increasing impairment of fetal growth and decreasing gestational age - in other words, they demonstrate a “dose-response” relationship. In common with all studies of child abuse and neglect, we relied on child protection registration to identify children who had been abused. Misclassification bias is likely to arise because some children who have been abused may not come to the atten-
tion of the services and may not be registered. It is important to be aware of this limitation when interpreting the study findings.
In practical terms, these results might help to alert practitioners to higher levels of risk among infants born small and/or early but there remain questions that our findings do not answer. Do impaired fetal growth and short gestation predispose to abuse and neglect? Is the association causal? Although the data are effectively longitudinal with birth weight, gestational age, maternal age and socioeconomic status data collected before the outcome of interest, we cannot say from the findings whether the relationship is causal or the result of a shared pathway related to an unmeasured maternal or paternal characteristic. Growth impaired and preterm infants are known to have developmental problems (5) that might make them more difficult to manage. They are also more likely to be separated from their mothers in the neonatal period and suffer illness and hospital admission in early infancy. These factors may increase the vulnerability of these infants to abuse and neglect. Alternatively, the association may not be causal but may result from other family characteristics that predispose to both poor pregnancy outcomes and abuse and neglect.
In summary, we think that, despite unanswered questions about the association, our study provides the most comprehensive data so far on the links between pregnancy outcome and child abuse. Future research should focus on eliminating other family characteristics as the underlying cause of this association and understanding the mechanisms by which the pathways from pregnancy outcome to abuse operate.
References
1.N. Spencer, A. Wallace, R. Sundrum, C. Bacchus, S. Logan. Child abuse registration, fetal growth, and preterm birth: a population based study. J Epidemiol Community Health 2006;60:337-340.
2. Leventhal JM. Risk factors for child abuse: methodologic standards in case-control studies. Pediatrics 1981;68:684-690.
3.Needell B, Barth RP. Infants entering foster care compared with other infants using birth status indicators. Child Abuse Negl 1998;22:1179-1187.
4.Sidebotham P, Heron J; ALSPAC Study Team. Child maltreatment in the “children of the nineties”: the role of the child. Child Abuse Negl 2003;27:337-352.
5.Pharoah PO, Stephenson CJ, Cooke RW, Stephenson RC. Clinical and sub-clinical deficits at 8 years in a geographically defined cohort of low birthweight infants. Arch Dis Child 1994;70:264-270.
School of Health and Social Studies and Warwick Medical School, University of Warwick, Coventry, UK
Correspondence: E-mail: n.j.spencer@warwick.ac.uk
¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋
∞ÈÌ·ÙÔÏÔÁ›·˜/√ÁÎÔÏÔÁ›·˜,
¶·ÓÂÈÛÙËÌÈ·Îfi ¡ÔÛÔÎÔÌ›Ô
∏Ú·ÎÏ›Ԣ ∫Ú‹Ù˘
AÏÏËÏÔÁÚ·Ê›·:
ª·Ú›· ∫·ÏÌ·ÓÙ‹
¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋
∞ÈÌ·ÙÔÏÔÁ›·˜/√ÁÎÔÏÔÁ›·˜
¶·ÓÂÈÛÙËÌÈ·Îfi ¡ÔÛÔÎÔÌ›Ô
∏Ú·ÎÏ›Ԣ ∫Ú‹Ù˘
∆.£. 2208, ∆.∫. 710 03, ∏Ú¿ÎÏÂÈÔ ∫Ú‹Ù˘ E-mail: pedhem@med.uoc.gr
∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 28-01-2005
∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 28-03-2006
∞ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈÎfi Û‡Ó‰ÚÔÌÔ Û¯ÂÙÈ˙fiÌÂÓÔ
Ì ÙÔÓ Èfi Epstein-Barr. ¶ÂÚÈÁÚ·Ê‹ ‰‡Ô
ÂÚÈÙÒÛˆÓ
Ì ‰È·ÊÔÚÂÙÈ΋ ÎÏÈÓÈ΋ ¤Î‚·ÛË
∂. ¢ËÏ·Ó¿˜, µ. ¢·ÓËÏ¿ÙÔ˘, ∞. ∫·ÌÔ˘Ú¿Î˘, ∂.-∞. ª·ÚοÎË, ∂. ™ÙÂȷοÎË, ª. ∫·ÏÌ·ÓÙ‹
¶ÂÚ›ÏË„Ë: ∆Ô ·ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈÎfi Û‡Ó‰ÚÔÌÔ Â›Ó·È Û¿ÓÈ· ÔÈÎÔÁÂÓ‹˜ ‹ ‰Â˘ÙÂÚÔ·ı‹˜ Û˘ÛÙËÌ·ÙÈ΋ ‰È·Ù·Ú·¯‹ Ô˘ ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ˘ÂÚ‰Ú·ÛÙËÚÈfiÙËÙ· ÙˆÓ Ì·ÎÚÔÊ¿ÁˆÓ, ∆- Î·È ¡∫-ÏÂÌÊÔ΢ÙÙ¿ÚˆÓ. √ Èfi˜ Epstein-Barr (EBV) ıˆÚÂ›Ù·È Ô Î‡ÚÈÔ˜ ÂÎÏ˘ÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜. ¶ÂÚÈÁÚ¿ÊÔÓÙ·È ‰‡Ô ¿ÚÚÂÓ˜ ·ÛıÂÓ›˜, ËÏÈΛ·˜ 10 Î·È 3,5 ÂÙÒÓ, Ì ·ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈÎfi Û‡Ó‰ÚÔÌÔ Û¯ÂÙÈ˙fiÌÂÓÔ Ì EBV Î·È ‰È·ÊÔÚÂÙÈ΋ ÎÏÈÓÈ΋ ÔÚ›· Î·È ¤Î‚·ÛË. √ ÚÒÙÔ˜ ·ÛıÂÓ‹˜ ·ÚÔ˘Û›·Û ·Ó΢ÙÙ·ÚÔÂÓ›·, Ë·ÙÔÛÏËÓÔÌÂÁ·Ï›·, ÏÂÌÊ·‰ÂÓÔ¿ıÂÈ·
ÎÂÚȉ·ÈÌ›·.
Î·È ÙË ‰Â˘ÙÂÚÔ·ı‹ ÌÔÚÊ‹ Â›Ó·È ‰‡ÛÎÔϘ. √È ·ÛıÂÓ›˜ ÌÔÚ› Ó·
Paediatric Clinic of Haematology/Oncology, University Hospital of Heraklion, Crete
Correspondence: Maria Kalmanti
Paediatric Clinic of Haematology/Oncology University Hospital of Heraklion, Crete PO Box 2208, 710 03, Heraklion, Crete
E-mail: pedhem@med.uoc.gr
Date of submission: 28-01-2005
Date of approval: 28-03-2006
Haemophagocytic syndrome related to Epstein-Barr virus. Report of two cases with different clinical outcome
E. Dilanas, V. Danilatou, A. Kambourakis, E.-A. Markaki, E. Stiakaki, M. Kalmanti
Abstract: Haemophagocytic syndrome is a rare familial or secondary systemic disorder, which is characterized by excessive function of macrophages and T- and NK-lymphocytes. The major triggering factor is the Epstein-Barr virus (EBV). The described cases are of two male patients, aged 10 and 3.5 years respectively with EBV-associated haemophagocytic syndrome, with different course and outcome. The first patient presented with cytopenia, hepatospenomegaly and lymphadenopathy, and later developed CNS involvement, disseminated intravascular coagulopathy, hyperferritinaemia, hypofibrinogenaemia, and hypertriglyceridaemia. Diagnosis was documented by splenectomy and splenic histopathological examination, while serial bone marrow biopsies were negative for haemophagocytosis. He was treated in accordance with the HLH-94 protocol. Allogeneic bone marrow transplantation was performed four years after diagnosis, because of recurrent relapses, but the outcome was fatal. The second patient presented with infectious mononucleosis syndrome, anaemia, neutropenia, hypertriglyceridaemia and hyperferritinaemia. Diagnosis was established early by bone marrow biopsy. He was treated according to the HLH-94 protocol and is free of disease 12 months after completion of therapy. Haemophagocytic syndrome is a clinically heterogenous disease with high mortality. Diagnosis and distinction between familial and secondary disease can be difficult. One or more criteria may be met late in the course of the disease, but it is stressed that when that there
is strong clinical suspicion therapy should be initiated despite absence of diagnostic criteria. Bone marrow transplantation is indicated in cases of familial or refractory secondary haemophagocytic syndrome.
Key words: Haemophagocytic syndrome, Epstein-Barr virus, children, bone marrow transplantation.
™˘ÓÙÔÌÔÁڷʛ˜
∞º™∞ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈÎfi Û‡Ó‰ÚÔÌÔ
¢∂¶¢È¿¯˘ÙË ÂÓ‰·ÁÁÂȷ΋ ‹ÍË
π∞™πÔÁÂÓ¤˜ ·ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈÎfi Û‡Ó‰ÚÔÌÔ
∫¡™∫ÂÓÙÚÈÎfi Ó¢ÚÈÎfi Û‡ÛÙËÌ·
§ª§ÔÈÌ҉˘ ÌÔÓÔ˘Ú‹ÓˆÛË
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√∞™√ÈÎÔÁÂÓ¤˜ ·ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈÎfi Û‡Ó‰ÚÔÌÔ
º§À¡º˘ÏÔÛ‡Ó‰ÂÙË ÏÂÌÊÔ¸ÂÚÏ·ÛÙÈ΋ ÓfiÛÔ˜
EBVπfi˜ Epstein-Barr
PCRPolymerase chain reaction
CTComputerized tomography
MRIMagnetic resonance imaging
∂ÈÛ·ÁˆÁ‹
To ·ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈÎfi Û‡Ó‰ÚÔÌÔ (∞º™), ÁÓˆÛÙfi Î·È ˆ˜ ·ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈ΋ ÏÂÌÊÔ˚ÛÙÈÔ΢ÙÙ¿ÚˆÛË, ÂÚÈÏ·Ì‚¿ÓÂÈ ¤Ó· ¢ڇ Ê¿ÛÌ· ÎÏËÚÔÓÔÌÈÎÒÓ Î·È Â›ÎÙËÙˆÓ ÓÔÛËÌ¿ÙˆÓ, Ô˘ ¯·Ú·ÎÙËÚ›˙ÔÓÙ·È ·fi ‰È·Ù·Ú·¯‹ Ù˘ ·ÓÔÛÔÏÔÁÈ΋˜ Ú‡ıÌÈÛ˘. ÷ڷÎÙËÚ›˙ÂÙ·È ·fi ˘ÚÂÙfi, Ë·ÙÔÛÏËÓÔÌÂÁ·Ï›·, ÏÂÌÊ·‰ÂÓÔ¿ıÂÈ·, ÂÍ¿ÓıËÌ·, ÚÔÛ‚ÔÏ‹ ÙÔ˘ ÎÂÓÙÚÈÎÔ‡ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ (∫¡™), ΢ÙÙ·ÚÔÂӛ˜ Û ≥2 ·ÈÌÔÔÈËÙÈΤ˜ ÛÂÈÚ¤˜, ‰˘ÛÏÈȉ·ÈÌ›· Î·È ‰È·Ù·Ú·¯¤˜ ‹Í˘. ∏ ÓfiÛÔ˜ Û·ÓÈfiÙÂÚ· Â›Ó·È ÎÏËÚÔÓÔÌÈ΋ (ÔÈÎÔÁÂÓ¤˜ ·ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈÎfi Û‡Ó‰ÚÔÌÔ√∞™), ÂÌÊ·Ó›˙ÂÙ·È Û˘Ó‹ıˆ˜ Û ¿ÙÔÌ· ËÏÈΛ·˜ <2 ÂÙÒÓ, Ì ıÂÙÈÎfi ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi, ÁÔÓÂ˚΋ Û˘ÁÁ¤ÓÂÈ· ÂÍ ·›Ì·ÙÔ˜ Î·È ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ˘„ËÏ‹ ıÓËÙfiÙËÙ·. ∏ Û˘ÓËı¤ÛÙÂÚË ÌÔÚÊ‹
Â›Ó·È Ë ‰Â˘ÙÂÚÔ·ı‹˜ ÌÂÙ·ÏÔÈÌ҉˘ ‹ Û¯ÂÙÈ˙fiÌÂÓË Ì ηÎÔ‹ıÂȘ (΢ڛˆ˜ ÏÂÌÊÒÌ·Ù·) Î·È ·˘ÙÔ¿ÓÔÛ· ÓÔÛ‹Ì·Ù· (1).
√ Èfi˜ Epstein-Barr (EBV) ıˆÚÂ›Ù·È Ô Î‡ÚÈÔ˜ ÂÎÏ˘ÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ ÙÔ˘ ‰Â˘ÙÂÚÔ·ıÔ‡˜ ∞º™. ¶ÚÔÛ‚¿ÏÏÂÈ Ù· ∆- Î·È NK-ÏÂÌÊÔ·ÙÙ·Ú·, ÚÔηÏÒÓÙ·˜ ÙÔÓ ·ÓÂͤÏÂÁÎÙÔ ÌÔÓÔ‹ ÔÏÈÁÔÎψÓÈÎfi ÔÏÏ·Ï·ÛÈ·ÛÌfi ÙÔ˘˜ Î·È Û˘ÓÔ‰fi ˘ÂÚ·Ú·ÁˆÁ‹ ΢ÙÙ·ÚÔÎÈÓÒÓ (2). ∏
ÏÂÈÔÓfiÙËÙ· ÙˆÓ ÂÚÈÙÒÛÂˆÓ ÂÌÊ·Ó›˙ÂÙ·È ÛÂ
·È‰È¿ Î·È ÂÊ‹‚Ô˘˜ ÌÂ Ê˘ÛÈÔÏÔÁÈ΋ ·ÓÔÛÔÏÔÁÈ΋ ·ÓÙ·fiÎÚÈÛË (2). ¶ÂÚÈÁÚ¿ÊÔÓÙ·È ‰‡Ô
ËÏÈΛ·˜ 10 Î·È 3,5 ÂÙÒÓ Ì ∞º™ Û¯ÂÙÈ˙fiÌÂÓÔ ÌÂ
ÙÔÓ Èfi EBV (EBV-∞º™) Ì ‰È·ÊÔÚÂÙÈ΋ ÎÏÈÓÈ΋ ÔÚ›· Î·È ¤Î‚·ÛË.
¶ÂÚÈÁÚ·Ê‹ ÂÚÈÙÒÛˆÓ
¶ÂÚ›ÙˆÛË ÚÒÙË
ËÏÈΛ·˜ 10 ÂÙÒÓ ÚÔÛ‹Ïı ÛÙËÓ ¶·È-
Â̇ÚÂÙ· Î·È ‰ÈfiÁΈÛË ‹·ÙÔ˜ Î·È ÛÏËÓfi˜, ÙÔ˘˜ ÚÔËÁÔ‡ÌÂÓÔ˘˜ 10 Ì‹Ó˜. ∞fi ÙËÓ ÎÏÈÓÈ΋ ÂͤٷÛË ‰È·ÈÛÙÒıËΠ‰ÈfiÁΈÛË ‹·ÙÔ˜ Î·È ÛÏËÓfi˜ (2 cm Î·È 10 cm οو ·fi ÙÔ Ï¢ÚÈÎfi ÙfiÍÔ, ·ÓÙ›ÛÙÔȯ·) Î·È ·fi ÙÔÓ ·Ú¯ÈÎfi ÂÚÁ·ÛÙËÚÈ·Îfi ¤ÏÂÁ¯Ô Ï¢ÎÔÂÓ›·, Ô˘‰ÂÙÂÚÔÂÓ›·, ·Ó·ÈÌ›·, ‹È· ıÚÔÌ‚ÔÂÓ›·, ·˘ÍË̤ӷ Ë·ÙÈο ¤Ó˙˘Ì·
Î·È LDH (¶›Ó·Î·˜ 1).
√ ÏÂÙÔÌÂÚ‹˜ ¤ÏÂÁ¯Ô˜ ÁÈ· ÏÔÈÌÒ‰ÂȘ ·Ú¿ÁÔÓÙ˜, ·˘ÙÔ¿ÓÔÛ· ÓÔÛ‹Ì·Ù· Î·È Î·ÎÔ‹ıÂȘ, Ô˘ ÂÚÈÂÏ¿Ì‚·Ó ηÏÏȤÚÁÂȘ ‚ÈÔÏÔÁÈÎÒÓ
˘ÁÚÒÓ, ·ÓÔÛÔÛÊ·ÈÚ›Ó˜ ÔÚÔ‡, ÔÛÊ˘ÔÓˆÙÈ·›· ·Ú·Î¤ÓÙËÛË (√¡¶), Ì˘ÂÏfiÁÚ·ÌÌ·, ÔÛÙÂÔÌ˘ÂÏÈ΋ ‚ÈÔ„›· Î·È ·ÂÈÎÔÓÈÛÙÈÎfi ¤ÏÂÁ¯Ô, ‹Ù·Ó
·ÚÓËÙÈÎfi˜, ÏËÓ ÙÔ˘ ÔÚÔÏÔÁÈÎÔ‡ ÂϤÁ¯Ô˘ Ô˘
¤‰ÂÈÍ ÔÍ›· Ïԛ̈ÍË ·fi Èfi EBV. ∏ PCR ÁÈ·
ÙÔÓ EBV ÛÙ· ·ÙÙ·Ú· ÙÔ˘ ÂÚÈÊÂÚÈÎÔ‡ ·›Ì·ÙÔ˜
‹Ù·Ó ıÂÙÈ΋. √ ¤ÏÂÁ¯Ô˜ ÙˆÓ ÏÂÌÊÔ΢ÙÙ·ÚÈÎÒÓ
ÏËı˘ÛÌÒÓ ¤‰ÂÈÍ ·Ó·ÛÙÚÔÊ‹ Ù˘ ·Ó·ÏÔÁ›·˜
CD4+/CD8+ (0,76).
∞Ú¯Èο ¯ÔÚËÁ‹ıËΠÂÌÂÈÚÈ΋ ‰ÈÏ‹ ·ÓÙÈ‚ÈÔÙÈ΋ ·ÁˆÁ‹. ∆Ë 15Ë Ë̤ڷ Ù˘ ÓfiÛÔ˘ ÂÌÊ¿ÓÈÛ ÁÂÓÈÎÂ˘Ì¤ÓÔ ÎËÏȉԂϷÙȉ҉˜, ÂÙ¯ÂÈ҉˜ ÂÍ¿ÓıËÌ·, ·Ó·Ó¢ÛÙÈ΋ ‰˘Û¯¤ÚÂÈ· Î·È Âȉ›ӈÛË Ù˘ ·Ó΢ÙÙ·ÚÔÂÓ›·˜ Ì ‰È·Ù·Ú·¯¤˜ ‹Í˘ (¶›Ó·Î·˜ 1). ∆Ô 18Ô 24ˆÚÔ Ù˘ ÓfiÛÔ˘ ÂȉÂÈÓÒıËÎÂ Ë ÁÂÓÈ΋ ÙÔ˘ ηٿÛÙ·ÛË, Ì ¤ÓÙÔÓË ·Ó·Ó¢ÛÙÈ΋ ‰˘Û¯¤ÚÂÈ·, ÂÒ‰˘ÓË
ÂÈÎfiÓ· ηٷÏËÍ›·˜, ‰È¿¯˘ÙË
‹ÍË (¢∂¶) (¶›Ó·Î·˜ 1) Î·È ÎÏÈÓÈÎÔ-·ÎÙÈÓÔÏÔÁÈο Â˘Ú‹Ì·Ù· Ó¢ÌÔÓ›·˜ ÌÂ
Ï¢ÚÈÙÈ΋ Î·È ·ÛÎÈÙÈ΋
¶›Ó·Î·˜ 1. ∂ÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ ÙÔ˘ ·ÛıÂÓÔ‡˜ Ù˘
∏̤ڷ ÓfiÛÔ˘5Ë15Ë18Ë23Ë30‹37Ë57Ë87Ë
Ht (%)32,228,725,529,725,523,234,433,6 Hb (g/dl)10,59,58,89,98,77,711,311,5
§Â˘Î¿/Ìl1400150017001600120016002170013600
∞∞¶/Ìl750500500480500490154004000
∞ª¶ x103/Ìl143165525473793,2367
¢∂∫ (%)0,760,550,53
SGOT (U/L)1811015529913317464228
SGPT (U/L)554910089732545862
LDH (U/L)546768124311871876197625981165
ÁGT (U/L)874752262315206241
∆°∫ (mg/dl)115781396289
ºÂÚÚÈÙ›ÓË (ng/ml)176996403188153335412813
PT (sec)15,219,114,313,21312,422,1
aPTT (sec)44,958,637,944,348,43076,5
πÓˆ‰ÔÁfiÓÔ (mg/dl)18110996238155233152
D-dimers (Ìg/ml)5,278,873,919,792,461,7132,8
ATIII (%)58,271
∞∞¶: ∞fiÏ˘ÙÔ˜ ·ÚÈıÌfi˜
∞ª¶: ∞ÈÌÔÂÙ¿ÏÈ·
∆°∫: ∆ÚÈÁÏ˘ÎÂÚ›‰È·
Û˘ÏÏÔÁ‹. ÃÔÚËÁ‹ıËÎ·Ó ÎÔÚÙÈÎÔÂȉ‹ ÁÈ· ÙËÓ
·Ó·ÎÔ‡ÊÈÛË ÙˆÓ ·Ó·Ó¢ÛÙÈÎÒÓ Û˘Ìو̿-
ÙˆÓ, ηıÒ˜ Î·È Û˘Ì˘Îӈ̤ӷ ÂÚ˘ıÚ¿, ÊÚ¤-
ÛÎÔ Î·Ù„˘Á̤ÓÔ Ï¿ÛÌ· Î·È ·ÓÙÈıÚÔÌ‚›ÓË πππ
ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ·Ó·ÈÌ›·˜ Î·È Ù˘
¢∂¶. √ ΢ÙÙ·ÚÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÙÔ˘ ˘ÏÈÎÔ‡
·fi ÙËÓ ·Ú·Î¤ÓÙËÛË Ù˘ Ï¢ÚÈÙÈ΋˜ ηÈ
·ÛÎÈÙÈ΋˜ Û˘ÏÏÔÁ‹˜ ‹Ù·Ó ·ÚÓËÙÈÎfi˜ ÁÈ· η-
ÎÔ‹ıÂÈ·.
∆ËÓ 23Ë Ë̤ڷ Ù˘ ÓfiÛÔ˘, ÂÓÒ Ô ·ÛıÂÓ‹˜
Û˘Ó¤¯È˙ ӷ ˘Ú¤ÛÛÂÈ Ì ÂÈÎfiÓ· ·Ó΢ÙÙ·ÚÔ-
ÂÓ›·˜, ¢∂¶ Î·È ·‡ÍËÛ˘ ÙˆÓ ÙÚ·ÓÛ·ÌÈÓ·ÛÒÓ (¶›Ó·Î·˜ 1), ·ÚÔ˘Û›·Û ÂÂÈÛfi‰ÈÔ ÁÂÓÈÎÂ˘Ì¤ÓˆÓ ÙÔÓÈÎÔÎÏÔÓÈÎÒÓ Û·ÛÌÒÓ. ∆Ô Ì˘ÂÏfiÁÚ·ÌÌ· Î·È Ë ÔÛÙÂÔÌ˘ÂÏÈ΋ ‚ÈÔ„›· ·ӷϋÊıË-
ηÓ, ·ÏÏ¿ ‰ÂÓ ‹Ù·Ó ‰È·ÁÓˆÛÙÈο. ¢ÈÂÓÂÚÁ‹ıËΠ‚˘ıÔÛÎfiËÛË, √¡¶ Î·È CT ÂÁÎÂÊ¿ÏÔ˘ ‰›¯ˆ˜ ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù·. ÃÔÚËÁ‹ıËΠ·Î˘ÎÏÔ‚›ÚË Ì ÙËÓ ˘fiÓÔÈ· ÂÁÎÂÊ·Ï›Ùȉ·˜ Î·È Ê·ÈÓ˘-
ÁÈ·
Û˘Ìو̿وÓ. ∏ ÈÛÙÔÏÔÁÈ΋ ÂͤٷÛË ÙÔ˘ ÛÏËÓfi˜ ‹Ù·Ó Û˘Ì‚·Ù‹ Ì ·ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈÎfi Û‡Ó‰ÚÔÌÔ. O ·ÛıÂÓ‹˜ ·ÓÂÌÊ¿ÓÈÛ ˘ÚÂÙfi, Ï‹ı·ÚÁÔ, ‰È¿¯˘ÙÔ ÎËÏȉԂϷÙȉ҉˜ ÂÍ¿ÓıËÌ·, ÙÚ·¯ËÏÈ΋ ÏÂÌÊ·‰ÂÓÔ¿ıÂÈ·, ˘ÔÓ·ÙÚÈ·ÈÌ›·, ¢∂¶ Î·È ·˘ÍË̤ӷ Ë·ÙÈο ¤Ó˙˘Ì·, LDH Î·È ÙÚÈÁÏ˘ÎÂÚ›‰È· ÙËÓ 87Ë Ë̤ڷ Ù˘ ÓfiÛÔ˘ (¶›Ó·Î·˜ 1). ™ÙË Ê¿ÛË ·˘Ù‹ ¤Ï·‚ ıÂڷ¢ÙÈο Á-ÛÊ·ÈÚ›ÓË. §fiÁˆ Âȉ›ӈÛ˘ Ù˘ ·Ó·Ó¢ÛÙÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ ‰È·ÛˆÏËÓÒıËÎÂ Î·È ÌÂٷʤÚıËΠÛÙË ÌÔÓ¿‰· ÂÓÙ·ÙÈ΋˜ ıÂڷ›·˜. ∏ ·ӿÏË„Ë Ù˘ ÔÛÙÂÔÌ˘ÂÏÈ΋˜ ‚ÈÔ„›·˜ ‹Ù·Ó Û˘Ì‚·Ù‹ Ì ·ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈÎfi Û‡Ó‰ÚÔÌÔ Î·È Ë ÂͤٷÛË ÙÔ˘ ÂÁÎÂÊ·ÏÔÓˆÙÈ·›Ô˘ ˘ÁÚÔ‡ (∂¡À) ¯ˆÚ›˜ ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù·. ÕÌÂÛ· ÍÂΛÓËÛ ¯ËÌÂÈÔıÂڷ›· Û‡Ìʈӷ Ì ÙÔ ÚˆÙfiÎÔÏÏÔ HLH94 (∂ÈÎfiÓ· 1), ÙÔ ÔÔ›Ô ÂÚÈÏ·Ì‚¿ÓÂÈ ÂÓ‰ÔÊϤ‚È· ÂÙÔÔÛ›‰Ë Î·È ‰ÂÍ·ÌÂı·˙fiÓË. ∏ ·ÓÙ·fiÎÚÈÛË ÛÙË ıÂڷ›· ‹Ù·Ó ¿ÌÂÛË Î·È ı·̷ÙÈ΋. ™ÙË Û˘Ó¤¯ÂÈ·, fï˜, ·ÚÔ˘Û›·Û ÔÏϷϤ˜ ˘ÔÙÚÔ¤˜ Ì ˘ÚÂÙfi, ·Ó·Ó¢ÛÙÈ΋ ‰˘Û¯¤ÚÂÈ·, Ó¢ÌÔÓ›·, ÂÍ¿ÓıËÌ· Î·È ·‡ÍËÛË ÙˆÓ ‚ÈÔ¯ËÌÈÎÒÓ ‰ÂÈÎÙÒÓ Ù˘ ÓfiÛÔ˘ (ÊÂÚÚÈÙ›Ó˘, ÙÚ·ÓÛ·ÌÈÓ·ÛÒÓ, ÙÚÈÁÏ˘ÎÂÚȉ›ˆÓ). √ ¤ÏÂÁ¯Ô˜ ÁÈ· HLA Û˘Ì‚·ÙfiÙËÙ· ÙÔ˘ ·‰ÂÏÊÔ‡ ÙÔ˘ ·¤‚Ë ·ÚÓËÙÈÎfi˜ Î·È Ë ·Ó·˙‹ÙËÛË ÌË Û˘ÁÁÂÓÔ‡˜ Û˘Ì‚·ÙÔ‡ ‰fiÙË ‰ÂÓ ·Ó¤‰ÂÈÍ ·fiÏ˘Ù· Û˘Ì‚·Ùfi ‰fiÙË. ∏ ¡∫ ‰Ú·ÛÙËÚÈfiÙËÙ· ‹Ù·Ó ÂÏ·Ùو̤ÓË.
∂ÈÎfiÓ· 1. ÃËÌÂÈÔıÂڷ¢ÙÈÎfi
HLH-94 (4). DEXA: ¢ÂÍ·ÌÂı·˙fiÓË. ∫·Ù¿ ÙË ıÂڷ›· ÂÊfi‰Ô˘ ¯ÔÚËÁÂ›Ù·È ‰ÂÍ·ÌÂı·˙fiÓË Ì ·Ú¯È΋ ‰fiÛË 10 mg/m2 Î·È ˘Ô‰ÈÏ·ÛÈ·ÛÌfi Ù˘ ‰fiÛ˘ ·Ó¿ ‰‡Ô ‚‰ÔÌ¿‰Â˜. ™ÙË ıÂڷ›· Û˘ÓÙ‹ÚËÛ˘ ¯ÔÚËÁÔ‡ÓÙ·È ÒÛÂȘ ‰ÂÍ·ÌÂı·˙fiÓ˘ ÙˆÓ 10 mg/m2 ÙÚÈ‹ÌÂÚ˘ ‰È¿ÚÎÂÈ·˜ οı ‰Â‡ÙÂÚË Â‚‰ÔÌ¿‰·.
VP-16: ∂ÙÔÔÛ›‰Ë 150 mg/m2 ÂÓ‰ÔÊÏ‚›ˆ˜. ∫·Ù¿ ÙËÓ ÂÊfi‰Ô ¯ÔÚËÁÔ‡ÓÙ·È ÙȘ ‰‡Ô ÚÒÙ˜ ‚‰ÔÌ¿‰Â˜ ‰‡Ô ‰fiÛÂȘ Î·È ÙȘ ÂfiÌÂÓ˜ Ì›· ‰fiÛË ·Ó¿ ‚‰ÔÌ¿‰·. ∫·Ù¿ ÙË ‰È¿ÚÎÂÈ·
Ì›· ‰fiÛË Î¿ı ‰‡Ô ‚‰ÔÌ¿‰Â˜.
CSA: ∫˘ÎÏÔÛÔÚ›ÓË. ∫·ıËÌÂÚÈÓ‹ ¯ÔÚ‹ÁËÛË ÛÙË Ê¿ÛË Û˘ÓÙ‹ÚËÛ˘.
π∆ ª∆Ã: ∂Ó‰ÔÚ·¯È·›· ÌÂıÔÙÚÂÍ¿ÙË Î·Ù¿ ÙËÓ ·Ú¯‹ Ù˘ 3˘, 4˘, 5˘ Î·È 6˘ ‚‰ÔÌ¿‰·˜ Ù˘ ıÂڷ›·˜ ÂÊfi‰Ô˘, ¿Ó
˘¿Ú¯Ô˘Ó ÂÍÂÏÈÛÛfiÌÂÓ· Ó¢ÚÔÏÔÁÈο Û˘ÌÙÒÌ·Ù· ‹ ÛÙ·ıÂÚ¿ ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù· ÛÙÔ ∂¡À. ∞ÛıÂÓ›˜ ¯ˆÚ›˜ √∞™ ‹ ÂÓÂÚÁ‹ ÓfiÛÔ, ÚÔÙ›ÓÂÙ·È Ó· ‰È·ÎfiÙÔ˘Ó ÙË ıÂڷ›· ÌÂÙ¿ ÙËÓ ÔÏÔÎÏ‹ÚˆÛË Ù˘ ‰fiÛ˘ ÂÊfi‰Ô˘ Î·È Ó· ÙËÓ ÍÂÎÈÓÔ‡Ó ÂÎ Ó¤Ô˘ Û ÂÚ›ÙˆÛË ·Ó·˙ˆ‡ÚˆÛ˘.
BMT: ªÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ Ê¿Û˘ Û˘ÓÙ‹ÚËÛ˘.
‹Ù·Ó 2460 U/ml, ÂÓÒ ÙËÓ 57Ë Â‚‰ÔÌ¿‰· ıÂÚ·-
›·˜ ÂÏ·ÙÙÒıËÎ·Ó Û 1348 IU/ml (º∆ <1000 IU/ml). ¢ÂÓ ‚Ú¤ıËΠÌÂÙ¿ÏÏ·ÍË ÙÔ˘ ÁÔÓȉ›Ô˘
ÂÚÊÔÚ›Ó˘, ÂÓÒ Ô ÁÔÓȉȷÎfi˜ ¤ÏÂÁ¯Ô˜ ÁÈ· Ê˘ÏÔÛ‡Ó‰ÂÙË ÏÂÌÊÔ¸ÂÚÏ·ÛÙÈ΋ ÓfiÛÔ (º§À¡)
‰ÂÓ ˘‹Ú¯Â ‰È·ı¤ÛÈÌÔ˜ Û˘Ì‚·Ùfi˜ ‰fiÙ˘ ÁÈ· ·ÏÏÔÁÂÓ‹ ÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ (ªª√). ¢¤Î· ̤Ú˜ ·ÚÁfiÙÂÚ· ˘ÔÙÚÔ›·ÛÂ
Î·È ·fi ÙfiÙ ¯ÔÚËÁ‹ıËÎ·Ó ÌÔÓÔ‹ÌÂÚ˜ ÒÛÂȘ
‰ÂÍ·ÌÂı·˙fiÓ˘ ‚‰ÔÌ·‰È·›·, Ì ÛÙ·‰È·Î‹ Ì›ˆÛË Ù˘ ‰fiÛ˘ Î·È ‰È·ÎÔ‹ ÌÂÙ¿ ¤Ó· ¯ÚfiÓÔ.
∆ÚÂȘ Ì‹Ó˜ ÌÂÙ¿ ÙË ‰È·ÎÔ‹ Ù˘ ıÂڷ›·˜ Ô
¤‰ÂÈÍÂ Ê˘ÛÈÔÏÔÁÈÎfi ÔÏ˘ÌÔÚÊÈÛÌfi. ªÂ ÙË Û˘ÌÏ‹ÚˆÛË 62 ‚‰ÔÌ¿‰ˆÓ ıÂڷ›·˜, Ô˘ ÂÚÈÂÏ¿Ì‚·Ó ÒÛÂȘ ÂÙÔÔÛ›‰Ë˜, ‰ÂÍ·ÌÂı·˙fiÓ˘ Î·È Î˘ÎÏÔÛÔÚ›ÓË (∂ÈÎfiÓ· 1), ·ÔÊ·Û›ÛÙËÎÂ Ë ‰È·ÎÔ‹ ÙÔ˘ ÚˆÙÔÎfiÏÏÔ˘ ÁÈ·Ù› ‰ÂÓ ·ÓÙ·ÔÎÚÈÓfiÙ·Ó ÛÙË ıÂڷ›· Î·È Â›¯Â ·Ó·Ù‡ÍÂÈ ˘ÂÚ‚ÔÏÈ΋ Ê·Ú̷΢ÙÈ΋ ÙÔÍÈÎfiÙËÙ· (ÈÓÛÔ˘ÏÈÓÔÂÍ·ÚÙÒÌÂÓÔ˜ ۷ί·Ú҉˘ ‰È·‚‹Ù˘, ηٷÚÚ¿ÎÙ˘, ·¯˘Û·ÚΛ·, ÂÚÈÔÚÈÛÙÈ΋ Ó¢ÌÔÓÔ¿ıÂÈ·, ˘¤ÚÙ·ÛË, ÔÛÙÂÔÂÓ›·, ¤ÎÙˆÛË Ù˘ ÓÂÊÚÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜, ÏȈ̿وÛË ÌÂÛÔıˆÚ·Î›Ô˘), ÂÈϤÔÓ ‰Â, Ô Î›Ó‰˘ÓÔ˜ ·Ó¿Ù˘Í˘ ‰Â˘ÙÂÚÔ·ıÔ‡˜ ηÎÔ‹ıÂÈ·˜ ‹Ù·Ó ·˘ÍË̤ÓÔ˜ Î·È Ë ·ÓÙ·fiÎÚÈÛË ÛÙË ıÂڷ›· ‰ÂÓ ‹Ù·Ó ÈηÓÔÔÈËÙÈ΋. ∂Ó·ÏÏ·ÎÙÈο ¯ÔÚËÁ‹ıËΠډÓÈ˙fiÓË, ‚ÈÓÎÚÈÛÙ›ÓË, ‰ÔÍÔÚÔ˘ÌÈΛÓË Î·È Î˘ÎÏÔʈÛÊ·Ì›‰Ë, ‰Â‰Ô̤ÓÔ˘ fiÙÈ
¶›Ó·Î·˜ 2. ∂ÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ ÙÔ˘ ·ÛıÂÓÔ‡˜ Ù˘ ‰Â‡ÙÂÚ˘
∏̤ڷ ÓfiÛÔ˘20‹22Ë26Ë41Ë331Ë
Hb (g/dl)8,57,878,911,7 Ht (%)24,22321,127,735,1
§Â˘Î¿/Ìl11200110007100132007700
∞∞¶/Ìl2700190040018001500
∞ª¶ x103/Ìl197000231000162000287000423000
SGOT (U/L)16315312310838
SGPT (U/L)1261026210543
ÁGT (U/L)210167924411
LDH (mg/dl)87910551109611250
∆°∫ (mg/dl)71930844865
ºÂÚÚÈÙ›ÓË (ng/ml)23411282266
PT (sec)12,712,61314,6 aPTT (sec)35,534,299,641
πÓˆ‰ÔÁfiÓÔ (mg/dl)341246307272
D-dimers (Ìg/ml)3,13,4 ATIII (%)124134
∞∞¶: ∞fiÏ˘ÙÔ˜ ·ÚÈıÌfi˜ ÔÏ˘ÌÔÚÊÔ‡ÚËÓˆÓ
∞ª¶: ∞ÈÌÔÂÙ¿ÏÈ·
∆°∫: ∆ÚÈÁÏ˘ÎÂÚ›‰È·
Î·È ÔÍ›· ÓfiÛÔ ÌÔۯ‡̷ÙÔ˜
ηٿ ÍÂÓÈÛÙ‹.
¶ÂÚ›ÙˆÛË ‰Â‡ÙÂÚË
ÀÁȤ˜, ηٿ ÙÔ ·ÚÂÏıfiÓ, ¿ÚÚÂÓ Ó‹ÈÔ ËÏÈΛ·˜ 3,5 ÂÙÒÓ ·Ú·¤ÌÊıËΠ·fi ‰Â˘ÙÂÚÔ‚¿ıÌÈÔ ÓÔÛÔÎÔÌÂ›Ô ÁÈ· ˘ÚÂÙfi ‰È¿ÚÎÂÈ·˜ 20 ËÌÂÚÒÓ. ∞fi ÙËÓ ÎÏÈÓÈ΋ ÂͤٷÛË ‰È·ÈÛÙÒıËÎÂ
ÂËÚ·Ṳ̂ÓË ÁÂÓÈ΋ ηٿÛÙ·ÛË, ˆ¯ÚfiÙËÙ·, ‰ÈfiÁΈÛË ÔÌ¿‰·˜ ÙÚ·¯ËÏÈÎÒÓ ÏÂÌÊ·‰¤ÓˆÓ
(‰È·Ì¤ÙÚÔ˘ 4 cm), ˘Ò‰Ë˜ ·Ì˘Á‰·Ï›Ùȉ· Î·È ‰ÈfiÁΈÛË ‹·ÙÔ˜ Î·È ÛÏËÓfi˜ (5 cm Î·È 9 cm
οو ·fi ÙÔ Ï¢ÚÈÎfi ÙfiÍÔ, ·ÓÙ›ÛÙÔȯ·). ∞fi
ÙÔÓ ÂÚÁ·ÛÙËÚÈ·Îfi ¤ÏÂÁ¯Ô ‰È·ÈÛÙÒıËΠ·Ó·ÈÌ›·, ·ÓÔÛÔÏÔÁÈο ‰ÈÂÁÂṲ́ӷ ÏÂÌÊÔ·ÙÙ·Ú· Ù‡Ô˘ ÏÔÈÌÒ‰Ô˘˜ ÌÔÓÔ˘Ú‹ÓˆÛ˘ ÛÙÔ Â›¯ÚÈÛÌ· ÙÔ˘ ÂÚÈÊÂÚÈÎÔ‡ ·›Ì·ÙÔ˜ Î·È ·‡ÍËÛË ÙˆÓ
¯ˆÚ›˜ ÎÏÈÓÈÎÔ-·ÎÙÈÓÔÏÔÁÈο ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù·, ÂÓÒ ÛÙËÓ ·Ó·ÈÌ›· ÚÔÛÙ¤ıËΠԢ‰ÂÙÂÚÔÂÓ›· (∞∞¶: 400/mm3). §fiÁˆ Ù˘ ÂÈÌÔÓ‹˜ ÙÔ˘ ˘ÚÂÙÔ‡, ¤ÁÈÓ·Ó √¡¶ Î·È MRI ÂÁÎÂÊ¿ÏÔ˘ Ô˘ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈΤ˜. ∆Ô Ì˘ÂÏfiÁÚ·ÌÌ· Î·È Ë ÔÛÙÂÔÌ˘ÂÏÈ΋ ‚ÈÔ„›· ¤‰ÂÈÍ·Ó ÂÚ˘ıÚÔÊ·ÁÔ΢ÙÙ¿ÚˆÛË. ªÂ ÙË ‰È¿ÁÓˆÛË ·ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘ Ô˘ Û¯ÂÙ›˙ÂÙ·È Ì EBV Ïԛ̈ÍË ÍÂΛÓËÛ ·ÁˆÁ‹ Ì ·Î˘ÎÏÔ‚›ÚË Î·È ÂÓ‰ÔÊϤ‚È· Á-ÛÊ·ÈÚ›ÓË, ÛÙËÓ ÔÔ›· Ô ·ÛıÂÓ‹˜ ‰ÂÓ ·ÓÙ·ÔÎÚ›ıËÎÂ. ∆ËÓ 41Ë Ì¤Ú· Ù˘ ÓfiÛÔ˘ ·ÚÔ˘Û›·Û ‰È·Ù·Ú·¯¤˜ ËÎÙÈÎfiÙËÙ·˜ (·Ú¿Ù·ÛË ÙÔ˘ aPTT), ÔfiÙ ·ÔÊ·Û›ÛÙËÎÂ Ë ¤Ó·ÚÍË ıÂڷ›·˜ Û‡Ìʈӷ Ì ÙÔ ÚˆÙÔÎfiÏÏÔ HLH 94. √ ·ÛıÂÓ‹˜ ·ÚÔ˘Û›·Û ÛÙ·‰È·Î‹ ˘Ô¯ÒÚËÛË Ù˘ Ë·ÙÔÛÏËÓÔÌÂÁ·Ï›·˜ Î·È ‚ÂÏÙ›ˆÛË ÙˆÓ ·ÈÌ·ÙÔÏÔÁÈÎÒÓ Â˘ÚËÌ¿ÙˆÓ, ηıÒ˜ Î·È ÙÔ˘ ‚ÈÔ¯ËÌÈÎÔ‡ ÚÔÊ›Ï (¶›Ó·Î·˜ 2). √È ·ÓÔÛÔÛÊ·ÈÚ›Ó˜ ÔÚÔ‡ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈΤ˜, ÂÓÒ Ô ¤ÏÂÁ¯Ô˜ ÙˆÓ ÏÂÌÊÔ΢ÙÙ·ÚÈÎÒÓ ÏËı˘ÛÌÒÓ ¤‰ÂÈÍ ·Ó·ÛÙÚÔÊ‹ ÙÔ˘ ÏfiÁÔ˘ CD4+/CD8+ (0,15 ¤ˆ˜ 0,4) ÚÈÓ ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂڷ›·˜. √ ·ÓÔÛÔÊ·ÈÓfiÙ˘Ô˜ ÙÔ˘ ÂÚÈÊÂÚÈÎÔ‡ ·›Ì·ÙÔ˜ ¤‰ÂÈÍ ÈÛ¯˘Ú‹ ¤ÎÊÚ·ÛË ÙˆÓ Î˘ÙÙ·ÚÔÙÔÍÈÎÒÓ ÏÂÌÊÔ΢ÙÙ¿ÚˆÓ Î·È ˘„ËÏfi ÔÛÔÛÙfi ·ÓÙÈÁÔÓÔ·ÚÔ˘ÛÈ·ÛÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ (CD8+ 78,4%, CD2+/CD86+ 30%, CD1a+ 56,2%, CD40+ 60%). √ ÏËı˘ÛÌfi˜ ÙˆÓ ¡∫-΢ÙÙ¿ÚˆÓ ‹Ù·Ó ÂÏ·Ùو̤ÓÔ˜ (1,8%). ∆· ›‰· ÙÔ˘ sCD25 Î·È ÙÔ˘ TNFa ÛÙÔÓ ÔÚfi ‹Ù·Ó ·˘ÍË̤ӷ (2190 U/ml Î·È 915 pg/ml, ·ÓÙ›ÛÙÔȯ·), ÂÓÒ ÌÂÙ¿ ÙË ıÂڷ›· ÂÏ·ÙÙÒıËÎ·Ó (588 U/ml Î·È 37,8 pg/ml, ·ÓÙ›ÛÙÔȯ·).
¶›Ó·Î·˜ 3. ∞Ó·ıˆÚË̤ӷ ‰È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· (3)
∏̤ڷ ÓfiÛÔ˘20‹22Ë26Ë41Ë331Ë
● √ÈÎÔÁÂÓ‹˜ ÓfiÛÔ˜ / ÁÓˆÛÙ‹ ÁÂÓÂÙÈ΋ ‰È·Ù·Ú·¯‹
● ∫ÏÈÓÈο Î·È ÂÚÁ·ÛÙËÚȷο Â˘Ú‹Ì·Ù· (¤ÓÙ ·fi Ù· ÔÎÙÒ)
ñ ¶˘ÚÂÙfi˜
ñ ™ÏËÓÔÌÂÁ·Ï›·
ñ ∫˘ÙÙ·ÚÔÂÓ›· (›‰Ú·ÛË Û ≥2 ÛÂÈÚ¤˜ ÙÔ˘ ÂÚÈÊÂÚÈÎÔ‡ ·›Ì·ÙÔ˜)
- ·ÈÌÔÛÊ·ÈÚ›ÓË <9 g/dl (ÚÈÓ ÙȘ 4 ‚‰ÔÌ¿‰Â˜ <10 g/dl)
- ·ÈÌÔÂÙ¿ÏÈ· (<100x103/ÌL)
- Ô˘‰ÂÙÂÚfiÊÈÏ· (<1,0x103/ÌL)
ñ ÀÂÚÙÚÈÁÏ˘ÎÂÚȉ·ÈÌ›· ηÈ/‹ ˘Ô˚Óˆ‰ÔÁÔÓ·ÈÌ›· (ÙÚÈÁÏ˘ÎÂÚ›‰È· ÓËÛÙ›·˜ ≥264 mg/dL, ÈÓˆ‰ÔÁfiÓÔ <150 mg/dL)
ñ ºÂÚÚÈÙ›ÓË ≥500 Ìg/L
ñ sCD25 ≥2400 U/ml
ñ ªÂȈ̤ÓË ‹ ·Ô‡Û· ¡∫ ‰Ú·ÛÙËÚÈfiÙËÙ·
ñ ∞ÈÌÔÊ·ÁÔ΢ÙÙ¿ÚˆÛË ÛÙÔÓ Ì˘ÂÏfi, ÙÔ ÂÁÎÂÊ·ÏÔÓˆÙÈ·›Ô ˘ÁÚfi ‹ ÛÙÔ˘˜ ÏÂÌÊ·‰¤Ó˜
™ËÌ›ˆÛË: ∂ÈϤÔÓ ÛÙÔȯ›· ·ÔÙÂÏÔ‡Ó Û˘ÌÙÒÌ·Ù· ·fi ÙÔ ∫¡™ Ì ̤ÙÚÈ· ÏÂÈÔ΢ÙÙ¿ÚˆÛË Î·È/‹ ·˘ÍË̤ÓÔ
χΈ̷, ·˘ÍË̤Ó˜ ÙÚ·ÓÛ·ÌÈÓ¿Û˜, ¯ÔÏÂÚ˘ıÚ›ÓË Î·È LDH >1000 U/L.
∂›ÁÔ˘Û· ıÂڷ›· Û ·ÛıÂÓ›˜ Ì ÛÔ‚·Ú‹ ‰È·Ù·Ú·¯‹ ‹Í˘ ÌÔÚ› Ó· ÍÂÎÈÓ‹ÛÂÈ Ì ¤Ó· ÎÚÈÙ‹ÚÈÔ ÏÈÁfiÙÂÚÔ. ¶ÚÈÓ ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂڷ›·˜, ‰Â›ÁÌ·Ù·
¢ÂÓ ‰È·ÈÛÙÒıËΠÌÂÙ¿ÏÏ·ÍË ÙÔ˘ ÁÔÓȉ›Ô˘ Ù˘
ÂÚÊÔÚ›Ó˘. √ ¤ÏÂÁ¯Ô˜ ÁÈ· ∏LA Û˘Ì‚·ÙfiÙËÙ· ¤‰ÂÈÍ fiÙÈ Â›Ó·È Ù·˘ÙfiÛËÌÔ˜ Ì ÙÔÓ ·‰ÂÏÊfi ÙÔ˘.
∏ ¯ÔÚ‹ÁËÛË ‰ÂÍ·ÌÂı·˙fiÓ˘ Î·È ÂÙÔÔÛ›‰Ë˜ ‰È·ÎfiËΠÙËÓ 39Ë Â‚‰ÔÌ¿‰· ıÂڷ›·˜, ÂÓÒ Û˘Ó¯›ÛÙËÎÂ Ë Î·ıËÌÂÚÈÓ‹ ¯ÔÚ‹ÁËÛË Î˘ÎÏÔÛÔÚ›Ó˘. ªÂ ÙËÓ ÔÏÔÎÏ‹ÚˆÛË 52 ‚‰ÔÌ¿‰ˆÓ ıÂڷ›·˜ Î·È ÂÓÒ Ô ·ÛıÂÓ‹˜ ‹Ù·Ó Û ϋÚË ÎÏÈÓÈÎÔÂÚÁ·ÛÙËÚȷ΋ ‡ÊÂÛË Ù˘ ÓfiÛÔ˘, ‰È·ÎfiËΠÙÔ ıÂڷ¢ÙÈÎfi ÚˆÙfiÎÔÏÏÔ. ∆Ô
·È‰› ·Ú·Ì¤ÓÂÈ ÂχıÂÚÔ ÓfiÛÔ˘ 12 Ì‹Ó˜ ÌÂ-
Ù¿ ÙÔ Ù¤ÏÔ˜ Ù˘ ıÂڷ›·˜.
™˘˙‹ÙËÛË
∆Ô Û¯ÂÙÈ˙fiÌÂÓÔ Ì EBV ∞º™ Â›Ó·È Ì›· Û¿ÓÈ· Î·È ÂÙÂÚÔÁÂÓ‹˜ ÓfiÛÔ˜ Ô˘ ·ÔÙÂÏ› ÚfiÎÏËÛË ÁÈ· ÙÔÓ ·È‰›·ÙÚÔ-·ÈÌ·ÙÔÏfiÁÔ. ∏ ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË ·fi ¿ÏÏ· ÓÔÛ‹Ì·Ù·, fiˆ˜ ÏÔÈÌ҉˘ ÌÔÓÔ˘Ú‹ÓˆÛË (§ª), Û˘ÛÙËÌ·ÙÈΤ˜ ·˘ÙÔ¿ÓÔÛ˜ ‰È·Ù·Ú·¯¤˜ Î·È ·Ï·ÛÙÈ΋ ·Ó·ÈÌ›·, Û˘¯Ó¿ Â›Ó·È ‰‡ÛÎÔÏË Î·È ¯ÚÂÈ¿˙ÂÙ·È ¿ÓÙ· Ó· ·ÔÎÏÂÈÛÙÔ‡Ó Î·ÎÔ‹ıÂȘ ‹ ˘ÔΛÌÂÓ· ÓÔÛ‹Ì·Ù·, fiˆ˜ ¯ÚfiÓÈ· ÂÓÂÚÁfi˜ Ïԛ̈ÍË ·fi EBV, √∞™ Î·È º§À¡ (1). ∏ ‰È¿ÁÓˆÛË ÛÙËÚ›˙ÂÙ·È
‡·ÚÍË ÙˆÓ ‰È·ÁÓˆÛÙÈÎÒÓ ÎÚÈÙËÚ›ˆÓ Ô˘
¤¯Ô˘Ó ÚfiÛÊ·Ù· ·Ó·ıˆÚËı› (¶›Ó·Î·˜ 3) (3). ™Ùo √∞™ ÌÔÚ› Ó· Û˘ÌÌÂÙ¤¯ÂÈ Î·È ÏÔÈÌ҉˘ ·Ú¿ÁÔÓÙ·˜ (2,4). ∆Ô ·ÚÓËÙÈÎfi ÔÈÎÔÁÂÓÂÈ-
ÌÂÙ·ÏÏ¿ÍÂȘ ÙÔ˘ ÁÔÓȉ›Ô˘ Ù˘ ÂÚÊÔÚ›Ó˘ (3). ∏ ÂÚÊÔÚ›ÓË ·›˙ÂÈ ÛËÌ·ÓÙÈÎfi ÚfiÏÔ ÛÙËÓ ÂÏÂÁ¯fiÌÂÓË ÂÓÂÚÁÔÔ›ËÛË Ù˘ ΢ÙÙ·ÚÈ΋˜
·ÓÔÛ›·˜ Î·È ÛÙËÓ ÂÈÙ‹ÚËÛË ÙˆÓ ÌÔÏ˘Ṳ̂ӈÓ
·fi ÈÔ‡˜ ΢ÙÙ¿ÚˆÓ ÛÙËÓ ·È‰È΋ ËÏÈΛ· (3). ∏ º§À¡ Â›Ó·È Ì›· ·ÓÔÛÔÏÔÁÈ΋ ‰È·Ù·Ú·¯‹
Ô˘ ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ·˘ÍË̤ÓË Â˘·ÈÛıËÛ›·
ÛÙÔÓ Èfi EBV, ÎÂÚ·˘ÓÔ‚fiÏÔ §ª, ηÎÔ‹ıË ÏÂÌÊÒÌ·Ù· Î·È ˘ÔÁ·ÌÌ·ÛÊ·ÈÚÈÓ·ÈÌ›· (5). ∏ ÁÂÓÂÙÈ΋ ‚Ï¿‚Ë ÂÓÙÔ›˙ÂÙ·È ÛÙÔ ÁÔÓ›‰ÈÔ SH2D1A Î·È ÌÂÙ·‚È‚¿˙ÂÙ·È ÌÂ Ê˘ÏÔÛ‡Ó‰ÂÙÔ ˘ÔÏÂÈfiÌÂÓÔ ¯·Ú·ÎÙ‹Ú· (5). ∞ÚÎÂÙ¿ Û˘¯Ó¿ ÂÌÊ·Ó›˙Â-
Ù·È Ì ÚÒÙË ÂΉ‹ÏˆÛË ÙÔ ∞º™. ∂Âȉ‹ ·Ó·-
ʤÚÔÓÙ·È Î·È ÛÔÚ·‰ÈΤ˜ ÌÔÚʤ˜ Ù˘ ÓfiÛÔ˘, fiÏ· Ù· ¿ÚÚÂÓ· ·È‰È¿ Ì ∞º™ ı· Ú¤ÂÈ Ó·
ÂϤÁ¯ÔÓÙ·È ÁÂÓÂÙÈο ÁÈ· º§À¡, ·ÓÂÍ¿ÚÙËÙ· ·fi ÙËÓ ‡·ÚÍË ‹ fi¯È ıÂÙÈÎÔ‡ ÔÈÎÔÁÂÓÂÈ·ÎÔ‡ ÈÛÙÔÚÈÎÔ‡ (5). ∏ ·Ô˘Û›· ˘ÔÁ·ÌÌ·ÛÊ·ÈÚÈÓ·ÈÌ›·˜ Î·È ÛÙÔ˘˜ ‰‡Ô ·ÛıÂÓ›˜ Â›Ó·È ÈÛ¯˘Ú‹ ¤Ó‰ÂÈÍË ·ÔÎÏÂÈÛÌÔ‡ Ù˘ ‰È¿ÁÓˆÛ˘ Ù˘ º§À¡ (2). ∂ÓÙÔ‡ÙÔȘ, Ë ÌÂÚÈ΋ ·ÓÙ·fiÎÚÈÛË ÛÙË ıÂڷ›· Î·È ÔÈ Â·ÓÂÈÏËÌ̤Ó˜ ˘ÔÙÚÔ¤˜ Ù˘
Ó·
ÂΉËÏÒÓÔÓÙ·È ÓˆÚ›˜ ηٿ ÙËÓ ÂͤÏÈÍË Ù˘ Ófi-
ÛÔ˘, ÂÂȉ‹ fï˜ ÌÔÚ› Ó· ÌËÓ Â›Ó·È ÛÔ‚·Ú¤˜, ıˆÚÂ›Ù·È ÂÚÈÛÛfiÙÂÚÔ ‰È·ÁÓˆÛÙÈ΋ Ë ÚÔÔ-
‰Â˘ÙÈ΋ ÂÁηٿÛÙ·Û‹ ÙÔ˘˜, ·Ú¿ Ô ·fiÏ˘ÙÔ˜ ·ÚÈıÌfi˜ ÙˆÓ Î˘ÙÙ¿ÚˆÓ ÙÔ˘ ·›Ì·ÙÔ˜ (6). ™ÙÔÓ
ÚÒÙÔ ·ÛıÂÓ‹, ÔÈ Â·ÓÂÈÏËÌ̤Ó˜ ÔÛÙÂÔÌ˘ÂÏÈ-
Τ˜ ‚ÈÔ„›Â˜ ‰ÂÓ ‹Ù·Ó ‰È·ÁÓˆÛÙÈΤ˜ Î·È Ë ‰È¿-
ÁÓˆÛË Ù¤ıËΠ̠ÛÏËÓÂÎÙÔÌ‹ Î·È ÈÛÙÔÏÔÁÈ΋
ÂͤٷÛË ÙÔ˘ ÛÏ‹Ó·. ™ÙË ‚È‚ÏÈÔÁÚ·Ê›· ·Ó·Ê¤-
ÚÂÙ·È fiÙÈ ÌfiÓÔ ÛÙÔ 1/3 ÙˆÓ ÂÚÈÙÒÛÂˆÓ ·ÓÈ-
¯Ó‡ÂÙ·È ·ÈÌÔÊ·ÁÔ΢ÙÙ¿ÚˆÛË ÛÙÔ ·Ú¯ÈÎfi ‰Â›ÁÌ· Ì˘ÂÏÔ‡ (3). ™ÙÔÓ ÚÒÙÔ ·ÛıÂÓ‹, Ë ˘Ô˚Óˆ‰ÔÁÔÓ·ÈÌ›· Î·È Ë ˘ÂÚÙÚÈÁÏ˘ÎÂÚȉ·ÈÌ›·, Ô˘ ·ÔÙÂÏÔ‡Ó Ù· Û˘ÓËı¤ÛÙÂÚ· ·fiÓÙ· ÎÚÈÙ‹ÚÈ· (1), ÙÂÎÌËÚÈÒıËÎ·Ó ·ÚÎÂÙ¤˜ Ë̤Ú˜ ÌÂÙ¿ ÙËÓ ÂÌÊ¿ÓÈÛË ÙˆÓ ÚÒÙˆÓ Û˘Ìو̿وÓ. ∂Í¿ÏÏÔ˘, ÛÙÔÓ ‰Â‡ÙÂÚÔ
Ë ¡∫ ‰Ú·ÛÙËÚÈfiÙËÙ· ·Ú¤ÌÂÈÓ ÂÏ·Ùو̤ÓË, fï˜ ÙÔ Â‡ÚËÌ· ·˘Ùfi ‰ÂÓ ÙÂÎÌËÚÈÒÓÂÈ
√∞™. ∏ ·Ó·ÛÙÚÔÊ‹ ÙÔ˘ ÏfiÁÔ˘ CD4+/CD8+ ÛÙÔ˘˜ ‰‡Ô ·ÛıÂÓ›˜ ·ÓÙ·Ó·ÎÏ¿ ÙËÓ ·‡ÍËÛË ÙˆÓ Î˘ÙÙ·ÚÔÙÔÍÈÎÒÓ CD8+ ¤Ó·ÓÙÈ ÙˆÓ ‚ÔËıËÙÈÎÒÓ CD4+ ÏÂÌÊÔ΢ÙÙ¿ÚˆÓ.
Ù˘ ÓfiÛÔ˘ ÙÔ˘ ÚÒÙÔ˘ ·ÛıÂÓÔ‡˜ (1,2).
ÙÂÎÌËÚÈÒıËΠÌÂ Û˘Ó‰˘·ÛÌfi ÔÚÔÏÔÁÈÎÔ‡
¤ÏÂÁ¯Ô˘ Î·È PCR ÛÙ· ·ÙÙ·Ú· ÙÔ˘ ÂÚÈÊÂÚÈÎÔ‡
·›Ì·ÙÔ˜. ∏ ÔÚÔÏÔÁÈ΋ ·Ó›¯Ó¢ÛË ÙÔ˘ ÈÔ‡ ‰ÂÓ
Â›Ó·È ¿ÓÙ· ·Ú΋˜ Î·È Ë ÌÔÚȷ΋ ·Ó¿Ï˘ÛË ÌÂ
PCR ıˆÚÂ›Ù·È ·Ó·Áη›· (2).
∆Ô ÂÂÈÛfi‰ÈÔ Û·ÛÌÒÓ Î·È Ô Ï‹ı·ÚÁÔ˜ Ô˘
·ÚÔ˘Û›·ÛÂ Ô ÚÒÙÔ˜ ·ÛıÂÓ‹˜, ·ÔÙÂÏÔ‡Ó ÂÓ-
‰Â›ÍÂȘ ÚÔÛ‚ÔÏ‹˜ ÙÔ˘ ∫¡™, ·Ú¿ ÙÔ ÁÂÁÔÓfi˜
fiÙÈ Ù· Â˘Ú‹Ì·Ù· ·fi ÙËÓ √¡¶ Î·È ÙÔÓ ·ÂÈÎÔ-
ÓÈÛÙÈÎfi ¤ÏÂÁ¯Ô ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈο. ∏ ÓfiÛÔ˜ ÙÔ˘
∫¡™ Â›Ó·È Û˘¯Ó‹ (7) Î·È ÌÔÚ› Ó· ÂΉËψı›
Ì ‰È¤ÁÂÚÛË, ·˘¯ÂÓÈ΋ ‰˘Ûη̄›·, ·Ù·Í›·, ·-
Ú¿Ï˘ÛË ÂÁÎÂÊ·ÏÈÎÒÓ Û˘˙˘ÁÈÒÓ, ÂÓ‰ÔÎÚ¿ÓÈ·
˘¤ÚÙ·ÛË Î·È ÎÒÌ·. ¢ÂÓ ·ÔÙÂÏ› ‰˘ÛÌÂÓ‹
ÚÔÁÓˆÛÙÈÎfi ·Ú¿ÁÔÓÙ· Û ÌÂÁ¿Ï˜ ÛÂÈÚ¤˜ ·ÛıÂÓÒÓ (8), ÂÊfiÛÔÓ ÂÈÙ¢¯ı› ‡ÊÂÛË Î·È Á›-
ÓÂÈ ·ÏÏÔÁÂÓ‹˜ ªª√. µ·ÛÈÎfi ÚfiÏÔ ÛÙËÓ ·ıÔÁ¤ÓÂÛË ÙÔ˘ ∞º™
·›˙Ô˘Ó Ù· ΢ÙÙ·ÚÔÙÔÍÈο ∆- Î·È ¡∫-ÏÂÌÊÔ·ÙÙ·Ú·. º˘ÛÈÔÏÔÁÈο, Ù· ·ÙÙ·Ú· ·˘Ù¿ ÚÔηÏÔ‡Ó ÙËÓ ·fiÙˆÛË ¿ÏÏˆÓ ‰˘ÓËÙÈο ÂÈΛÓ-
∏ ÌË ÂÏÂÁ¯fiÌÂÓË ÂÓÂÚÁÔÔ›ËÛË ÙˆÓ ∆-ÏÂÌÊÔ΢ÙÙ¿ÚˆÓ Î·È Ë Â·ÎfiÏÔ˘ıË ÂÓÂÚÁÔÔ›ËÛË ÙˆÓ Ì·ÎÚÔÊ¿ÁˆÓ Ô‰ËÁ› Û ˘ÂÚ·Ú·ÁˆÁ‹ ΢ÙÙ·ÚÔÎÈÓÒÓ (2). ∆· ›‰· ÙˆÓ πL-8, TNF-· Î·È sCD-25 ÛÙÔÓ ÔÚfi, Ô˘ ·ÓÙ·Ó·ÎÏÔ‡Ó ÙË ‰Ú·ÛÙËÚÈfiÙËÙ· ÙˆÓ Î˘ÙÙ·ÚÔÙÔÍÈÎÒÓ Î˘ÙÙ¿ÚˆÓ, ¤¯Ô˘Ó ‰È·ÁÓˆÛÙÈ΋ Î·È ÚÔÁÓˆÛÙÈ΋ ÛËÌ·Û›· (2,3). ∆· ›‰· ÙÔ˘ sCD25 ÛÙÔÓ ÔÚfi ÙˆÓ ‰‡Ô ·ÛıÂÓÒÓ ‹Ù·Ó ·˘ÍË̤ӷ, ·ÏÏ¿ Ôχ ¯·ÌËÏfiÙÂÚ· ·fi 10.000 U/ml, ÙÈÌ‹ Ô˘ ¯·Ú·ÎÙËÚ›˙ÂÈ ÙÔ EBV-∞º™ (10). ªÂÙ¿ ÙËÓ ¤Ó·ÚÍË Ù˘ ¯ËÌÂÈÔıÂڷ›·˜ ÛÙÔÓ ÚÒÙÔ ·ÛıÂÓ‹, Ù· ›‰· ÙÔ˘ sCD25 ·ÚÔ˘Û›·Û·Ó Ì›ˆÛË, ·ÏÏ¿ ·Ú¤ÌÂÈÓ·Ó ·ıÔÏÔÁÈο. ∂›Û˘, ·˘ÍË̤ӷ ‹Ù·Ó Ù· ›‰· ÙˆÓ IL-8 Î·È TNF-a ÛÙÔÓ ÚÒÙÔ ·ÛıÂÓ‹ Î·È TNF-a ÛÙÔÓ ‰Â‡ÙÂÚÔ, Â˘Ú‹Ì·Ù· Û˘Ì‚·Ù¿ Ì π∞™ (2). ¶·Ú¿ ÙÔ ÁÂÁÔÓfi˜ fiÙÈ Ù· ·ÔÙÂϤÛÌ·Ù· ·˘Ù¿ ‰ÂÓ Â›Ó·È ¿ÌÂÛ· ‰È·ı¤ÛÈÌ·, ¤¯Ô˘Ó ·Ó·‰ÚÔÌÈ΋ ·Í›· ÛÙËÓ ·Ó·ÁÓÒÚÈÛË Ù˘ ˘ÔΛÌÂÓ˘ ÓfiÛÔ˘ Î·È ÌÔÚ› Ó· Û˘Ì‚¿ÏÏÔ˘Ó ÌÂÏÏÔÓÙÈο ÛÙÔÓ Î·ıÔÚÈÛÌfi ıÂڷ¢ÙÈÎÒÓ ÛÙÚ·ÙËÁÈÎÒÓ.
¶·Ú¿ ÙËÓ ‡·ÚÍË ·˘ÛÙËÚÒÓ ‰È·ÁÓˆÛÙÈÎÒÓ ÎÚÈÙËÚ›ˆÓ, Ë ÓfiÛÔ˜ ·ÚÔ˘ÛÈ¿˙ÂÈ ÌÂÁ¿ÏË ÎÏÈÓÈ΋ ÂÙÂÚÔÁ¤ÓÂÈ·, ·fi ‹È˜ ·˘ÙÔÂÚÈÔÚÈ˙fiÌÂÓ˜ ÌÔÚʤ˜ ̤¯ÚÈ ·ÓıÂÎÙÈΤ˜ ı·Ó·ÙËÊfiÚ˜ ÂÚÈÙÒÛÂȘ. ∏ ıÓËÙfiÙËÙ· ·fi EBV-∞º™ Â›Ó·È ÛËÌ·ÓÙÈ΋ (ÔÛÔÛÙfi 20-40%) Î·È Â›Ó·È Â›Ù ÚÒÈÌË ÏfiÁˆ ¢ηÈÚÈ·ÎÒÓ ÏÔÈÌÒÍÂˆÓ Î·È ¢∂¶, ›Ù fi„ÈÌË Î˘Ú›ˆ˜ ÏfiÁˆ ·ÓıÂÎÙÈ΋˜ ÓfiÛÔ˘ Î·È ÙÔÍÈÎfiÙËÙ·˜ Ù˘ ıÂڷ›·˜ (11).
∏ ıÂڷ›· ÙÔ˘ EBV-∞º™ ·Ú¯Èο ÂÚÈÏ·Ì‚¿ÓÂÈ ˘ÔÛÙËÚÈÎÙÈ΋ ·ÁˆÁ‹ Î·È Û ‹È˜ ÂÚÈÙÒÛÂȘ ¯ÔÚ‹ÁËÛË ÂÓ‰ÔÊϤ‚È·˜ Á-ÛÊ·ÈÚ›Ó˘ Î·È ·Î˘ÎÏÔ‚›Ú˘, Ì ·ÌÊ›‚ÔÏË ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· (2,6). ∆· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ· ˘ÔÛÙËÚ›˙ÂÙ·È Ë ¤ÁηÈÚË ¯ÔÚ‹ÁËÛË ıÂڷ›·˜ Ì ‚¿ÛË ÙÔ ÚˆÙfiÎÔÏÏÔ HLH-94 (3). ∆Ô ÚˆÙfiÎÔÏÏÔ ÂÚÈÏ·Ì‚¿ÓÂÈ Û˘Ó‰˘·Ṳ̂ÓË ¯ÔÚ‹ÁËÛË ÂÙÔÔÛ›‰Ë˜, ‰ÂÍ·ÌÂı·˙fiÓ˘ Î·È ÚÔÛı‹ÎË Î˘ÎÏÔÛÔÚ›Ó˘ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ Ê¿Û˘ ‰È·Ù‹ÚËÛ˘ Ù˘ ‡ÊÂÛ˘ (∂ÈÎfiÓ· 1). ªÂÙ¿ ÙËÓ ÂÓÙ·ÙÈ΋ ¯ËÌÂÈÔıÂڷ›· ÂÊfi‰Ô˘, ‰È¿ÚÎÂÈ·˜ 8 ‚‰ÔÌ¿‰ˆÓ, ·ÔÊ·Û›˙ÂÙ·È Ë ÂÚ·ÈÙ¤Úˆ Û˘Ó¤¯ÈÛË ‹ ‰È·ÎÔ‹ Ù˘ ıÂڷ›·˜ ·Ó¿ÏÔÁ· Ì ÙËÓ ·ÓÙ·fiÎÚÈÛË (4). ∂ÓÙÔ‡ÙÔȘ, ·Ó·Ê¤ÚÂÙ·È ¤ˆ˜ Î·È 30% ˘ÔÙÚÔ‹ Ù˘ ÓfiÛÔ˘ Û ·ÛıÂÓ›˜ Ì ϋÚË
·ÓÙ·fiÎÚÈÛË Ô˘ ‰È¤ÎÔ„·Ó ÙÔ HLH-94 ÛÙȘ 8
‚‰ÔÌ¿‰Â˜ ıÂڷ›·˜ (12). ™Â ÂÚÈÙÒÛÂȘ ·Ó-
ıÂÎÙÈΤ˜ ÛÙÔ ÚˆÙfiÎÔÏÏÔ HLH-94 ÂÊ·ÚÌfi˙Ô-
ÓÙ·È ‰È¿ÊÔÚ· ¯ËÌÂÈÔıÂڷ¢ÙÈο Û¯‹Ì·Ù·
ÏÂÌʈ̿وÓ, ˆ˜ ıÂڷ›· ‰È¿ÛˆÛ˘ ̤¯ÚÈ Ó·
ÔÏÔÎÏËÚˆı› Ë ·Ó·˙‹ÙËÛË Û˘Ì‚·ÙÔ‡ ‰fiÙË ÁÈ·
·ÏÏÔÁÂÓ‹ ªª√, Ô˘ ·ÔÙÂÏ› Î·È ÙË ÚÈ˙È΋
ıÂڷ›· (2).
∞fiÏ˘Ù˜ ÂӉ›ÍÂȘ ÁÈ· ·ÏÏÔÁÂÓ‹ ªª√ ıˆÚÔ‡ÓÙ·È Ë º§À¡, ÙÔ √∞™, ÔÈ ·ÓıÂÎÙÈΤ˜ ÌÔÚʤ˜ EBV-∞º™ Ì ˘„ËÏÔ‡˜ Ù›ÙÏÔ˘˜ ·ÓÙÈۈ̿وÓ, ˘„ËÏfi ·ÚÈıÌfi ΢ÎÏÔÊÔÚÔ‡ÓÙˆÓ ¡∫ (>40% ÛÙÔ ÂÚÈÊÂÚÈÎfi ·›Ì·), Ë ·Ú·ÌÔÓ‹ ˘„ËÏÔ‡ ÈÈ-
ÎÔ‡ ÊÔÚÙ›Ô˘ Î·È Ë ÊÙˆ¯‹ ·ÓÙ·fiÎÚÈÛË ÛÙË ¯Ë-
ÌÂÈÔıÂڷ›· Ì ÂÙÔÔÛ›‰Ë (2). ™Â Ì›· ÚfiÛÊ·-
ÙË ÔÏ˘ÎÂÓÙÚÈ΋ ÌÂϤÙË 40 ·ÛıÂÓÒÓ, Ë ÙÚÈÂÙ‹˜
ÂÈ‚›ˆÛË ÌÂÙ¿ ·ÏÏÔÁÂÓ‹ ªª√ ‹Ù·Ó 62% (4).
™ÙËÓ ÂÚ›ÙˆÛË ÙÔ˘ ÚÒÙÔ˘ ·ÛıÂÓÔ‡˜ ÌÂÛÔÏ¿-
‚ËÛ ÌÂÁ¿ÏÔ ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ· ̤¯ÚÈ ÙË ÌÂÙ·-
ÌfiÛ¯Â˘ÛË, ‰Â‰Ô̤ÓÔ˘ fiÙÈ ‰ÂÓ ˘‹Ú¯Â ‰È·ı¤ÛÈÌÔ˜ Û˘Ì‚·Ùfi˜ ‰fiÙ˘. ∏ ÌÂÁ¿ÏË Î·ı˘ÛÙ¤ÚËÛË
Î·È Ë ·ıÚÔÈÛÙÈ΋ ÙÔÍÈÎfiÙËÙ· ·fi ÙË ¯ÔÚËÁÔ‡ÌÂÓË ¯ËÌÂÈÔıÂڷ›·, ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÙË ÌÂÚÈ΋
Û˘Ì‚·ÙfiÙËÙ· ÙÔ˘ ÌÔۯ‡̷ÙÔ˜, Û˘ÓÂÙ¤ÏÂÛ·Ó
ÛÙÔ ÌÔÈÚ·›Ô ·ÔÙ¤ÏÂÛÌ·. ∞ÓÙ›ıÂÙ·, Ë ¤ÁηÈÚË
¤Ó·ÚÍË ıÂڷ›·˜ ÛÙÔÓ ‰Â‡ÙÂÚÔ ·ÛıÂÓ‹, ηıÒ˜
˘‹Ú¯Â Î·È ÌÂÁ·Ï‡ÙÂÚÔ˜ ‚·ıÌfi˜ ÎÏÈÓÈ΋˜ ˘Ô„›·˜, Û˘ÓÂÙ¤ÏÂÛ ÛÙËÓ ÂÈÙ˘¯‹ ¤Î‚·ÛË.
™˘ÌÂÚ·ÛÌ·ÙÈο, ÙÔ ·ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈÎfi
Û‡Ó‰ÚÔÌÔ Â›Ó·È ÎÏÈÓÈο Î·È ÁÂÓÂÙÈο ÂÙÂÚÔÁÂÓ‹˜ ÓfiÛÔ˜, ‰˘ÓËÙÈο ı·Ó·ÙËÊfiÚ·, Ô˘ Û˘¯Ó¿ Û˘Ó‰¤ÂÙ·È Ì Ïԛ̈ÍË ·fi ÙÔÓ EBV ηٿ ÙËÓ
·È‰È΋ ËÏÈΛ·. √ ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ ΢ÙÙ·ÚÔÎÈÓÒÓ, ·ÓÔÛÔÊ·ÈÓÔÙ‡Ô˘, ‚ÈÔ¯ËÌÈÎÒÓ ‰ÂÈÎÙÒÓ, ¡∫ ‰Ú·ÛÙËÚÈfiÙËÙ·˜ Î·È ÁÔÓȉȷ΋˜ ·Ó¿Ï˘Û˘ ¤¯ÂÈ ÚÔÁÓˆÛÙÈ΋ ·Í›· Î·È ÌÔÚ› Ó· ηıÔÚ›ÛÂÈ ÙË ıÂڷ›·. ∏ ¤ÁηÈÚË ¤Ó·ÚÍË ıÂڷ›·˜ ‚ÂÏÙÈÒÓÂÈ ÛËÌ·ÓÙÈο ÙËÓ ÚfiÁÓˆÛË, ÂÓÒ Û ÛÔ‚·Ú¤˜ Î·È ·ÓıÂÎÙÈΤ˜ ÂÚÈÙÒÛÂȘ ‰ÈÂÓÂÚÁÂ›Ù·È ·ÏÏÔÁÂÓ‹˜ ÌÂÙ·ÌfiÛ¯Â˘ÛË ·Ú¯¤ÁÔÓˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ.
∂˘¯·ÚÈÛٛ˜
∂˘¯·ÚÈÛÙԇ̠ıÂÚÌ¿ ÙËÓ Î·ıËÁ‹ÙÚÈ· G. JankaSchaub ÛÙÔ ¶·ÓÂÈÛÙËÌÈ·Îfi ¡ÔÛÔÎÔÌÂ›Ô ÙÔ˘ ∞Ì‚Ô‡ÚÁÔ˘ Ù˘ °ÂÚÌ·Ó›·˜, Ô˘ ·Ó¤Ï·‚ ÙË ÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡
ÙÔ˘ ÚÒÙÔ˘ ·ÛıÂÓÔ‡˜, ηıÒ˜ Î·È ÙË
Dr. E. Marion Schneider ÛÙÔ ∆Ì‹Ì· Ù˘ ¶ÂÈÚ·Ì·ÙÈ΋˜
∞Ó·ÈÛıËÛÈÔÏÔÁ›·˜
µÈ‚ÏÈÔÁÚ·Ê›·
1.Imashuku S. Differential diagnosis of hemophagocytic syndrome: underlying disorders and selection of the most effective treatment. Int J Hematol 1997; 66:135-151.
2.Imashuku S. Clinical features and treatment strategies of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis. Crit Rev Oncol Hematol 2002;44:259-272.
3.Janka GE, Schneider EM. Modern management of children with haemophagocytic lymphohistiocytosis. Br J Haematol 2004;124:4-14.
4.Henter JI, Samuelsson-Horne A, Arico M, Egeler RM, Elinder G, Filipovich A∏ et al. Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation. Blood 2002;100:2367-2373.
5. Arico M, Imashuku S, Clementi R, Hibi S, Teramura T, Danesino C et al. Hemophagocytic lymphohistiocytosis due to germline mutations in SH2D1A, the Xlinked lymphoproliferative disease gene. Blood 2001;97:1131-1133.
6.Tsuda H. Hemophagocytic syndrome (HPS) in children and adults. Int J Hematol 1997;65:215-226.
7.Haddad E, Sulis ML, Jabado N, Blanche S, Fischer A, Tardieu M. Frequency and severity of central nervous system lesions in hemophagocytic lymphohistiocytosis. Blood 1997;89:794-800.
8.Baker KS, DeLaat CA, Steinbuch M, Gross TG, Shapiro RS, Loechelt B et al. Successful correction of hemophagocytic lymphohistiocytosis with related or unrelated bone marrow transplantation. Blood 1997;89:3857-3863.
9. Filipovich AH. Hemophagocytic lymphohistiocytosis. Immunol Allergy Clin North Am 2002;22:281-300.
10.Imashuku S, Hibi S, Sako M, Ishida Y, Mugishima H, Chen J et al. Soluble interleukin-2 receptor: a useful prognostic factor for patients with hemophagocytic lymphohistiocytosis. Blood 1995;86:4706-4707.
11. Imashuku S, Teramura T, Tauchi H, Ishida Y, Otoh Y, Sawada M et al. Longitudinal follow-up of patients with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis. Haematologica 2004;89:183-188.
12.Imashuku S. Advances in the management of hemophagocytic lymphohistiocytosis. Int J Hematol 2000; 72:1-11.
B’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋,
µÂÓÈ˙¤ÏÂÈÔ °ÂÓÈÎfi
¡ÔÛÔÎÔÌ›Ô, ∏Ú¿ÎÏÂÈÔ
∫Ú‹Ù˘
AÏÏËÏÔÁÚ·Ê›·:
∞. ∆ÛÈÏÈÌÈÁοÎË
∏ÚÔ‰fiÙÔ˘ 214, T.K. 716 01
¡¤· ∞ÏÈηÚÓ·ÛÛfi˜,
∏Ú¿ÎÏÂÈÔ, ∫Ú‹Ù˘
E-mail: tsilimig@in.gr
∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 15-02-2005
∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 14-12-2005
2nd Paediatric Clinic, Venizeleio General Hospital, Herakleion, Crete
Correspondence: A. Tsilimigaki 214, Herodotou Str. 716 01, Herakleion, Crete, Greece E-mail: tsilimig@in.gr
Date of submission: 15-02-2005
Date of approval: 14-12-2005
¶. ¡ÂÔÓ¿ÎË, ™. ª¿Ú·Î·, ™. ™ÙÂÊ·Ó¿ÎË, ∞. ∆ÛÈÏÈÌÈÁοÎË
¶ÂÚ›ÏË„Ë: ¶ÂÚÈÁÚ¿ÊÂÙ·È ÂÚ›ÙˆÛË ·ÁÔÚÈÔ‡ ËÏÈΛ·˜ 14 ÂÙÒÓ Ô˘ ÓÔÛËχÙËΠÛÙËÓ ÎÏÈÓÈ΋ Ì·˜ ÁÈ· ¿Ù˘Ë Ó¢ÌÔÓ›·. √ ·ÛıÂÓ‹˜, ÙËÓ ¤ÌÙË Ë̤ڷ Ù˘ ÓÔÛËÏ›·˜ ÙÔ˘, ÂÌÊ¿ÓÈÛ ÎÏÈÓÈο Î·È ÂÚÁ·ÛÙËÚȷο Â˘Ú‹Ì·Ù· ÔÍ›·˜ ·ÁÎÚ·ٛÙȉ·˜. ∞fi ÙÔÓ ÂÚÁ·ÛÙËÚÈ·Îfi ¤ÏÂÁ¯Ô, ÙÔ Ì˘ÎfiÏ·ÛÌ· Ù˘ Ó¢ÌÔÓ›·˜ ·Ô‰Â›¯ÙËΠˆ˜ o ·ÈÙÈÔÏÔÁÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ Ù˘ Ïԛ̈͢. ∏ ÔÍ›· ·ÁÎÚ·ٛÙȉ· ·ÔÙÂÏ› Û¿ÓÈ· Â͈Ó¢ÌÔÓÈ΋ ÂΉ‹ÏˆÛË Ù˘ Ïԛ̈͢ ·fi Ì˘ÎfiÏ·ÛÌ· Ù˘ Ó¢ÌÔÓ›·˜. ªfiÓÔ Ï›Á˜ ÙÂÎÌËÚȈ̤Ó˜ ÂÚÈÙÒÛÂȘ ÔÍ›·˜
Acute pancreatitis in a child with pneumonia due to Mycoplasma pneumoniae
P. Neonaki, S. Maraka, S. Stefanaki, ∞. Tsilimigaki
Abstract: The case is reported of a 14 year-old boy who was admitted with atypical pneumonia. He developed the symptoms, signs and laboratory findings of acute pancreatitis on the fifth day of his hospitalization. Laboratory examination demonstrated positive Mycoplasma pneumoniae serology. Acute pancreatitis is a very unusual non-respiratory manifestation of Mycoplasma pneumoniae. Only a few cases of acute pancreatitis associated with Mycoplasma pneumoniae have been reported in the international literature and this is the first to be described in the Greek literature.
Key words: Atypical pneumonia, acute pancreatitis, Mycoplasma pneumoniae.
ÙÂÏ› ÙËÓ ÈÔ Û˘¯Ó‹ ÂΉ‹ÏˆÛË Ù˘ Ïԛ̈͢
Î·È Î·Ï‡ÙÂÈ Ì¤¯ÚÈ Î·È ÙÔ 20% ÙˆÓ ÂÚÈÙÒÛÂˆÓ Ó¢ÌÔÓ›·˜ Ù˘ ÎÔÈÓfiÙËÙ·˜ ÛÙÔÓ ÁÂÓÈÎfi
ÏËı˘ÛÌfi (1,2). ∏ ÎÏÈÓÈ΋ ÔÚ›· Ù˘ Ó¢ÌÔÓ›·˜ Â›Ó·È Û˘Ó‹ıˆ˜ ‹È·, ·ÏÏ¿ ¤¯Ô˘Ó ·Ó·ÊÂÚı› ÛÔ‚·Ú¤˜ ÂÈÏÔΤ˜ Ô˘ ·˘Í¿ÓÔ˘Ó ÙË ÓÔÛËÚfiÙËÙ· Î·È ÙË ıÓËÙfiÙËÙ· ÙˆÓ ·ÛıÂÓÒÓ (1). √È
‰‡ÛÎÔÏË Î·È ÂÈÙ˘Á¯¿ÓÂÙ·È ÌfiÓÔ Ì ÔÚÔÏÔÁÈÎfi ¤ÏÂÁ¯Ô. ™ÙȘ ÂΉËÏÒÛÂȘ ·fi ÙÔ Á·ÛÙÚÂÓÙÂÚÈÎfi Û‡ÛÙËÌ· ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È ¤ÌÂÙÔÈ, ‰È¿ÚÚÔȘ, ÎÔÈÏȷο ¿ÏÁË, ÂÓÒ Û¿ÓÈ· ÌÔÚ› Ó· ÂÌÊ·ÓÈÛÙ› Ë·ÙÈ΋ ‹ ·ÁÎÚ·ÙÈ΋ ‰˘ÛÏÂÈÙÔ˘ÚÁ›· ‹ ·ÈÌÔÚÚ·Á›· ·fi ÙÔ ÂÙÈÎfi (3,4). ¶ÂÚÈÁÚ¿ÊÂÙ·È Ë ÂÚ›ÙˆÛË ·ÁÔÚÈÔ‡ 14 ÂÙÒÓ Ì ÂÈÎfiÓ· ÔÍ›·˜ ·ÁÎÚ·ٛÙȉ·˜ ÛÙË ‰È·‰ÚÔÌ‹ Ïԛ̈͢ ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡, Ô˘ ·Ô‰Â›¯ÙËΠÔÚÔÏÔÁÈο, fiÙÈ ÔÊÂÈÏfiÙ·Ó ÛÙÔ Ì˘ÎfiÏ·ÛÌ· Ù˘ Ó¢ÌoÓ›·˜.
¶ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘
∞ÁfiÚÈ ËÏÈΛ·˜ 14 ÂÙÒÓ ÂÈÛ‹¯ıË ÛÙËÓ ÎÏÈÓÈ΋ Ì·˜ ÌÂ
¤ˆ˜ 39ÔC ·fi ÂÓıË̤ÚÔ˘, ‚‹¯· Î·È ÎÔÈÏÈ·Îfi ¿ÏÁÔ˜ ·fi ‰ÈË̤ÚÔ˘. ∆Ô ·ÙÔÌÈÎfi Î·È ÙÔ ÔÈÎÔÁÂÓÂÈ·Îfi ·Ó·ÌÓËÛÙÈÎfi ·Ó·Ê¤ÚÔÓÙ·È
ÂχıÂÚ·. √ ·ÛıÂÓ‹˜ ‹Ù·Ó Û ηϋ ÁÂÓÈ΋ ηٿÛÙ·ÛË Î·È ·fi ÙËÓ ·ÓÙÈÎÂÈÌÂÓÈ΋ ÂͤٷÛË
ÙÔ˘ ıÒڷη ‰È·ÈÛÙÒıËÎ·Ó ˘ÁÚÔ› ÚfiÁ¯ÔÈ ÂÓÙÔ-
ÈṲ̂ÓÔÈ ÛÙË ‚¿ÛË ·ÚÈÛÙÂÚ¿ Î·È ‹È·, ‰È¿¯˘ÙË
¢·ÈÛıËÛ›· ÛÙËÓ „ËÏ¿ÊËÛË Ù˘ ÎÔÈÏÈ¿˜. ∞fi Ù· ˘fiÏÔÈ· Û˘ÛÙ‹Ì·Ù· ‰ÂÓ ÚԤ΢„·Ó ·ıÔ-
ÏÔÁÈο Â˘Ú‹Ì·Ù·.
√ ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ ¤‰ÂÈÍÂ: WBC: 9580/mmÑ (¶ 59%, § 31%, ª 10%), Hb 13,0 gr/dl, Hct 38,5%, PLT 456.000/mmÑ, ∆∫∂ 73 mm/1Ë ÒÚ· Î·È CRP 4,96 mg/dl, ÁÏ˘Îfi˙Ë ÔÚÔ‡ 108 mg/dl. ∏ Ë·ÙÈ΋ Î·È Ë ÓÂÊÚÈ΋ ÏÂÈÙÔ˘ÚÁ›· ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈΤ˜, Ë ¿ÌÂÛË Coombs ıÂÙÈ΋ (+++) Î·È ÔÈ „˘¯ÚÔÛ˘ÁÎÔÏÏËÙ›Ó˜ 1/250 (ıÂÙÈΤ˜). ∏ ·ÎÙÈÓÔÁÚ·Ê›· ıÒÚ·ÎÔ˜ ·ÔÎ¿Ï˘„ ‡-
ÎÓˆÛË ÛÙË ‚¿ÛË ÙÔ˘ ·ÚÈÛÙÂÚÔ‡ Ó‡ÌÔÓ·. √
·ÛıÂÓ‹˜ ÂÙ¤ıË Û ·ÁˆÁ‹ Ì ÎÏ·Ú˘ıÚÔÌ˘Î›ÓË
·fi ÙÔ ÛÙfiÌ·.
∆Ô Ù¤Ù·ÚÙÔ 24ˆÚÔ Ù˘ ÓÔÛËÏ›·˜ ÙÔ˘, ÙÔ
·È‰› ÂÌÊ¿ÓÈÛ ¤ÓÙÔÓË ÂÈÁ·ÛÙÚ·ÏÁ›·, Â̤ÙÔ˘˜ Î·È ·ÓÔÚÂÍ›·, Ì ¢·ÈÛıËÛ›· ÛÙËÓ „ËÏ¿ÊËÛË
ÛÙÔ ÂÈÁ¿ÛÙÚÈÔ Î·È ÂÚÈÔÌÊ·ÏÈο. √ Ó¤Ô˜ ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ ¤‰ÂÈÍÂ: WBC 5.160/mmÑ (¶ 58%, § 32%, ª 10%), Hb 12,8 gr/dl, Hct 38,2%, PLT 393.000/mmÑ, TKE 55mm/1Ë ÒÚ·
Î·È CRP 2,46 mg/dl, ÁÏ˘Îfi˙Ë ÔÚÔ‡ 97 mg/dl. ∏ Ë·ÙÈ΋ Î·È Ë ÓÂÊÚÈ΋ ÏÂÈÙÔ˘ÚÁ›· ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈΤ˜, ·Ì˘Ï¿ÛË ÔÚÔ‡ 1287 U/L (º.∆.<180 U/L). √ ·ÈÌÔÚÚ·ÁÈÎfi˜ Î·È o ÏÈȉ·ÈÌÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÙÔ˘ ·ÛıÂÓÔ‡˜ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈÎÔ›. ∆Ô ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ÎÔÈÏ›·˜ ¤‰ÂÈÍ ÌÈÎÚ‹ Û˘ÏÏÔÁ‹ ˘ÊË·ÙÈο, ÂÚÈÛÏËÓÈο Î·È ÛÙËÓ ÂÚÈÔ¯‹
ÙÔ˘ ˘ÔÁ·ÛÙÚ›Ô˘. ∆· ·ÓÙÈÛÒÌ·Ù· ÁÈ· ¯Ï·Ì‡‰È·, ÚÈΤÙÛȘ, ·‰ÂÓÔ˚Ô‡˜, EµV, CMV, ÈÔ‡˜ Echo Î·È Coxsackie, ‹Ù·Ó ·ÚÓËÙÈο, ÂÓÒ ÁÈ· ÙÔ
Ì˘ÎfiÏ·ÛÌ· Ù˘ Ó¢ÌÔÓ›·˜ ‹Ù·Ó ıÂÙÈο: Igª 22 Î·È IgG 9,4. ªÂÙ¿ ·fi ‰‡Ô ‚‰ÔÌ¿‰Â˜ ‹Ù·Ó: IgM 23 Î·È IgG 38. °È· ÙËÓ ·Ó›¯Ó¢ÛË Î·È ÙÔÓ ÔÛÔÙÈÎfi ÚÔÛ‰ÈÔÚÈÛÌfi ÙˆÓ ·ÓÙÈÛˆÌ¿ÙˆÓ ¤Ó·ÓÙÈ ÙÔ˘ Ì˘ÎÔÏ¿ÛÌ·ÙÔ˜ Ù˘ Ó¢ÌÔÓ›·˜ ¯ÚËÛÈÌÔÔÈ‹ıËÎÂ Ë ÂÓ˙˘ÌÔ-·ÓÔÛÔ-ÚÔÛÚÔÊËÙÈ΋ ̤ıÔ‰Ô˜, fiÔ˘ Ù›ÙÏÔ˜ ÙˆÓ IgM ·ÓÙÈÛˆÌ¿ÙˆÓ ÌÂÁ·Ï‡ÙÂÚÔ˜ ÙÔ˘ 10 ıˆÚÂ›Ù·È ıÂÙÈÎfi˜. ªÂ ÙË ‰È¿ÁÓˆÛË Ù˘ ÔÍ›·˜ ·ÁÎÚ·ٛÙȉ·˜, ¤ÁÈÓ ‰È·ÎÔ‹ Ù˘ Û›ÙÈÛ˘, ÂÓ‰ÔÊϤ‚È· ¯ÔÚ‹ÁËÛË ˘ÁÚÒÓ Î·È Á·ÛÙÚÔÚÔÛÙ·Û›· Ì ÔÌÂÚ·˙fiÏË. ∂›Û˘ ÔÏÔÎÏËÚÒıËÎÂ Ë ·ÁˆÁ‹ Ì ÎÏ·Ú˘ıÚÔÌ˘Î›ÓË ÁÈ· 10 Ë̤Ú˜. ∏ ·Ì˘Ï¿ÛË ÙÔ˘ ·ÛıÂÓÔ‡˜ ‚ÂÏÙÈÒıËÎÂ Î·È ·ÔηٷÛÙ¿ıËΠÛÙ· Ê˘ÛÈÔÏÔÁÈο fiÚÈ· ¤ÓÙ Ë̤Ú˜ ·ÚÁfiÙÂ-
¤Ó· Û¯ÂÙÈο Û¿ÓÈÔ ÓfiÛËÌ· ÛÙ· ·È‰È¿. ™ÙÔ˘˜ ·ÈÙÈÔÏÔÁÈ-
ÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ Ù˘ ÔÍ›·˜ ·ÁÎÚ·ٛÙȉ·˜
ÛÙËÓ ·È‰È΋ ËÏÈΛ· ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È: ȉÈÔ·ı‹ ·›ÙÈ· (22%), ÙÚ·‡Ì· (20%), Ïԛ̈ÍË (15%), ÓfiÛÔ˜ ÙˆÓ ¯ÔÏËÊfiÚˆÓ (14%), Ê¿Ú̷η (13%), Û˘ÁÁÂÓ›˜ ·ÓˆÌ·Ï›Â˜ (5%), ‰È¿ÊÔÚ· ¿ÏÏ· ·›ÙÈ· (11%) (1,5).
∆Ô 1973, ÔÈ Leinikki et al ÂÚȤÁÚ·„·Ó ÚÒÙÔÈ ÂÚÈÙÒÛÂȘ ÔÍ›·˜ ·ÁÎÚ·ٛÙȉ·˜ Ô˘ Û¯ÂÙÈ˙fiÙ·Ó Ì ·‡ÍËÛË ÙÔ˘ Ù›ÙÏÔ˘ ·ÓÙÈÛˆÌ¿ÙˆÓ ¤Ó·ÓÙÈ ÙÔ˘ Ì˘ÎÔÏ¿ÛÌ·ÙÔ˜ Ù˘ Ó¢ÌÔÓ›·˜ (6). ¶·ÚfiÌÔÈ·
fiÙÈ ÙÔ 1/3
(7,8).
Ó¢ÌÔÓ›·˜ (1,4), ηıÒ˜ ηÈ
Ì ·‡ÍËÛË
·ÓÙÈÛˆÌ¿ÙˆÓ ¯ˆÚ›˜ Û˘ÌÙÒÌ·Ù·
ÙÔ ·Ó·Ó¢ÛÙÈÎfi (3). ™Â ‰È¿ÊÔÚ˜ ÌÂϤÙ˜ (4,7,8) ¤¯ÂÈ ÙÂı› ÙÔ ÂÚÒÙËÌ· Â¿Ó ·˘Ù‹ Ë ·‡ÍËÛË ÙˆÓ ·ÓÙÈÛˆÌ¿ÙˆÓ Û ·ÚÎÂÙ¤˜ ÂÚÈÙÒÛÂȘ ÔÍ›·˜ ·ÁÎÚ·ٛÙȉ·˜, ·Ô‰ÂÈÎÓ‡ÂÈ ÙËÓ ·ÈÙÈÔÏÔÁÈ΋ Û˘Û¯¤ÙÈÛË Ù˘ ÓfiÛÔ˘ Ì ÙË Ïԛ̈ÍË ·fi ÙÔ Ì˘ÎfiÏ·ÛÌ· Ù˘ Ó¢ÌÔÓ›·˜ ‹ Â¿Ó Ù· ·ÓÙÈÛÒÌ·Ù· ·˘Ù¿ Â›Ó·È ÌË ÂȉÈο Î·È ÂÌÊ·Ó›˙ÔÓÙ·È ˆ˜ ·¿ÓÙËÛË Û ¿ÏÏ· ·ÓÙÈÁfiÓ·, fiˆ˜ Û˘-
ÛÙ·ÙÈο ·ÁÎÚ·ÙÈÎÒÓ ÈÛÙÒÓ ‹ ˆ˜ ‰È·ÛÙ·˘ÚÔ‡ÌÂÓË ·ÓÙ›‰Ú·ÛË Û ¿ÏÏÔ Ì˘ÎfiÏ·ÛÌ· Ô˘
ÚÔηÏ› ÙË ÓfiÛÔ. øÛÙfiÛÔ, Ë ÂΉ‹ÏˆÛË ÔÍ›·˜
·ÁÎÚ·ٛÙȉ·˜ ÛÙË ‰È·‰ÚÔÌ‹ Ó¢ÌÔÓ›·˜, fiˆ˜ ÛÙËÓ ÂÚ›ÙˆÛË ÙÔ˘ ·ÛıÂÓÔ‡˜ Ì·˜, ηıÈÛÙ¿ ·›ı·ÓË ÙËÓ ˘fiıÂÛË fiÙÈ Ë ·‡ÍËÛË ÙˆÓ ÂÈ-
‰ÈÎÒÓ ·ÓÙÈÛˆÌ¿ÙˆÓ ÁÈ· ÙÔ Ì˘ÎfiÏ·ÛÌ· Ù˘
Ó¢ÌÔÓ›·˜, Â›Ó·È ÌË ÂȉÈ΋. √ ÌÈÎÚÔÔÚÁ·ÓÈÛÌfi˜ ‰ÂÓ ¤¯ÂÈ ·ÔÌÔÓˆı›
ÈÛÙfi Î·È Ë ·ÈÙÈÔÏÔÁÈ΋ ÙÔ˘ Û˘Û¯¤ÙÈÛË Ì ÙËÓ ÔÍ›· ·ÁÎÚ·ٛÙȉ· ‚·Û›˙Â-
ÔÊ›ÏÂÙ·È ÂÓ Ì¤ÚÂÈ ÛÙÔ Ôχ ÌÈÎÚfi ̤ÁÂıÔ˜ ÙÔ˘ ÌÈÎÚÔÔÚÁ·ÓÈÛÌÔ‡ (<350 Ìm Ì‹ÎÔ˜) Ô˘ ÙÔÓ Î·ıÈÛÙ¿ ÌË ÔÚ·Ùfi ÛÙÔ ÊˆÙÂÈÓfi ÌÈÎÚÔÛÎfiÈÔ. ∂ÈϤÔÓ, ·ÓÙ›ıÂÙ· Ì ٷ ¿ÏÏ· ›‰Ë
Ì˘ÎÔÏ¿ÛÌ·ÙÔ˜, ·Ó·Ù‡ÛÛÂÙ·È Ôχ ·ÚÁ¿ Î·È ··ÈÙÔ‡ÓÙ·È 1-3 ‚‰ÔÌ¿‰Â˜ ÁÈ· ÙË ‰ËÌÈÔ˘Ú-
Á›· ·ÔÈÎÈÒÓ (1). ∏ ̤ıÔ‰Ô˜ PCR, Ô˘ ¤¯ÂÈ
ÂÊ·ÚÌÔÛÙ› ÚfiÛÊ·Ù· Û ‰Â›ÁÌ·Ù· Ê·Ú˘ÁÁÈ-
ÎÔ‡ ÂȯڛÛÌ·ÙÔ˜, ı· ‹Ù·Ó ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙÂ-
ÚË ÁÈ· ¿ÌÂÛË Ù·˘ÙÔÔ›ËÛË ÙÔ˘ ÌÈÎÚÔÔÚÁ·ÓÈ-
ÛÌÔ‡. ¶·Ú¤¯ÂÈ ÁÚ‹ÁÔÚ· ·ÔÙÂϤÛÌ·Ù·, Û 2-5
Ë̤Ú˜, ÂÓÒ ·ÓȯÓ‡ÂÈ Î·È ÓÂÎÚÔ‡˜ ÌÈÎÚÔÔÚÁ·-
ÓÈÛÌÔ‡˜, Û ÂÚ›ÙˆÛË Ô˘ Ô ·ÛıÂÓ‹˜ ¤¯ÂÈ ·Ú-
¯›ÛÂÈ ıÂڷ›· (2). ∏ ‰È¿ÁÓˆÛË Ù˘ ÔÍ›·˜ ·ÁÎÚ·ٛÙȉ·˜ ‚·Û›˙ÂÙ·È ÛÙÔÓ Û˘Ó‰˘·ÛÌfi Ù˘ ÎÏÈÓÈ΋˜ ÂÈÎfiÓ·˜, ÙˆÓ ÂÚÁ·ÛÙËÚÈ·ÎÒÓ ÂÍÂÙ¿ÛÂˆÓ Î·È ÙÔ˘ ·ÂÈÎÔ-
ÓÈÛÙÈÎÔ‡ ÂϤÁ¯Ô˘. ™ÙËÓ ÂÚ›ÙˆÛ‹ Ì·˜ Ë ‰È¿-
ÁÓˆÛË ‚·Û›ÛÙËΠÛÙ· Û˘ÌÙÒÌ·Ù· ÙÔ˘ ·ÛıÂ-
ÓÔ‡˜ ·fi ÙÔ Á·ÛÙÚÂÓÙÂÚÈÎfi, ÛÙËÓ ·‡ÍËÛË Ù˘
¿Óˆ ·fi ÙÔ ÙÚÈÏ¿ÛÈÔ
·ÁÎÚ·ٛÙȉ·˜.
·ıÔÁÂÓÂÙÈÎfi Ì˯·ÓÈÛÌfi ÙÔ Ô›‰ËÌ· ÙÔ˘ ʇ̷-
ÙÔ˜ ÙÔ˘ Vater, ÏfiÁˆ Ù˘ Ïԛ̈͢ ·fi ÙÔ Ì˘-
ÎfiÏ·ÛÌ· Ù˘ Ó¢ÌÔÓ›·˜, Î·È ÙËÓ ·‡ÍËÛË Ù˘
Û˘ÁΤÓÙÚˆÛ˘ Ù˘ ·ÁÎÚ·ÙÈ΋˜ Ï˘ÛÔÏÂÎÈı›-
Ó˘ ÛÙÔ˘˜ ·ÁÎÚ·ÙÈÎÔ‡˜ fiÚÔ˘˜, Ë ÔÔ›· ¤¯ÂÈ
ÁÓˆÛÙ‹ ΢ÙÙ·ÚÔÙÔÍÈ΋ ‰Ú¿ÛË. ÕÏÏÔÈ ıˆÚÔ‡Ó
Èı·Ó‹ ÙËÓ ·Ú·ÁˆÁ‹ ·˘ÙÔ·ÓÙÈÛˆÌ¿ÙˆÓ Ô˘
·ÓÙȉÚÔ‡Ó Ì ¤Ó· ·ÁÎÚ·ÙÈÎfi ·ÓÙÈÁfiÓÔ, fiˆ˜
¤¯ÂÈ ÚÔÙ·ı› Î·È ÁÈ· ÂΉËÏÒÛÂȘ ·fi ¿ÏÏ· Û˘ÛÙ‹Ì·Ù· (1,2,4). √ ¿ÌÂÛÔ˜ ÂÓÔÊı·ÏÌÈÛÌfi˜ ÙˆÓ
ÌÈÎÚÔÔÚÁ·ÓÈÛÌÒÓ ÛÙÔ ÚÔۂ‚ÏË̤ÓÔ fiÚÁ·ÓÔ ıˆÚÂ›Ù·È Â›Û˘ Èı·Ófi˜ ·ıÔÁÂÓÂÙÈÎfi˜ Ì˯·ÓÈÛÌfi˜ (1,2).
™ÙȘ ÂÚÈÛÛfiÙÂÚ˜ ÂÚÈÙÒÛÂȘ, ÔÈ Â͈Ó¢ÌÔÓÈΤ˜ ÂΉËÏÒÛÂȘ ·ÎÔÏÔ˘ıÔ‡Ó Ù· Û˘ÌÙÒÌ·Ù· ·fi ÙÔ ·Ó·Ó¢ÛÙÈÎfi Î·È Ë ıÂڷ›· Ì ̷ÎÚÔÏ›‰Â˜ ‹ ÙÂÙڷ΢ÎÏ›ÓË ÂÏ¿¯ÈÛÙ· ÌÔÚ› Ó· ÂËÚ¿ÛÂÈ ÙËÓ ÔÚ›· Ù˘ Â͈Ó¢ÌoÓÈ΋˜ ÓfiÛÔ˘ (1). ™˘ÌÂÚ·ÛÌ·ÙÈο, fiÙ·Ó ÛÙË ‰È·‰ÚÔÌ‹ ¿Ù˘Ë˜ Ó¢ÌÔÓ›·˜ ÂÌÊ·ÓÈÛÙÔ‡Ó Û˘ÌÙÒÌ·Ù· ·fi ÙÔ Á·ÛÙÚÂÓÙÂÚÈÎfi, ÛÙË ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË Ú¤ÂÈ Ó· ÂÚÈÏ·Ì‚¿ÓÂÙ·È Ë ÔÍ›· ·ÁÎÚ·ٛÙȉ· ˆ˜ Â͈Ó¢ÌÔÓÈ΋ ÂΉ‹ÏˆÛË
µÈ‚ÏÈÔÁÚ·Ê›·
1.Al-Abasi ∞. Acute pancreatitis associated with Mycoplasma pneumoniae: a case report of missed diagnosis. Med Princ Pract 2002;11:112-115.
2.Berger RP, Wadowksy RM. Rhabdomyolysis associated with infection by Mycoplasma pneumoniae: a case report. Pediatrics 2000;105:433-436.
3.Handrick W, Braun W, Hofmann V, Senger H, Spencker FB. Mycoplasma etiology of acute pancreatitis. Z Gesamte Inn Med 1986;41:611-612.
4.Herbaut C, Tielemans C, Burette A, Dratwa M. Mycoplasma pneumoniae infection and acute pancreatitis. Acta Clin Belg 1983;38:186-188.
5.Pietzak MM, Thomas DW. Pancreatitis in childhood. Pediatr Rev 2000;21:406-441.
6.Leinikki PO, Panzar P, Tykka H. Antibody response in patients with acute pancreatitis to Mycoplasma pneumoniae. Scand J Gastroenterol 1973;8:631-635.
7.Leinikki OP, Panzar P, Tykka H. Immunoglobulin M antibody response against Mycoplasma pneumoniae lipid antigen in patients with acute pancreatitis. J Clin Microbiol 1978;8:113-118.
8.Freeman R, McMahon MJ. Acute pancreatitis and serological evidence of infection with Mycoplasma pneumoniae. Gut 1978;19:367-370.
Ì›· Û¿ÓÈ· ÎÏÈÓÈ΋ ÔÓÙfiÙËÙ·
Õ. ∆Û·ÏΛ‰Ë˜1, ™. °·Ú‰›Î˘2, ∏. ∫·Ú·¿ÓÙ˙Ô˜3, ∫. ∫·ÌÔ‡ÚË2, ™. ¢Â˘ÙÂÚ·›Ô˜4, ∞. °È·ÙÚÔ̷ӈϿÎË5, ∂. ™È‚Ú›‰Ë˜5, ∞. ÷Ù˙ËÌȯ·‹Ï1
¶ÂÚ›ÏË„Ë: ∆Ô ÏÈÔ‚Ï¿Ûو̷ Â›Ó·È ¤Ó·˜ Û¿ÓÈÔ˜ ηÏÔ‹ı˘ fiÁÎÔ˜ Ù˘ ·È‰È΋˜ ËÏÈΛ·˜, Ô ÔÔ›Ô˜ ÚÔ¤Ú¯ÂÙ·È ·fi ÂÌ‚Ú˘˚Îfi ϛԘ. ™ÙË ‰ÈÂıÓ‹ ‚È‚ÏÈÔÁÚ·Ê›· ¤¯Ô˘Ó ηٷÁÚ·Ê› ÌfiÓÔ 14 ÂÚÈÙÒÛÂȘ ÙÚ·¯ËÏÈÎÔ‡ ÏÈÔ‚Ï·ÛÙÒÌ·ÙÔ˜. ™ÙËÓ ·ÚÔ‡Û· ÂÚÁ·Û›· ·ÚÔ˘ÛÈ¿˙ÂÙ·È Ë ÂÚ›ÙˆÛË ‚Ú¤-
ÊÔ˘˜ Ì ÏÈÔ‚Ï¿Ûو̷ ÛÙËÓ ·˘¯ÂÓÈ΋ ¯ÒÚ·. ∫ÔÚ›ÙÛÈ ËÏÈΛ·˜ 9 ÌËÓÒÓ ·ÚÔ˘Û›·Û ̷Ïı·Î‹, ·ÓÒ-
‰˘ÓË Ì¿˙·, ηٿ ÙËÓ Ô›ÛıÈ· ÙÚ·¯ËÏÈ΋ ¯ÒÚ·. ∆Ô ‚Ú¤ÊÔ˜ ÚÔ¤‚Ë Û ¤ÏÂÁ¯Ô Ì US, CT Î·È MRI.
∏ ·ÛıÂÓ‹˜ ˘Ô‚Ï‹ıËΠ۠ϋÚË ÂÎÙÔÌ‹ Ù˘ Ì¿˙·˜. ∏ ÌÂÙÂÁ¯ÂÈÚËÙÈ΋ ÔÚ›· Ù˘ ·ÛıÂÓÔ‡˜
‰ÈfiÁΈÛ˘ ÛÙËÓ ÙÚ·¯ËÏÈ΋ ¯ÒÚ·, Ô˘ Ú¤ÂÈ fï˜ Ó· Û˘ÌÂÚÈÏ·Ì‚¿ÓÂÙ·È
ÂÎÏÔÁ‹˜ ·ÔÙÂÏ› Ë Ï‹Ú˘ ¯ÂÈÚÔ˘ÚÁÈ΋
Posterior cervical lipoblastoma: a rare neck mass in childhood
A. Tsalkidis1, S. Gardikis2, E. Karapantzos3, K. Kabouri2, S. Deftereos4, A. Giatromanolaki5, E. Sibridis5, A. Chatzimichael1
Abstract: Lipoblastoma is a rare benign childhood tumour derived from embryonic fat, and only 14 cases of cervical lipoblastoma have been documented. The case is reported of a healthy 9 month-old girl with a posterior cervical lipoblastoma, who presented with a firm, posterior cervical mass. After evaluation by US, CT and MRI the tumour was completely resected. Histological examination of the resected tissue showed the typical pattern of lipoblastoma. The child’s postoperative progress was uneventful and she remained in remission 22 months after surgery. Lipoblastoma must be included in the differential diagnosis of cervical masses in childhood. The treatment of choice is complete surgical resection, in order to prevent local recurrence.
Key words: Lipoblastoma, childhood, neck.
™˘ÓÙÔÌÔÁڷʛ˜
USÀÂÚ˯ÔÁÚ¿ÊËÌ·
CTÀÔÏÔÁÈÛÙÈ΋ ÙÔÌÔÁÚ·Ê›·
MRIª·ÁÓËÙÈÎfi˜ Û˘ÓÙÔÓÈÛÌfi˜
∂ÈÛ·ÁˆÁ‹
∆Ô ÏÈÔ‚Ï¿Ûو̷ Â›Ó·È ¤Ó·˜ ηÏÔ‹ı˘ ÌÂÛÂÁ¯˘Ì·ÙÈÎfi˜ fiÁÎÔ˜ ÙˆÓ ÂÌ‚Ú˘˚ÎÒÓ ÏÈÔÂȉÒÓ Î˘ÙÙ¿ÚˆÓ (1-3). ∆· ·ÁfiÚÈ· ÚÔÛ‚¿ÏÏÔÓÙ·È ÂÚÈÛÛfiÙÂÚÔ Û ۯ¤ÛË Ì ٷ ÎÔÚ›ÙÛÈ· Û ·Ó·ÏÔÁ›·
3:1 (1,3,4). ∏ Û˘¯ÓfiÙÂÚË ÂÓÙfiÈÛ‹ ÙÔ˘ Â›Ó·È ÛÙ· ¿ÎÚ· Î·È ÛÙÔÓ ÎÔÚÌfi ÙÔ˘ ÛÒÌ·ÙÔ˜ (1,2). ™¿ÓÈ· ·ÚÔ˘ÛÈ¿˙ÂÙ·È ÛÙËÓ ÂÚÈÔ¯‹ Ù˘ ÎÂÊ·-
Ï‹˜ Î·È ÙÔ˘ ÙÚ·¯‹ÏÔ˘ (2). ªfiÓÔ 14 ÂÚÈÙÒÛÂȘ ÏÈÔ‚Ï·ÛÙÒÌ·ÙÔ˜ ÛÙËÓ ÂÚÈÔ¯‹ ÙÔ˘ ÙÚ·¯‹ÏÔ˘ ¤¯Ô˘Ó ÂÚÈÁÚ·Ê› ÛÙË ‰ÈÂıÓ‹ ‚È‚ÏÈÔÁÚ·Ê›· (4). ∏ Ï‹Ú˘ ¯ÂÈÚÔ˘ÚÁÈ΋ ·Ê·›ÚÂÛË Ù˘ Ì¿˙·˜ ·ÔÙÂÏ› ÙË ıÂڷ›· ÂÎÏÔÁ‹˜ (1).
Peripheral General Hospital of Alexandroupolis
1 Paediatric Clinic of Democritus University of Thrace
2 Department of Paediatric Surgery of Democritus University of Thrace
3 Otorhinolaryngology Clinic of Democritus University of Thrace
4 Department of Radiology
5 Department of Pathology of Democritus University of Thrace
Correspondence: Aggelos Tsalkidis 59, C. Paleologou Str. 681 00, Alexandroupolis E-mail: atsalkid@med.duth.gr
Date of submission: 17-05-2005
Date of approval: 19-05-2006
·ÚÔ˘Û›·˙ ÏÈÔ‚Ï¿Ûو̷ ÛÙËÓ Ô›ÛıÈ· ÙÚ·-
¯ËÏÈ΋ ¯ÒÚ·, ·ÊÂÓfi˜ ÏfiÁˆ Ù˘ ÂÍ·ÈÚÂÙÈ΋˜ Û·ÓÈfiÙËÙ·˜ Ù˘ ÂÓÙfiÈÛ‹˜ ÙÔ˘ Î·È ·ÊÂÙ¤ÚÔ˘
ÚÔÎÂÈ̤ÓÔ˘ Ó· ÂÈÛËÌ·Óı› fiÙÈ Ú¤ÂÈ Ó· ηıÔÚ›˙ÂÙ·È Ë ·ÎÚÈ‚‹˜ ʇÛË Ù˘ ‰ÈfiÁΈÛ˘ ·Ó·ÙÔÌÈο Î·È ÈÛÙÔ·ıÔÏÔÁÈο, ÒÛÙ ӷ ÂÈϤÁÂÙ·È Ë Î·Ù¿ÏÏËÏË ıÂڷ›·.
¶ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘
¶ÚfiÎÂÈÙ·È ÁÈ· ÎÔÚ›ÙÛÈ, ÙÔ ÌÔÓ·‰ÈÎfi Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜, ÙÔ ÔÔ›Ô ÁÂÓÓ‹ıËΠÌÂÙ¿ ·fi Ê˘ÛÈÔÏÔÁÈ΋ ·ËÛË Î·È ÙÔÎÂÙfi, Ì ‚¿ÚÔ˜ Á¤ÓÓËÛ˘ 3260 g. ™Â ËÏÈΛ· 8 ÌËÓÒÓ ÔÈ ÁÔÓ›˜ ·ÓÙÈÏ‹ÊıËÎ·Ó ÙËÓ ‡·ÚÍË ‰ÈfiÁΈÛ˘ ηٿ ÙËÓ Ô›ÛıÈ· ÙÚ·¯ËÏÈ΋ ¯ÒÚ·, ¯ˆÚ›˜ ¿ÏÏË Û˘ÓÔ‰fi Û˘Ìو̷ÙÔÏÔÁ›·.
Ì ٷ ÙfiÍ· ÙˆÓ ·˘¯ÂÓÈÎÒÓ ÛÔÓ‰‡ÏˆÓ, Ì ˘ÎÓfiÙËÙ˜
ÙÔ˘ ÌË Ù˘ÈÎÔ‡ ÙˆÓ ·ÂÈÎÔÓÈÛÙÈÎÒÓ Â˘ÚËÌ¿ÙˆÓ Î·È ÁÈ· ÙÔÓ ·ÎÚÈ‚‹ ηıÔÚÈÛÌfi Ù˘ Û˘Û¯¤ÙÈÛ˘ Ù˘ Ì¿˙·˜ Ì ÙÔÓ ÛÔÓ‰˘ÏÈÎfi ۈϋӷ, ˘Ô‚Ï‹ıËΠ۠ÂÚ·ÈÙ¤Úˆ ¤ÏÂÁ¯Ô Ì ̷ÁÓËÙÈÎfi Û˘ÓÙÔÓÈÛÌfi (MRI). ¢È·ÈÛÙÒıËÎÂ Ë Û˘ÁÎÂÎÚÈ̤ÓË Ì¿˙·, ÂÂÎÙÂÈÓfiÌÂÓË ·fi ÙÔ ‡„Ô˜ ÙÔ˘ £1 ÛÔÓ‰‡ÏÔ˘ ̤¯ÚÈ ÙÔ ÈÓÈ·Îfi fiÁΈ̷ ‰È·ÛÙ¿ÛÂˆÓ 7 cm (ÂÁοÚÛÈ·) x 5 cm (ÎÂÊ·ÏÔ˘Ú·›·) x 3,5 cm (ÚÔÛıÈÔ›ÛıÈ·), ÌË Û¯ÂÙÈ˙fiÌÂÓË Ì ÙÔÓ ÛÔÓ‰˘ÏÈÎfi ۈϋӷ Î·È ÂÚÈ‚·ÏÏfiÌÂÓË ·fi ÈÓÒ‰Ë Î¿„· (∂ÈÎfiÓ· 2). ªÂ ÙÔÌ‹ ÂÁοÚÛÈ· Ô›ÛıÈ· ÙÚ·¯ËÏÈ΋, Ë Ì¿˙· ÂÍ·ÈÚ¤ıËΠÔÏÔÎÏËÚˆÙÈο (∂ÈÎfiÓ· 3). √È
Ú·ÁÌ·ÙÈΤ˜ ‰È·ÛÙ¿ÛÂȘ ÌÂÙ¿ ÙËÓ ÂÍ·›ÚÂÛ‹ Ù˘ ‹Ù·Ó 7x5x4 cm. ∏ ÈÛÙÔÏÔÁÈ΋ ÂͤٷÛË ÙÔ˘ ·Ú·Û΢¿ÛÌ·ÙÔ˜ ¤‰ÂÈÍ ηÏfiËı˜ ÏÈÔ‚Ï¿Ûو̷ (∂ÈÎfiÓ· 4). ∏ ÌÂÙÂÁ¯ÂÈÚËÙÈ΋ ÔÚ›· Ù˘ ·ÛıÂÓÔ‡˜ ˘‹ÚÍ ÔÌ·Ï‹ Î·È ÙÔ
4x3 cm. ∆Ô ˘ÂÚΛÌÂÓÔ ‰¤ÚÌ· ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈÎfi, ¯ˆÚ›˜ ÛÙÔȯ›· ÊÏÂÁÌÔÓ‹˜. ∏ ·Ú¯È΋ ÂͤٷÛË Ì ˘¤ÚË¯Ô˘˜ (US) ·Ó¤‰ÂÈÍ ÙËÓ ·ÚÔ˘Û›· ÔÌÔÈÔÁÂÓÔ‡˜ Ì¿˙·˜ Ì ˯ÔÁ¤ÓÂÈ· ÏÈÒ‰Ô˘˜
ÈÛÙÔ‡, ‰È·ÛÙ¿ÛÂˆÓ 4,4x3,8x2,1 cm. ªÂ ÙË ‰È¿ÁÓˆÛË ÙÔ˘ ÏÈÒÌ·ÙÔ˜ ÙÔ ·È‰› ¤ÛÙÚ„ ÛÙÔ
Û›ÙÈ ÙÔ˘ Î·È Û˘ÓÂÛÙ‹ıË Â·ÓÂͤٷÛË ÌÂÙ¿ 2 ‚‰ÔÌ¿‰Â˜. ∫·Ù¿ ÙËÓ Â·ÓÂͤٷÛË ‰È·ÈÛÙÒıËΠ¿ÏÈ Ë Ì¿˙· Ì ÙÔ˘˜ ›‰ÈÔ˘˜ ¯·Ú·ÎÙ‹Ú˜, ÏËÓ fï˜ ˘‹ÚÍ ·‡ÍËÛË ÙˆÓ ‰È·ÛÙ¿ÛÂÒÓ
Ù˘ (6x4 cm) (∂ÈÎfiÓ· 1). √ ·ӤÏÂÁ¯Ô˜ Ì US ÂȂ‚·›ˆÛ ÙËÓ ÎÏÈÓÈ΋ ÂÎÙ›ÌËÛË, Û‡Ìʈӷ Ì ÙÔÓ ÔÔ›Ô Ë Ì¿˙· ›¯Â ‰È·ÛÙ¿ÛÂȘ
6,7x4,6x3,3 cm. ∆o ÎÔÚ›ÙÛÈ ÂÈÛ‹¯ıË ÛÙÔ ÓÔÛÔÎÔÌÂ›Ô Î·È ˘Ô‚Ï‹ıËΠ۠ÂͤٷÛË Ì ˘ÔÏÔ-
ÁÈÛÙÈ΋ ÙÔÌÔÁÚ·Ê›· (CT) ·˘¯¤ÓÔ˜. ∞˘Ù‹ ·Ó¤‰ÂÈÍ ̿˙· ˘fi ÙÔ˘ ÙÚ·Â˙ÔÂȉԇ˜ Ì˘fi˜, ·ÚÈÛÙÂÚ¿ ηÈ
∆Ô 1986, ÔÈ Sandberg Î·È Û˘Ó ÂÚȤÁÚ·„·Ó ÏÈ-
Ô‚Ï¿Ûو̷ Û ·ÁfiÚÈ ËÏÈΛ·˜ 2 ÂÙÒÓ, Ô˘ ÂÌ-
Ê¿ÓÈ˙ ÌÂÙ¿ıÂÛË ÌÂٷ͇ ÙˆÓ ¯ÚˆÌÔÛˆÌ¿ÙˆÓ 7
Î·È 8 (10). ŒÎÙÔÙÂ, ¤¯Ô˘Ó ·Ó·ÊÂÚı› ·ÚÎÂÙ¤˜
¯ÚˆÌÔÛˆÌÈΤ˜ ·ÓˆÌ·Ï›Â˜ Ì ÎÔÈÓfi ¯·Ú·ÎÙËÚÈ-
ÛÙÈÎfi ÙË Û˘ÌÌÂÙÔ¯‹ ÛÙȘ ·ÓˆÌ·Ï›Â˜ ·˘Ù¤˜ ÙÔ˘
¯ÚˆÌÔÛÒÌ·ÙÔ˜ 8 (ÂÚÈÔ¯‹ 8q11-q13) ÛÙÔ ÏÈ-
Ô‚Ï¿Ûو̷ Î·È ÛÙË ÏÈÔ‚Ï·Ûو̿وÛË (11)
Î·È ÙË ÌÂÙ¿ıÂÛË t(2;16)(q13;p11) ÛÙÔ ‚ÏÂÓÓ҉˜ ÏÈÔÛ¿ÚΈ̷ (1). ∏ ΢ÙÙ·ÚÔÁÂÓÂÙÈ΋ Âͤ-
Ù·ÛË ÙˆÓ fiÁÎˆÓ ·Ú¤¯ÂÈ ÚfiÛıÂÙ˜ ¯Ú‹ÛÈ̘
ÏËÚÔÊÔڛ˜, Û˘Ì‚¿ÏÏÔÓÙ·˜ ÛÙËÓ Î·ÙËÁÔÚÈÔ-
Ô›ËÛË ÙˆÓ ÏȈ‰ÒÓ fiÁÎˆÓ Î·È ÛÙË ‰È·ÊÔÚÈ΋
‰È¿ÁÓˆÛË ÙÔ˘ ÏÈÔ‚Ï·ÛÙÒÌ·ÙÔ˜ ·fi ÙÔ ÏÈÔ-
Û¿ÚΈ̷. ™‡Ìʈӷ Ì ÙÔ˘˜ Miller Î·È Û˘Ó
Ú¤ÂÈ Ó· Ï·Ì‚¿ÓÂÙ·È ˘ÏÈÎfi ·fi
ÙȘ ·Ú·Î›ÌÂÓ˜ ·Ó·ÙÔÌÈΤ˜ ÂÚÈÔ¯¤˜, fiˆ˜ Û˘Ó¤‚Ë Î·È ÛÙËÓ ·ÚÔ‡Û·
¶·Ú’ fiÏ· ·˘Ù¿, ÂÚÈÁÚ¿ÊÔÓÙ·È ‰‡Ô ÂÚÈÙÒ-
ÛÂȘ ÏÈÔ‚Ï·ÛÙÒÌ·ÙÔ˜ ÙÚ·¯‹ÏÔ˘, fiÔ˘ ·Ó·Ê¤-
ÚÂÙ·È ‰È·ÏÂ›Ô˘Û· ·Ó¿ÚÎÂÈ· ·Ó·Ó¢ÛÙÈÎÒÓ
Ô‰ÒÓ (2,12) Î·È Ì›· ÂÚ›ÙˆÛË ËÌÈ¿ÚÂÛ˘,
ÔÊÂÈÏfiÌÂÓË Û ÏÈÔ‚Ï·Ûو̿وÛË (12). ¢ÂÓ
·Ó·Ê¤ÚÔÓÙ·È ÂÚÈÙÒÛÂȘ Ì ÂͤÏÈÍË Û ηÎfiË-
ı˜ ÓÂfiÏ·ÛÌ· (13). ™Â ·ÚÎÂÙ¤˜ ÂÚÈÙÒÛÂȘ, Ë
·Ó¿Ù˘ÍË ÙÔ˘ ÏÈÔ‚Ï·ÛÙÒÌ·ÙÔ˜ Â›Ó·È ‰˘Ó·Ùfi
Ó· Ï¿‚ÂÈ ÙÂÚ¿ÛÙȘ ‰È·ÛÙ¿ÛÂȘ Î·È ÁÈ· ÙÔÓ ÏfiÁÔ
·˘Ùfi Ë ÔÏÈ΋ ¯ÂÈÚÔ˘ÚÁÈ΋ ·Ê·›ÚÂÛË ÙÔ˘ fiÁÎÔ˘
Â›Ó·È Ë Ì¤ıÔ‰Ô˜ ÂÎÏÔÁ‹˜ (13). øÛÙfiÛÔ, ˘¿Ú¯ÂÈ
·Ó·ÊÔÚ¿ ÛÙË ‚È‚ÏÈÔÁÚ·Ê›· fiÔ˘ ÂÚÈÁÚ¿ÊÂÙ·È ·˘ÙfiÌ·ÙË ˘Ô¯ÒÚËÛË Ù˘ ‚Ï¿‚˘ (13).
∏ ·ÓÂÌÊ¿ÓÈÛË ÙÔ˘ fiÁÎÔ˘ ÌÂÙ¿ ÙËÓ ÂÍ·›ÚÂÛ‹ ÙÔ˘ Â›Ó·È Û¿ÓÈ·
ÙÚ·¯ËÏÈ΋ ¯ÒÚ·, Ô˘ Ú¤ÂÈ fï˜ Ó· Û˘ÌÂÚÈÏ·Ì‚¿ÓÂÙ·È ÛÙË ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË. √ ˘„ËÏfi˜ ‚·ıÌfi˜ ˘Ô„›·˜ ÂÈÙÚ¤ÂÈ ÙÔÓ ¿ÌÂÛÔ Î·ıÔÚÈÛÌfi Ù˘ ʇÛ˘ Ù˘ ‰ÈfiÁΈÛ˘, ¤ÙÛÈ ÒÛÙÂ Ë ¯ÂÈÚÔ˘ÚÁÈ΋ ·Ê·›ÚÂÛË Ó· Ú·ÁÌ·ÙÔÔÈËı› Û ÚÒÈÌÔ ÛÙ¿‰ÈÔ, ‰ËÏ·‰‹ ÚÈÓ ÙÔ ÏÈÔ‚¿Ûو̷ ·˘ÍËı› Û ‰È·ÛÙ¿ÛÂȘ, ÁÂÁÔÓfi˜ Ô˘ Â›Ó·È Èı·Ófi Ó· ηٷÛÙ‹ÛÂÈ ÙËÓ ÂÍ·›ÚÂÛ‹ ÙÔ˘ ‰˘Û¯ÂÚ‹, ·˘Í¿ÓÔÓÙ·˜ ÙȘ Èı·ÓfiÙËÙ˜ ˘ÔÙÚÔ‹˜.
1.Harrer J, Hammon G, Wagner T, Bolkenius M. Lipoblastoma and lipoblastomatosis: a report of two cases and review of the literature. Eur J Pediatr Surg 2001;11:342-349.
2.Basaran UN, Inan M, Bilgi S, Pul M. Lipoblastoma: a rare cervical mass in childhood. Int J Pediatr Otorhinolaryngol 2001;61:265-268.
3.Chun YS, Kim WK, Park KW, Lee SC, Jung SE. Lipoblastoma. J Pediatr Surg 2001;36:905-907.
4.Ratan SK, Gambhir A, Mullick S, Ratan J. Lipoblastoma of the neck. Indian J Pediatr 2000;67:301-303.
5.Jaffe RH. Recurrent lipomatous tumors of the groin: liposarcoma and lipoma pseudomyxomatodes. Arch Pathol 1926;1:381-387.
6.Vellios F, Baez J, Shumacker HB. Lipoblastomatosis: a tumor of fetal fat different from hibernoma. Report of a case, with observations on the embryogenesis of human adipose tissue. Am J Pathol 1958;34: 1149-1159.
7.Shah AR, Wei JL, Maddalozzo J. Cervical lipoblastoma: an uncommon diagnosis of neck mass. Otolaryngol Head Neck Surg 2004;130:504-507.
8. Miller GG, Yanchar NL, Magee JF, Blair GK. Lipoblastoma and liposarcoma in children: an analysis of 9 cases and a review of the literature. Can J Surg 1998;41:455-458.
9.Mentzel T, Calonje E, Fletcher CD. Lipoblastoma and lipoblastomatosis: a clinicopathological study of 14 cases. Histopathology 1993;23:527-533.
10. Sandberg AA, Gibas Z, Saren E, Li FP, Limon J, Tebbi CK. Chromosome abnormalities in two benign adipose tumors. Cancer Genet Cytogenet 1986;22:55-61.
11.Fletcher CD, Akerman M, Dal Cin P, de Wever I, Mandahl N, Mertens F et al Correlation between clinicopathological features and karyotype in lipomatous tumors. A report of 178 cases from the Chromosomes and Morphology (CHAMP) Collaborative Study Group. Am J Pathol 1996;148:623-630.
12.Sun JJ, Rasgon BM, Hilsinger RL Jr. Lipoblastomatosis of the neck causing hemiparesis: a case report and review of the literature. Head Neck 2003;25:337-340.
13.Mognato G, Cecchetto G, Carli M, Talenti E, d’Amore ES, Pederzini F et al. Is surgical treatment of lipoblastoma always necessary? J Pediatr Surg 2000; 35:1511-1513.
¡ÙfiÈÓÁÎ Û ·È‰È¿, ÂÊ‹‚Ô˘˜
ηÈ
Ó·ÚÔ‡˜ ÂÓ‹ÏÈΘ
™. ∞ÓÙˆÓÈ¿‰Ë˜
¶ÂÚ›ÏË„Ë: ∆Ô ÓÙfiÈÓÁÎ ·ÔÙÂÏ› ¤Ó· ·fi Ù· ÚÔ‚Ï‹Ì·Ù· ‰ËÌfiÛÈ·˜ ˘Á›·˜, ÙÔ ÔÔ›Ô - Û ·ÓÙ›-
ıÂÛË Ì ¿ÏÏ·, fiˆ˜ .¯. ÙÔ Î¿ÓÈÛÌ·, Ù· Ó·ÚΈÙÈο Î.¿. - ¿ÚÁËÛ ӷ Á›ÓÂÈ ·ÓÙÈÏËÙfi. ∏ ¯Ú‹ÛË
‰È·ÊfiÚˆÓ Ô˘ÛÈÒÓ ÁÈ· ÙËÓ ·‡ÍËÛË Ù˘ ‰‡Ó·Ì˘, ÙËÓ ÂÓ›Û¯˘ÛË Ù˘ ·ÓÙÔ¯‹˜, ÙË ÌÂÁ·Ï‡ÙÂÚË ·ÓÔ¯‹
ÛÙÔÓ fiÓÔ Î.Ï. Â›Ó·È ÁÓˆÛÙ‹ ·fi ÙËÓ ·Ú¯·ÈfiÙËÙ·. ™ÙȘ ̤Ú˜ Ì·˜, fiˆ˜ Ê·›ÓÂÙ·È, ÙÔ ÓÙfiÈÓÁÎ
·ÔÙÂÏ› Û˘Ó‹ıË Ú·ÎÙÈ΋ ÛÙÔÓ “Âϛٔ ÚˆÙ·ıÏËÙÈÛÌfi, ÛÙÔÓ ¤ÓÙÔÓÔ ·ıÏËÙÈÛÌfi Î·È Û ·˘ÙÔ‡˜ Ô˘
·Û¯ÔÏÔ‡ÓÙ·È Ì ÙË ÛˆÌ·ÙÔ‰fiÌËÛË (body-building). ™ÙȘ ÎÔÈӈӛ˜ ÙˆÓ ÓÈÎËÙÒÓ Î·È ËÙÙË̤ӈÓ,
fiÔ˘ Ë ‰È¿ÎÚÈÛË Û ¤Ó· ¿ıÏËÌ· ÛËÌ·›ÓÂÈ ¯Ú‹Ì·, ‰fiÍ·, ·ÏÏ¿ Î·È ÔÈΛϷ ¿ÏÏ· ÔʤÏË (.¯. ÂÈÛ·Áˆ-
Á‹ ÛÙËÓ ÙÚÈÙÔ‚¿ıÌÈ· Âη›‰Â˘ÛË), Â›Ó·È ‰‡ÛÎÔÏÔ Ó· ·ÓÙÈÛÙ·ıÔ‡Ó ÔÈ Ó¤ÔÈ Î·È Ó· ·ÚÎÂÛÙÔ‡Ó ÌfiÓÔ
ÛÙȘ ‰˘Ó¿ÌÂȘ Ô˘ ÙÔ˘˜ ·Ú¤¯ÂÈ ÙÔ ÛÒÌ· ÙÔ˘˜. √È Ô˘Û›Â˜ Î·È ÔÈ Ì¤ıÔ‰ÔÈ Ô˘ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È Â›-
Ó·È ÔÏϤ˜ Î·È ÔÈΛϘ, ¿ÏϘ ÁÓˆÛÙ¤˜ ·fi ÙÔ ·ÚÂÏıfiÓ Î·È ¿ÏϘ ·ÔÙÂÏÔ‡Ó ÚfiÛÊ·Ù· ÂÈ-
Ù‡ÁÌ·Ù· Ù˘ ÂÈÛÙ‹Ì˘. √È ÂÈÙÒÛÂȘ ÂÍ·ÚÙÒÓÙ·È ·fi ÙË Û˘ÁÎÂÎÚÈ̤ÓË Ô˘Û›·, ÙË ‰fiÛË, ·ÏÏ¿
ÙÔ ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ· ÁÈ· ÙÔ ÔÔ›Ô ¯ÚËÛÈÌÔÔÈ›ٷÈ. ªÂٷ͇ ÙˆÓ ‰È·ÊfiÚˆÓ ÚÔ‚ÏËÌ¿ÙˆÓ ˘Á›-
‰ËÌÈÔ˘ÚÁÔ‡ÓÙ·È, ΢ڛ·Ú¯Ô ÚfiÏÔ ¤¯Ô˘Ó Ù· ηډÈÔÏÔÁÈο, ·fi Ù· ÔÔ›· Î·È ÚÔηÏÂ›Ù·È Ô
·ÚÈıÌfi˜ ÙˆÓ ı·Ó¿ÙˆÓ. øÛÙfiÛÔ, ˘¿Ú¯ÂÈ ¤ÏÂÁ¯Ô˜ ·ÓÙÈ-ÓÙfiÈÓÁÎ, ηıÒ˜ ‰È¿ÊÔÚ˜
ÂıÓÈΤ˜ Î·È ‰ÈÂıÓ›˜ ÂÈÙÚÔ¤˜ Ï·Ì‚¿ÓÔ˘Ó Ì¤ÙÚ· ÁÈ· ÙËÓ Î·Ù·ÔϤÌËÛË Ù˘ ¯Ú‹Û˘ ··ÁÔÚÂ˘Ì¤ÓˆÓ Ô˘ÛÈÒÓ ÛÙÔÓ ·ıÏËÙÈÛÌfi. ™Â fi,ÙÈ ·ÊÔÚ¿ Ù· ·È‰È¿, ÙÔ
ÚÔÛÔ¯‹. ∏ ¤̂·ÛË ÙˆÓ
Doping in children, adolescents and young adults
S. Antoniadis
Abstract: Doping is a public health problem which in contrast with others, such as smoking and drug abuse, was realized with some delay. The use of various performance enhancing substances to increase strength, endurance and tolerance to pain, has been observed since ancient times. Today doping appears to be a common practice among elite athletes, those playing sports at competition level and bodybuilders. In winner-loser societies, where distinction in sports means money, glory and other benefits such as entrance to higher education, it is very difficult for a young person to be convinced that his/her natural physical strength is enough. There is a long list of performance enhancing substances and methods some of which have been known for many years and others that represent recent scientific advances. The potential harmful effects depend on what is used and on the dosage and the length of the time of use. Among the various health problems that the athletes may develop, those involving the heart are more frequently related to death. For the prevention of doping there are national and international committees for anti-doping and lists of prohibited substances and methods. As far as children are concerned, doping does not appear to be a problem in the very young age group, but care needs to be taken for teenagers and young adults. The role of the paediatrician can obviously play a very significant role in the control of doping, which is the importance of this article.
Key words: Doping, anti-doping, children, adolescents, young adults.
Paediatric Cardiologist, Professor of the Highest Technological Educational Institution of Athens
Correspondence: Stilianos Antoniadis 4, Iraklitou Str. 106 73, Kolonaki
Date of submission: 24-11-2005
Date of approval: 28-03-2006
¶·È‰È·ÙÚÈ΋ 2006;69:317-322
∏ ¯Ú‹ÛË Ê·Ú̷΢ÙÈÎÒÓ ‹ ¿ÏÏˆÓ Ô˘ÛÈÒÓ
Ô˘ ‚ÔËıÔ‡Ó ÛÙËÓ ·‡ÍËÛË Ù˘ ·fi‰ÔÛ˘ η-
ıÒ˜ Î·È ÛÙË ‚ÂÏÙ›ˆÛË Ù˘ Â͈ÙÂÚÈ΋˜ ÂÌÊ¿ÓÈ-
Û˘ Â›Ó·È ÁÓˆÛÙ‹ Â‰Ò Î·È ÔÏϤ˜ ÂηÙÔÓÙ¿‰Â˜
¯ÚfiÓÈ· (1-3). ™ÙȘ ̤Ú˜ Ì·˜, Ì ÙËÓ ÂÌÔÚ¢-
Ì·ÙÔÔ›ËÛË ÙÔ˘ ·ıÏËÙÈÛÌÔ‡, fiÔ˘ Ë Ó›ÎË ÛË-
Ì·›ÓÂÈ ¯Ú‹Ì· Î·È ‰fiÍ·, Ê·›ÓÂÙ·È fiÙÈ Ë ·Ó·˙‹ÙË-
ÛË ıÂÌÈÙÒÓ ‹ ·ı¤ÌÈÙˆÓ ÌÂıfi‰ˆÓ Ô˘ ÌÔÚ› Ó·
ʤÚÔ˘Ó ÙË ‰È¿ÎÚÈÛË, ·ÔÙÂÏ› ÚÔÛÊÈÏ‹ Û˘Ó‹-
ıÂÈ· ÌÂٷ͇ ÙˆÓ ·ıÏËÙÒÓ ‹ ÙˆÓ Â›‰Ô͈Ó
ÚˆÙ·ıÏËÙÒÓ (4-6).
°È· ÙËÓ ÂÈÙ˘¯‹ ·ÓÙÈÌÂÙÒÈÛË ‹ ÙÔÓ ÂÚÈÔÚÈÛÌfi ÙÔ˘ ÓÙfiÈÓÁÎ, ÙÔ ÔÔ›Ô Ï¤ÔÓ ¤¯ÂÈ Ï¿‚ÂÈ ‰È·ÛÙ¿ÛÂȘ ÚÔ‚Ï‹Ì·ÙÔ˜ ‰ËÌfiÛÈ·˜ ˘Á›·˜ (7), Â›Ó·È ··Ú·›ÙËÙË
°ÂÓÈο, Ù· Ê¿Ú̷η Î·È ÔÈ Ì¤ıÔ‰ÔÈ Ô˘ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È Û‹ÌÂÚ·, ·ÏÏ¿ Î·È Ù· ¿ÏÏ· ̤۷ Ô˘ Èı·ÓÒ˜ ı· ¯ÚËÛÈÌÔÔÈËıÔ‡Ó ÛÙÔ Ì¤ÏÏÔÓ (11), ·ÔÙÂÏÔ‡Ó ÚfiÎÏËÛË ÁÈ· ÙÔ˘˜ ÂÈÛÙ‹ÌÔÓ˜. ∞ӷʤÚÔÓÙ·È Û˘ÓÔÙÈο ÛÙË Û˘Ó¤¯ÂÈ· ÔÈ Î˘ÚÈfiÙÂÚ˜ ηÙËÁÔڛ˜: ·) ¢ÈÂÁÂÚÙÈο: ηʽÓË, ÎÔη˝ÓË, ·ÌÊÂٷ̛Ә, ÂʉڛÓË, ÊÂÓÊÏÔ˘Ú·Ì›ÓË Î.Ï. ∞˘Í¿ÓÔ˘Ó ÙËÓ ·ÓÙÔ¯‹, ‰ËÌÈÔ˘ÚÁÔ‡Ó ·›ÛıËÌ· ·˘ÙÔÂÔ›ıËÛ˘ Î·È ÂÓÈÛ¯‡Ô˘Ó ÙËÓ ·˘ÙÔÛ˘ÁΤÓÙÚˆÛË. ™ÙȘ ·ÚÂÓ¤ÚÁÂȘ ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È: ‰È¤ÁÂÚÛË, ÙÚfiÌÔ˜, ·ÚÚ˘ı̛˜, ˘¤ÚÙ·ÛË Î.Ï. ™Â ·Ú·ÙÂٷ̤ÓË Î·È ˘ÂÚ‚ÔÏÈ΋ ¯Ú‹ÛË ÌÔÚ› Ó· ·Ú·ÙËÚËı› ·ÎfiÌË Î·È ı¿Ó·ÙÔ˜.
Ê·›ÓÂ-
Ù·È ·fi ÙË ‚È‚ÏÈÔÁÚ·Ê›·, ÙÔ ÓÙfiÈÓÁÎ Á›ÓÂÙ·È
ÛÙȘ ÂÚÈÛÛfiÙÂÚ˜ ÂÚÈÙÒÛÂȘ Ì ÙË Û˘Ì‚ÔÏ‹
ÙˆÓ ÁÈ·ÙÚÒÓ (Î·È ÙˆÓ Ê·ÚÌ·ÎÔÔÈÒÓ). §·Ì‚¿-
ÓÔÓÙ·˜, ÏÔÈfiÓ, ˘’ fi„ÈÓ ÙÔ Ì¤ÁÂıÔ˜ ÙÔ˘ ÚÔ‚Ï‹Ì·ÙÔ˜, ·ÏÏ¿ Î·È ıˆÚÒÓÙ·˜ fiÙÈ Ë ·ÔÙÂÏÂ-
ÛÌ·ÙÈÎfiÙÂÚË ·ÓÙÈÌÂÙÒÈÛË ÂÍ·ÚÙ¿Ù·È ·fi ÙÔÓ
‚·ıÌfi ÂÓË̤ڈÛ˘-Âη›‰Â˘Û˘, ΢ڛˆ˜ ÙˆÓ
ÁÈ·ÙÚÒÓ, Á›ÓÂÙ·È Â‡ÎÔÏ· ·ÓÙÈÏËÙfi fiÛÔ Â›-
ηÈÚË Â›Ó·È ·˘Ù‹ Ë ·Ó·ÛÎfiËÛË (9).
πÛÙÔÚ›· ÙÔ˘ ÓÙfiÈÓÁÎ
∆Ô ÓÙfiÈÓÁÎ Â›Ó·È Û˘Ó‰Â‰Â̤ÓÔ Ì ÙËÓ ÈÛÙÔÚ›· ÙÔ˘ ·ıÏËÙÈÛÌÔ‡. À¿Ú¯Ô˘Ó ΛÌÂÓ· ÙÔ˘ ¶˘ı·ÁfiÚ·, ÙÔ˘ πÔÎÚ¿ÙË Î·È ÙÔ˘ ∞ÚÈÛÙÔÙ¤ÏË Ô˘ ·Ó·Ê¤ÚÔÓÙ·È ÛÙË ¯Ú‹ÛË ‰È·ÊfiÚˆÓ Ô˘ÛÈÒÓ/‚ÔËıËÌ¿ÙˆÓ ÁÈ· ÙËÓ ·‡ÍËÛË Ù˘ ·fi‰ÔÛ˘. ™ÙË ÚˆÌ·˚΋ ÂÔ¯‹, ›Û˘, ˘¿Ú¯Ô˘Ó ÔÏϷϤ˜ ·Ó·ÊÔÚ¤˜ ·fi ÙÔÓ ºÈÏfiÛÙÚ·ÙÔ, ÙÔÓ æ¢‰ÔÁ·ÏËÓfi, ÙÔÓ √ÚÂÈ‚¿ÛÈÔ Î.Ï. ∂›Ó·È ‰Â, ÁÓˆÛÙ‹ Ë ÂÚ›-
‚) ∞Ó·‚ÔÏÈο ÛÙÂÚÔÂȉ‹: ÙÂÛÙÔÛÙÂÚfiÓË, ‰È¸‰ÚÔÙÂÛÙÔÛÙÂÚfiÓË, ‰È¸‰ÚÔÂÈ·Ó‰ÚÔÛÙÂÚfiÓË, ÎÏÂÓ‚Ô˘ÙÂÚfiÏË Î.Ï. ¶ÚÔηÏÔ‡Ó ·‡ÍËÛË Ù˘ Ì˘˚΋˜ ‰‡Ó·Ì˘ Î·È Ù˘ Ì˘˚΋˜ Ì¿˙·˜. ™ÙȘ ·ÚÂÓ¤ÚÁÂȘ ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È ·ÏÏ·Á¤˜ ÛÙ· Â͈ÙÂÚÈο ¯·Ú·ÎÙËÚÈÛÙÈο ÙÔ˘ ʇÏÔ˘. ™ÙȘ Á˘Ó·›Î˜ ÚÔηÏÂ›Ù·È ÌÈÎÚfi ̤ÁÂıÔ˜ Ì·ÛÙÒÓ, ‰È·Ù·Ú·¯¤˜ Ù˘ ÂÌÌËÓÔÚÚ˘Û›·˜ Î·È Ù˘ ʈӋ˜. ™ÙÔ˘˜ ¿ÓÙÚ˜ ·Ú·ÙËÚÂ›Ù·È Á˘Ó·ÈÎÔÌ·ÛÙ›· (12), ·ÙÚÔÊ›· ÙˆÓ fiÚ¯ÂˆÓ Î·È ·ÒÏÂÈ· ÙÚȯÒÓ Ù˘ ÎÂÊ·Ï‹˜ (13-16). ∂›Û˘, ÌÔÚ› Ó· ÚÔÎÏËıÔ‡Ó ˘¤ÚÙ·ÛË, ‰ÂÚÌ·Ù›Ùȉ˜ (17), ÚÔ‚Ï‹Ì·Ù· ‹·ÙÔ˜ (18) Î·È ÓÂÊÚÒÓ, ‰È·Ù·Ú·¯¤˜ ÚÔÛˆÈÎfiÙËÙ·˜, ÂÈıÂÙÈ΋ Û˘ÌÂÚÈÊÔÚ¿ Î.¿. (19-22). ™Ô‚·Úfiٷ٘ ·ÚÂÓ¤ÚÁÂȘ ·ÔÙÂÏÔ‡Ó Ë ÛÙÂÊ·ÓÈ·›· ÓfiÛÔ˜ (23,24) Î·È Ë ·˘ÍË̤ÓË Èı·ÓfiÙËÙ· ·Ó¿Ù˘Í˘ ηÎÔ‹ıˆÓ ÂÍÂÚÁ·ÛÈÒÓ (25,26). Á) ‚-∞Ó·ÛÙÔÏ›˜: ÚÔÚ·ÓÔÏfiÏË, ÛÔÙ·ÏfiÏË, ·ÎÂÙÔ‚Ô˘Ù˘ÏfiÏË Î.¿. ¶ÚÔηÏÔ‡Ó ‚Ú·‰˘Î·Ú‰›·, ÂÏ¿ÙÙˆÛË ÙÔ˘ Ì˘˚ÎÔ‡ ÙÚfiÌÔ˘ Î.Ï. ™Â ÌÈÎÚ¤˜ ‰fiÛÂȘ ‰ÂÓ ÚÔηÏÔ‡Ó È‰È·›ÙÂÚ· ÚÔ‚Ï‹Ì·Ù·. øÛÙfiÛÔ, Û ·Ú·ÙÂٷ̤ÓË ¯Ú‹ÛË Î·È ÌÂÁ¿Ï˜ ‰fiÛÂȘ ˘¿Ú¯ÂÈ Èı·ÓfiÙËÙ· ÂÁÎÂÊ·ÏÈÎÒÓ ÂÂÈÛÔ‰›ˆÓ (27). ‰) ¢ÈÔ˘ÚËÙÈο: ÊÔ˘ÚÔÛÂÌ›‰Ë, ÛÈÚÔÓÔÏ·ÎÙfiÓË, ˘‰ÚԯψÚÔıÂÈ·˙›‰Ë Î.Ï. ÃÚËÛÈÌÔÔÈÔ‡ÓÙ·È ÁÈ· ·ÒÏÂÈ· ‚¿ÚÔ˘˜ (΢ڛˆ˜ ÛÙËÓ ¿ÚÛË ‚·ÚÒÓ, ÙËÓ ¿ÏË Î.¿.), ·ÏÏ¿ Î·È ÁÈ· ·Ô‚ÔÏ‹ ‹ ·Ú·›ˆÛË ‰È·ÊfiÚˆÓ Ô˘ÛÈÒÓ ÛÙÔ ·›Ì·. ™Â ·ÏfiÁÈÛÙË ¯Ú‹ÛË ÌÔÚ› Ó· ÚÔηϤÛÔ˘Ó ·Ê˘‰¿ÙˆÛË, Ì˘˚Τ˜ ‰È·Ù·Ú·¯¤˜, ηıÒ˜ Î·È ‰È·Ù·Ú·¯¤˜ ·fi ÙÔ Î·Ú‰È·ÁÁÂÈ·Îfi Û‡ÛÙËÌ· (27). Â) ¡·ÚΈÙÈο ·Ó·ÏÁËÙÈο: ÌÔÚÊ›ÓË, Âıȉ›ÓË, ÌÂı·‰fiÓË, Έ‰Â˝ÓË, ÔÈÔ‡¯· Î.Ï. ÃÚËÛÈÌÔÔÈÔ‡ÓÙ·È ÁÈ· ÙË Ì›ˆÛË ÙÔ˘ ¿ÏÁÔ˘˜ (΢ڛˆ˜ ˘ÁÌ·¯›·). ¶ÚÔηÏÔ‡Ó ÂıÈÛÌfi, ·ÏÏ¿ ηÈ
ÂÊ‹‚Ô˘˜
·ÚÚ˘ı̛˜, ‰È·Ù·Ú·¯¤˜ ÙÔ˘ ηډȷÁÁÂÈ·ÎÔ‡
Û˘ÛÙ‹Ì·ÙÔ˜, ÂÁÎÂÊ·ÏÈ΋ ·ÈÌÔÚÚ·Á›· Î.¿.
ÛÙ) ¶ÂÙȉÈΤ˜ ÔÚÌfiÓ˜: ACTH, ÂÚ˘ıÚÔ-
ÔÈËÙ›ÓË, ·˘ÍËÙÈ΋ ÔÚÌfiÓË Î.Ï. ∏ ∞CTH ·˘-
Í¿ÓÂÈ ÙË Ì˘˚΋ Ì¿˙· Î·È ÙË Ì˘˚΋ ÈÛ¯‡ Ì ÙËÓ
·‡ÍËÛË ÙˆÓ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ ÛÙÔ ·›Ì·. ∏
ÂÚ˘ıÚÔÔÈËÙ›ÓË ÂÈÙ˘Á¯¿ÓÂÈ ÙËÓ ·‡ÍËÛË ÙˆÓ
ÂÚ˘ıÚÒÓ ·ÈÌÔÛÊ·ÈÚ›ˆÓ ÙÔ˘ ·›Ì·ÙÔ˜ Î·È ÙË ÌÂ-
Ù·ÊÔÚ¿ Ô͢ÁfiÓÔ˘ ÛÙÔ˘˜ ÈÛÙÔ‡˜. §fiÁˆ Ù˘ ·˘-
ÍË̤Ó˘
ËÎÙÈÎfiÙËÙ·˜ ÙÔ˘ ·›Ì·ÙÔ˜, ÌÔÚ› Ó·
ÚÔÎÏËıÔ‡Ó ¤ÌÊÚ·ÁÌ· ÙÔ˘ Ì˘Ôηډ›Ô˘ ηÈ
ıÚÔÌ‚ÔÂÌ‚ÔÏÈο ÂÂÈÛfi‰È· ÛÙ· ÂÁÎÂÊ·ÏÈο
Î·È ¿ÏÏ· ·ÁÁ›· (28,29). ∏ ·˘ÍËÙÈ΋ ÔÚÌfiÓË
¯ÚËÛÈÌÔÔÈÂ›Ù·È Û˘Ó‹ıˆ˜ ÛÂ Û˘Ó‰˘·ÛÌfi Ì ·Ó·‚ÔÏÈο ÛÙÂÚÔÂȉ‹ ÁÈ· ÙËÓ ·‡ÍËÛË Ù˘ Ì˘˚΋˜ ‰‡Ó·Ì˘ (30). ¶·Ú¿ ÙËÓ Â˘Ú›· ¯Ú‹ÛË Ù˘ (31,32), ‰ÂÓ ˘¿Ú¯Ô˘Ó ·Ô‰Â›ÍÂȘ ÁÈ· ÙËÓ ·‡-
ÍËÛË Ù˘ ·fi‰ÔÛ˘ ÛÙ· ‰È¿ÊÔÚ· ·ıÏ‹Ì·Ù· (΢ڛˆ˜ ÛÙËÓ ¿ÚÛË ‚·ÚÒÓ). ˙) ∫Ú·ٛÓË: ∞˘Í¿ÓÂÈ ÙË Ì˘˚΋ Ì¿˙·. ÃÚËÛÈÌÔÔÈÂ›Ù·È Î˘Ú›ˆ˜ ·fi fiÛÔ˘˜ ·Û¯ÔÏÔ‡ÓÙ·È Û˘-
ÛÙËÌ·ÙÈο Ì ÙË ÛˆÌ·ÙÔ‰fiÌËÛË (body-builders).
Ë) °ÔÓȉȷÎfi ÓÙfiÈÓÁÎ: ∂ÂÌ‚·›ÓÔÓÙ·˜ ÁÔÓÈ-
‰È·Î¿ Â›Ó·È ‰˘Ó·ÙfiÓ Ó· ÌÂÙ·Ùڷ› Ë ·Ó·ÏÔÁ›·
ÙˆÓ Ì˘˚ÎÒÓ ÈÓÒÓ Ì ·˘ÍË̤ÓË ‹ ÂÏ·Ùو̤ÓË
Û˘ÛÙ·ÏÙÈÎfiÙËÙ·, ‚ÔËıÒÓÙ·˜ ¤ÙÛÈ ÙÔ˘˜ ·ıÏËÙ¤˜
ÙˆÓ ‰ÚfiÌˆÓ ÁÈ· ÌÈÎÚ¤˜ (sprinters) ‹ ÁÈ· ÌÂÁ¿Ï˜ (Ì·Ú·ıˆÓÔ‰ÚfiÌÔÈ) ·ÔÛÙ¿ÛÂȘ (33).
ı) πÓÛÔ˘Ï›ÓË: ÃÚËÛÈÌÔÔÈÂ›Ù·È ˆ˜ ·Ó·‚ÔÏÈ΋ ÔÚÌfiÓË ÛÙ· ·ıÏ‹Ì·Ù· ‰‡Ó·Ì˘, fiˆ˜ Ë ¿ÚÛË ‚·ÚÒÓ. ¢ÂÓ ˘¿Ú¯ÂÈ ‚È‚ÏÈÔÁÚ·ÊÈ΋ ÙÂÎÌËÚ›ˆÛË ˆ˜ ÚÔ˜ ÙË ıÂÙÈ΋ Ù˘ ›‰Ú·ÛË, ÙÔ˘Ó·ÓÙ›ÔÓ ˘¿Ú¯Ô˘Ó ·Ó·ÊÔÚ¤˜ ÁÈ· ˘ÔÁÏ˘Î·ÈÌÈο
ÂÂÈÛfi‰È· (34,35). È) ™˘ÌÏËÚÒÌ·Ù· ‰È·ÙÚÔÊ‹˜ - ÔÏ˘‚ÈÙ·ÌÈÓÔ‡¯· Û΢¿ÛÌ·Ù·: À¿Ú¯ÂÈ ÌÂÁ¿ÏË ÔÈÎÈÏ›·
ÔÏ˘‚ÈÙ·ÌÈÓÔ‡¯ˆÓ Û΢·ÛÌ¿ÙˆÓ, Ì ۯ‰fiÓ
fiϘ ÙȘ ˘‰·ÙÔ‰È·Ï˘Ù¤˜ Î·È ÏÈÔ‰È·Ï˘Ù¤˜ ‚Èٷ̛Ә Û ‰È¿ÊÔÚ˜ ÂÚÈÂÎÙÈÎfiÙËÙ˜. ∂›Û˘, ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È Û·Ó Û˘Ìϋڈ̷ ‰È·ÙÚÔÊ‹˜ Û΢¿ÛÌ·Ù· Ì ̤ٷÏÏ·, ȯÓÔÛÙÔȯ›·, Âί˘Ï›ÛÌ·Ù· ‚ÔÙ¿ÓˆÓ Î.Ï. ™Â ·ÚÎÂÙ¤˜ ÂÚÈÙÒÛÂȘ, Èı·ÓÒ˜ Ù· ÂÚÈÛÛfiÙÂÚ· ·fi ·˘Ù¿ - Î·È È‰È·›ÙÂÚ· ÛÙȘ ÌÂÁ¿Ï˜ ‰fiÛÂȘ Ô˘ Û˘¯Ó¿ Û˘ÓÈÛÙÒÓÙ·È - Â›Ó·È ¿¯ÚËÛÙ· (16). È·) ∞Ó·„˘ÎÙÈο Ù‡Ô˘ sports drinks Î·È energy drinks: ¶·Ú’ fiÏÔ Ô˘ ¤¯ÂÈ Û˘˙ËÙËı› ·ÚÎÂÙ¿, Ù· sports drinks ‰ÂÓ Â›Ó·È ÂÈΛӉ˘Ó·, ·ÊÔ‡ ÛÙËÓ Ú·ÁÌ·ÙÈÎfiÙËÙ· Â›Ó·È ËÏÂÎÙÚÔÏ˘ÙÈο ‰È·Ï‡Ì·Ù·. ∆· energy drinks ÂÚȤ¯Ô˘Ó ΢ڛˆ˜ ηʽÓË, ·ÏÏ¿ Î·È ¿ÏϘ Ô˘Û›Â˜ fiˆ˜ ‚Èٷ̛Ә, Ù·˘Ú›ÓË Î.Ï. ™Â ηÓÔÓÈ΋
ÛÂȘ, ˆÛÙfiÛÔ, Ô˘ ·Ó·ÌÂÈÁÓ‡ÔÓÙ·È Ì ¿ÏϘ ‡ÔÙ˜ Ô˘Û›Â˜ ‹ ÔÈÓÔÓÂ˘Ì·ÙÒ‰Ë ÔÙ¿, ÌÔÚ› Ó· ‰ËÌÈÔ˘ÚÁËıÔ‡Ó ·ÓÂÈı‡ÌËÙ˜ ÂÓ¤ÚÁÂȘ
fiˆ˜ ̤ıË Î.Ï. (13).
ŸÏ· fiÛ· ·Ó·Ê¤ÚıËÎ·Ó ·ÔÙÂÏÔ‡Ó ¤Ó· ÌÈ-
ÎÚfi ̤ÚÔ˜ ÙˆÓ Ì¤ÛˆÓ Ô˘ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È
ÁÈ· ÓÙfiÈÓÁÎ. ∏ ·Ó·ÊÔÚ¿ Û ÂÚÈÛÛfiÙÂÚ· ı·
ÍÂÂÚÓÔ‡Û ٷ fiÚÈ· ·˘ÙÔ‡ ÙÔ˘ ¿ÚıÚÔ˘. ¶Ú¤ÂÈ
Ó· ÚÔÛÙÂı› fiÙÈ ÔÏÏ¿ ·fi Ù· Û΢¿ÛÌ·Ù·, ȉȷ›ÙÂÚ· ·˘Ù¿ Ô˘ ‰ÂÓ ·Ó‹ÎÔ˘Ó ÛÙËÓ Î·ÙËÁÔ-
Ú›· ÙˆÓ Ê·Ú̿ΈÓ, ‰È·Ù›ıÂÓÙ·È ÂχıÂÚ· ·fi
Ù· ÂȉÈο ηٷÛÙ‹Ì·Ù·, ÙÔ ‰È·‰›ÎÙ˘Ô ‹
Ù· Ê·Ú̷Λ·. ∂›Û˘, ı· Ú¤ÂÈ Ó· ·Ó·ÊÂÚı›
fiÙÈ ÙÔ ÓÙfiÈÓÁÎ Â›Ó·È È‰È·›ÙÂÚ· ‰È·‰Â‰Ô̤ÓÔ Î·È
ÛÙ· ˙Ò·, ΢ڛˆ˜ ÛÙ· ¿ÏÔÁ· ·ÁÒÓˆÓ (36-38).
ËÏÈ˘, ÛÙ· ·È‰È¿ ÙÔ˘ ‰ËÌÔÙÈÎÔ‡ ‹ ÛÙ· ÌÈÎÚfiÙÂÚ·, Ú·ÎÙÈο ‰ÂÓ ˘¿Ú¯ÂÈ
ÓÙfiÈÓÁÎ. ™ÙËÓ ÂÊË‚È΋ ËÏÈΛ·, fiˆ˜ ηÈ
Ó·ÚÔ‡˜ ÂÓ‹ÏÈΘ, ÔÈ Èı·ÓfiÙËÙ˜ ÙÔ˘
ÓÙfiÈÓÁÎ ·˘Í¿ÓÔÓÙ·È, ΢ڛˆ˜ fiÛÔÓ ·ÊÔÚ¿ ÛÙ· ·È‰È¿ Ô˘ οÓÔ˘Ó ÚˆÙ·ıÏËÙÈÛÌfi ‹ Û ·˘Ù¿
ÙˆÓ ÔÔ›ˆÓ ÔÈ ÂȉfiÛÂȘ ÙÔ˘˜ “˘fiÛ¯ÔÓÙ·È”
ÌÂÏÏÔÓÙÈÎÔ‡˜ ÚˆÙ·ıÏËÙ¤˜ (39,40). ™ÙË ‚È‚ÏÈÔÁÚ·Ê›· ˘¿Ú¯Ô˘Ó ÌÂϤÙ˜ Ô˘ ·Ó·Ê¤ÚÔÓÙ·È Û ÔÛÔÛÙ¿ 1-2% ÛÙ· ÎÔÚ›ÙÛÈ· Î·È 3-12%
ÛÙ· ·ÁfiÚÈ·. ™Â ¿ÏϘ ÌÂϤÙ˜ Ô˘ ÂÚÈÏ·Ì‚¿-
ÓÔ˘Ó Î·È Ù· ‰‡Ô ʇϷ, ·Ó·Ê¤ÚÔÓÙ·È ÔÛÔÛÙ¿ 36% (41,42). ∂Âȉ‹, fï˜, ÔÈ ÂÚÁ·Û›Â˜ ·˘Ù¤˜
Û˘Ó‹ıˆ˜ ÛÙËÚ›˙ÔÓÙ·È Û ÂÚˆÙËÌ·ÙÔÏfiÁÈ· Ô˘
Û˘ÌÏËÚÒÓÔÓÙ·È ·fi ÙÔ˘˜ ›‰ÈÔ˘˜ ÙÔ˘˜ ÂÊ‹‚Ô˘˜ ‹ ÙÔ˘˜ Ó·ÚÔ‡˜ ÂÓ‹ÏÈΘ, Â›Ó·È Èı·Ófi Ù·
ÔÛÔÛÙ¿ ·˘Ù¿ Ó· Â›Ó·È Ôχ ÌÂÁ·Ï‡ÙÂÚ·.
À¿Ú¯Ô˘Ó ÌÂϤÙ˜ Ô˘ ·Ó·Ê¤ÚÔÓÙ·È Û ·È‰È¿
ËÏÈΛ·˜ 12-15 ÂÙÒÓ, fiÔ˘ Ù· ÔÛÔÛÙ¿ ·Ó¤Ú¯Ô-
ÓÙ·È Û 35%, ηıÒ˜ Î·È ¿ÏϘ Ô˘ ·Ó·Ê¤ÚÔ-
ÓÙ·È Û ËÏÈ˘ ÌÂÁ·Ï‡ÙÂÚ˜ ÙˆÓ 17 ÂÙÒÓ, fiÔ˘ Ù· ÔÛÔÛÙ¿ ÊÙ¿ÓÔ˘Ó ÙÔ 80% (43,45).
øÛÙfiÛÔ, fiÔÈ· Î·È ·Ó Â›Ó·È Ë Ú·ÁÌ·ÙÈ΋ Û˘¯ÓfiÙËÙ· ÙÔ˘ ÓÙfiÈÓÁÎ ÛÙËÓ ·È‰È΋ ËÏÈΛ·, Â›Ó·È ¿‰ÈÎÔ Ó· ‰ËÏËÙËÚÈ¿˙ÂÙ·È Ô ÂÓıÔ˘ÛÈ·ÛÌfi˜
Û˘ÓËıÂÈÒÓ Î·È ¿Óˆ ·’ fiÏ· ‰È·ÛΤ‰·ÛË Î·È ¯·Ú¿ (46). ∏ ¯Ú‹ÛË ··ÁÔÚÂ˘Ì¤ÓˆÓ Ô˘ÛÈÒÓ ÌÔÚ› Ó· ·ÓÙÈÌÂÙˆÈÛÙ› Ì ÂÓË̤ڈÛË, Âη›‰Â˘ÛË Î·È Ï‹ıÔ˜ ¿ÏÏˆÓ ÙÚfiˆÓ Ô˘ ·Ó·Ê¤ÚÔÓÙ·È ÛÙË Û˘Ó¤¯ÂÈ· (47).
Ú›˙ÂÙ·È ‹ Ó· ÂÓı·ÚÚ‡ÓÂÙ·È ÔÔÈ·‰‹ÔÙ ÌÔÚÊ‹
ÓÙfiÈÓÁÎ. ¶·Ú’ fiÏ· ·˘Ù¿, fiˆ˜ Ê·›ÓÂÙ·È ·fi
ÙȘ Ô˘Û›Â˜ Ô˘ ·Ó·Ê¤ÚıËηÓ, ˘¿Ú¯ÂÈ ÌÂÁ¿ÏË
‰È·ÊÔÚ¿ .¯. ÌÂٷ͇ Ù˘ ¯Ú‹Û˘ ηÊ½Ó˘ ηÈ
ÎÔη˝Ó˘, ‰ÈÔ˘ÚËÙÈÎÒÓ Î·È ÌÔÚÊ›Ó˘ Î.Ï.
∂ÎÙfi˜, fï˜, ·fi ÙÔ Â›‰Ô˜ Ù˘ Ô˘Û›·˜, ·˘Ùfi
Ô˘ ηıÔÚ›˙ÂÈ ÙË ÛÔ‚·ÚfiÙËÙ· ÙˆÓ ‚Ï·‚ÒÓ Â›-
Ó·È Ë ‰fiÛË Î·È Ë ‰È¿ÚÎÂÈ· ¯Ú‹Û˘ Ù˘. √È ÌÈ-
ÎÚ¤˜ ‰fiÛÂȘ ‰ÂÓ ÚÔηÏÔ‡Ó È‰È·›ÙÂÚ· ÛÔ‚·Ú¤˜
ÂÈÙÒÛÂȘ, ·ÏÏ¿ Ë ·Ú·ÙÂٷ̤ÓË Î·È ˘ÂÚ‚Ô-
ÏÈ΋ ¯Ú‹ÛË ÔÔÈ·Û‰‹ÔÙÂ Ô˘Û›·˜ - ·ÎfiÌË Î·È
fiÙ·Ó ·˘Ù‹ Â›Ó·È Û¯ÂÙÈο ·ıÒ· - ÌÔÚ› Ó· ÚÔ-
ηϤÛÂÈ ÛÔ‚·Ú¿ ÚÔ‚Ï‹Ì·Ù· ˘Á›·˜ (6,48,49). ∆· ÈÔ ÂÈΛӉ˘Ó· ÚÔ‚Ï‹Ì·Ù·, Ù· ÔÔ›·
ÛÙȘ ÂÚÈÛÛfiÙÂÚ˜ ÂÚÈÙÒÛÂȘ ÚÔηÏÔ‡Ó
ÙÔÓ ı¿Ó·ÙÔ, ·ÊÔÚÔ‡Ó ÛÙÔ Î·Ú‰È·ÁÁÂÈ·Îfi Û‡ÛÙËÌ· (50,51). ∞Ó¿ÏÔÁ· Ì ÙÔ ¿ıÏËÌ·, ηٿ ÙË
‰È¿ÚÎÂÈ· ÙˆÓ ÚÔÔÓ‹ÛÂˆÓ ‰ËÌÈÔ˘ÚÁÔ‡ÓÙ·È
·ÏÏ·Á¤˜, ÔÈ Ôԛ˜ Â›Ó·È ··Ú·›ÙËÙ˜ ÁÈ· ÙËÓ ˘ÔÛÙ‹ÚÈÍË Ù˘ ΢ÎÏÔÊÔÚ›·˜ ÛÙÔ Û˘ÁÎÂÎÚÈ̤ÓÔ ¿ıÏËÌ·. ™ÙÔ˘˜ ·ıÏËÙ¤˜ ‰ÚfïÓ, Ô˘ ›ӷÈ
Ë Î·Ú‰È¿ ÌÂÙ·ÙÚ¤ÂÙ·È ÛÂ
fiÁÎÔ˘. ∞˘Í¿ÓÔÓÙ·È ÔÈ ‰È·ÛÙ¿ÛÂȘ ÙˆÓ
Î·È ÏÂÙ‡ÓÂÙ·È ÙÔ ÙÔ›¯ˆÌ¿ ÙÔ˘˜. ∏ ¯ˆÚËÙÈÎfiÙËÙ· - ·fi Ù· Ê˘ÛÈÔÏÔÁÈο ÂÚ›Ô˘ 600 mlÌÔÚ›, .¯. Û ̷ڷıˆÓÔ‰ÚfiÌÔ˘˜, Ó· ÊÙ¿ÛÂÈ
¤ˆ˜ Î·È Ù· 1000 ml. ™ÙȘ ÂÚÈÙÒÛÂȘ ·ıÏËÌ¿-
ÙˆÓ fiˆ˜ Ë ¿ÚÛË ‚·ÚÒÓ, ·Ú·ÙËÚÂ›Ù·È ¿¯˘ÓÛË ÙÔ˘ Ì˘Ôηډ›Ô˘ ̤¯ÚÈ Î·È 13-15 mm. (Ê.Ù.
7-11 mm) Î·È ÂÏ¿ÙÙˆÛË ÙˆÓ ‰È·ÛÙ¿ÛÂˆÓ ÙˆÓ
ÎÔÈÏÈÒÓ, ÂÓÒ Ë Î·Ú‰È¿ ÌÂÙ·ÙÚ¤ÂÙ·È Û ÎÔÈÏfiÙËÙ· ȤÛÂˆÓ (52). ¶·Ú¿ÏÏËÏ·, ·Ú·ÙËÚÔ‡-
ÓÙ·È ·ÏÏ·Á¤˜ Î·È ÛÙ· ÛÙÂÊ·ÓÈ·›· ·ÁÁ›·, Ë ‰È¿-
ÌÂÙÚÔ˜ ÙˆÓ ÔÔ›ˆÓ ÌÔÚ› Ó· ÊÙ¿ÓÂÈ Ì¤¯ÚÈ Î·È
Ù· 7 ¯ÈÏÈÔÛÙ¿ (fiÙ·Ó Ë Ê˘ÛÈÔÏÔÁÈ΋ Â›Ó·È ÂÚ›-
Ô˘ 3 ¯ÈÏÈÔÛÙ¿) (53). Ÿˆ˜ Â›Ó·È Â˘ÓfiËÙÔ, ÔÈ
Èı·ÓfiÙËÙ˜ ·Ó·ÛÙÚÔÊ‹˜ ÙˆÓ ·ÏÏ·ÁÒÓ ·˘ÙÒÓ
Â›Ó·È Â˘ı¤ˆ˜ ·Ó¿ÏÔÁ˜ Ì ÙË ‰È¿ÚÎÂÈ·, ÙËÓ
¤ÓÙ·ÛË, ·ÏÏ¿ Î·È ÙÔ Â›‰Ô˜ ÙÔ˘ ·ıÏ‹Ì·ÙÔ˜.
˘¤ÚÙ·ÛË Î.Ï.
Óˆ˜,
(56,57), ηıÒ˜ Î·È ÔÈ ‚Ï¿‚˜ ÛÙÔ ‹·Ú (Ë·ÙÈ΋ ·Ó¿ÚÎÂÈ·) Î·È ÙÔ˘˜ ÓÂÊÚÔ‡˜ (ÓÂÊÚÈ΋ ·Ó¿ÚÎÂÈ·) Ô˘ ÔÊ›ÏÔÓÙ·È ÛÙËÓ Ë·ÙÔÙÔÍÈÎfiÙËÙ· Î·È ÓÂÊÚÔÙÔÍÈÎfiÙËÙ· ÙˆÓ ‰È·ÊfiÚˆÓ Ô˘ÛÈÒÓ Ô˘ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ÛÙÔ ÓÙfiÈÓÁÎ (58). ªÔÏÔÓfiÙÈ Ë ÏÂÈÔ„ËÊ›· ÙˆÓ ·ıÏËÙÒÓ ‰ÂÓ Ê·›ÓÂÙ·È Ó· ·Ó·Ù‡ÛÛÂÈ ÂÍ¿ÚÙËÛË ·fi οÔÈ· Ô˘Û›· Ô˘ ·˘Í¿ÓÂÈ ÙËÓ ·fi‰ÔÛË ‹ Ô˘ ÌÔÚ› Ó· ÚÔηϤÛÂÈ ÂıÈÛÌfi, ˆÛÙfiÛÔ Ô Î›Ó‰˘ÓÔ˜ Â›Ó·È ˘·ÚÎÙfi˜ (59).
∞ÓÙÈ-ÓÙfiÈÓÁÎ
¶·Ú¿ ÙÔ ÁÂÁÔÓfi˜ fiÙÈ ÙÔ ÓÙfiÈÓÁÎ ‹Ù·Ó ÁÓˆÛÙfi ·fi ÙËÓ ÚÒÙË √Ï˘ÌÈ¿‰· Î·È ·Ú’ fiÏÔ Ô˘ ÛÙȘ √Ï˘ÌÈ¿‰Â˜ ÙˆÓ ÂÙÒÓ 1920-1930 ›¯Â Ï¿‚ÂÈ ÌÂÁ¿Ï˜ ‰È·ÛÙ¿ÛÂȘ, Ë ·ıÏËÙÈ΋ ÎÔÈÓfiÙËÙ· ÌfiÏȘ ÙÔ 1967 ¤Ï·‚ ٷ ÚÒÙ· ̤ÙÚ· ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒÈÛ‹ ÙÔ˘. √È ¤ÏÂÁ¯ÔÈ ·ÓÙÈ-ÓÙfiÈÓÁÎ ÂÊ·ÚÌfiÛÙËÎ·Ó ÁÈ· ÚÒÙË ÊÔÚ¿ ÛÙË ¯ÂÈÌÂÚÈÓ‹ √Ï˘ÌÈ¿‰· Ù˘ Grenoble Î·È ÛÙËÓ Î·ÏÔηÈÚÈÓ‹ ÙÔ˘ Mexico City ÙÔ 1968. ªfiÏȘ ÙÔÓ ¡Ô¤Ì‚ÚÈÔ ÙÔ˘ 1999 ȉڇıËÎÂ Ë ·ÁÎfiÛÌÈ· ÔÚÁ¿ÓˆÛË ·ÓÙÈ-ÓÙfiÈÓÁÎ (WADA), ÂÓÒ Ô ·ÁÎfiÛÌÈÔ˜ ÎÒ‰Èη˜ ·ÓÙÈ-ÓÙfiÈÓÁÎ „ËÊ›ÛÙËΠÙÔÓ π·ÓÔ˘¿ÚÈÔ ÙÔ˘ 2004 ÁÈ· Ó· ÂÊ·ÚÌÔÛÙ› ·fi ÙÔ 2006. π‰Ú‡ıËηÓ, ›Û˘, ÂıÓÈÎÔ› ÔÚÁ·ÓÈÛÌÔ› ·ÓÙÈ-ÓÙfiÈÓÁÎ, fiˆ˜ Ë USADA, AADA Î.Ï. (60). ™ÙËÓ ∂ÏÏ¿‰· ˘¿Ú¯Ô˘Ó ÔÈ ÓfiÌÔÈ 2725/99 Î·È 3057/2002, ηıÒ˜ Î·È ÙÔ ∂ıÓÈÎfi ™˘Ì‚Ô‡ÏÈÔ ∫·Ù·ÔϤÌËÛ˘ ÙÔ˘ ¡ÙfiÈÓÁÎ (∂™∫∞¡).
Ÿˆ˜ Ê·›ÓÂÙ·È ·fi ÙËÓ ·Ú·¿Óˆ Û‡ÓÙÔÌË ¯ÚÔÓÔÏÔÁÈ΋ ·Ú¿ıÂÛË, Ë ÔÚÁ·ÓˆÌ¤ÓË ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ ÓÙfiÈÓÁΠηı˘ÛÙ¤ÚËÛ ÁÈ· ÂÚÈÛÛfiÙÂÚ· ·fi ÂηÙfi ¯ÚfiÓÈ·. ∆· ηٷÛÙ·ÏÙÈο ̤ÙÚ·, ÛÙ· ÔÔ›· ÌÔÚ› Ó· ˘¿ÚÍÂÈ Î·È ¤Ó·˜ ‚·ıÌfi˜ ÌÂÚÔÏË„›·˜, ‰ÂÓ Â›Ó·È Â‡ÎÔÏÔ Ó· ¤¯Ô˘Ó ·fiÏ˘Ù· ÈηÓÔÔÈËÙÈ΋ ·fi‰ÔÛË. °È· Ó· ·ÓÙÈÌÂÙˆÈÛÙ› ÙÔ ÓÙfiÈÓÁÎ ·ÔÙÂÏÂÛÌ·ÙÈο, οÙÈ Ô˘ - fiˆ˜ Ê·›ÓÂÙ·È - Â›Ó·È ·ÚÎÂÙ¿ ‰‡ÛÎÔÏÔ, ı· Ú¤ÂÈ Ó· ÏËÊıÔ‡Ó ˘’fi„ÈÓ Ôχ ÚÔÛÂÎÙÈο Ù· ·ÎfiÏÔ˘ı· (61-63): ·) Ÿˆ˜ ‹‰Ë ·Ó·Ê¤ÚıËÎÂ, “ÛÔ‚·Úfi” ÓÙfiÈÓÁÎ ¯ˆÚ›˜ ÙË Û˘ÌÌÂÙÔ¯‹, Û˘ÓÙ·ÁÔÁÚ¿ÊËÛË (64,65) Î·È Î·ıÔ‰‹ÁËÛË ÙˆÓ ÁÈ·ÙÚÒÓ ‰ÂÓ ÌÔÚ› Ó· Á›ÓÂÈ. °È· ÙÔÓ ÏfiÁÔ ·˘Ùfi ı· Ú¤ÂÈ Î·Ù’ ·Ú¯¿˜ Ó· ÂÓËÌÂÚˆıÔ‡Ó fiÏÔÈ ÔÈ ÁÈ·ÙÚÔ› ÁÈ· ÙÔ Ì¤ÁÂıÔ˜ ÙÔ˘ ÚÔ‚Ï‹Ì·ÙÔ˜, ÙȘ ‰È·ÛÙ¿ÛÂȘ Ô˘ ¤¯ÂÈ Ï¿‚ÂÈ Î·È ÙÔ˘˜ ÎÈÓ‰‡ÓÔ˘˜ ÁÈ· Ù·
ÔÈÓÔÓÂ˘Ì·ÙÒ‰Ë, Ó·ÚΈÙÈο Î.Ï.). √ ÁÈ·ÙÚfi˜
Ô˘ ÁÓˆÚ›˙ÂÈ ÙȘ ÂÈÏÔΤ˜ Ù˘ ¯Ú‹Û˘ ÙˆÓ
‰È·ÊfiÚˆÓ ÌÂıfi‰ˆÓ/Ô˘ÛÈÒÓ, ı· Ú¤ÂÈ Ó· ›ӷÈ
Î·È Ô ÚÒÙÔ˜ Ô˘ ı· οÓÂÈ ÙË ÛˆÛÙ‹ ÂÓË̤ڈ-
ÛË. ¶·Á›‰Â˜ fiˆ˜ ÔÈ ·Ú·ÎÏ‹ÛÂȘ ·fi ÙÔÓ
ÚˆÙ·ıÏËÙ‹ ‹ ·ÎfiÌË Î·È ÙÔ ·ÎÚ·›Ô Ê·ÈÓfiÌÂÓÔ
Ù˘ ·Ú¿ÎÏËÛ˘ ·fi ÁÔÓ›˜ ÁÈ· ÓÙÔ¿ÚÈÛÌ·
ÙÔ˘ ·È‰ÈÔ‡ ÙÔ˘˜ Ì ÛÎÔfi ÙËÓ ÂÈÙ˘¯›·, Û›-
ÁÔ˘Ú· Â›Ó·È Â‡ÎÔÏÔ Ó· ·ÔÊ¢¯ıÔ‡Ó ·fi ÙÔ˘˜
ÁÈ·ÙÚÔ‡˜. ∏ ‰Â ÛˆÛÙ‹ ÂÓË̤ڈÛË Î·È Âη›-
‰Â˘ÛË, ΢ڛˆ˜ ÙˆÓ ÂÊ‹‚ˆÓ Î·È ÙˆÓ Ó·ÚÒÓ
ÂÓËϛΈÓ, Ê·›ÓÂÙ·È fiÙÈ Ú¿ÁÌ·ÙÈ ·Ô‰›‰ÂÈ, ·ÊÔ‡ Û ¤Ó· ÛËÌ·ÓÙÈÎfi ÔÛÔÛÙfi Ë ¯Ú‹ÛË Á›ÓÂ-
Ù·È ¯ˆÚ›˜ ÙË ÁÓÒÛË ÙˆÓ Û˘ÓÂÂÈÒÓ (66). ‚) £· Ú¤ÂÈ - ÛÂ Û˘ÓÂÓÓfiËÛË Ì ÙÔ˘˜ ÁÈ·ÙÚÔ‡˜ - Ó· Û˘ÌʈÓËı› ·fi fiÏÔ˘˜ ÙÔ˘˜ ÂÌÏÂÎfiÌÂÓÔ˘˜ (·ıÏËÙ¤˜, ÚÔÔÓËÙ¤˜, Û˘ÏÏfiÁÔ˘˜, ÔÌÔÛÔÓ‰›Â˜ Î.Ï.) Â¿Ó ÂÈı˘ÌÔ‡Ó ÙÔ ÓÙfiÈÓÁÎ
ÛÙÔÓ ·ıÏËÙÈÛÌfi ‹ fi¯È. ∂›Ó·È ÛËÌ·ÓÙÈÎfi ·˘Ù‹ Ë
Û˘ÓÂÓÓfiËÛË Ó· Â›Ó·È ·fiÏ˘ÙË, ηıÒ˜ Ë ¯Ú‹ÛË
ÓÙfiÈÓÁÎ ·fi ÔÚÈṲ̂ÓÔ˘˜ ÌfiÓÔ ·ıÏËÙ¤˜ Ô‰ËÁ› ·˘ÙfiÌ·Ù· Û ·ÔÙ˘¯›· fiÛÔ˘˜ ÙËÓ Î·Ù·‰Èο˙Ô˘Ó. ¢˘ÛÙ˘¯Ò˜, ˘¿Ú¯Ô˘Ó ·ıÏËÙ¤˜, ·Ú¿ÁÔÓÙ˜ ‹ ·ÎfiÌË Î·È ÁÈ·ÙÚÔ› Ô˘ ˘ÔÛÙËÚ›˙Ô˘Ó ÙË
¯ÚËÛÈÌÔÔ›ËÛË ‰È·ÊfiÚˆÓ ·ı¤ÌÈÙˆÓ ÌÂıfi‰ˆÓ Î·È Ô˘ÛÈÒÓ ÛÙÔÓ ·ıÏËÙÈÛÌfi, ÛÙÔ Ï·›ÛÈÔ Ù˘
ÂÏ¢ıÂÚ›·˜ ÛÙËÓ ÂÈÏÔÁ‹ Î·È Ù˘ ·ÙÔÌÈ΋˜ ¢ı‡Ó˘ (67).
√ “ηı·Úfi˜” ·ıÏËÙÈÛÌfi˜ Â›Ó·È Ë ‚·ÛÈ΋
Âȉ›ˆÍË (68), ·ÏÏ¿ Â›Ó·È È‰È·›ÙÂÚ· ‰‡ÛÎÔÏÔ Ó·
˘¿ÚÍÂÈ Î·ıÒ˜ ÚÔ¸Ôı¤ÙÂÈ ·ÏÏ·Á‹ ÓÔÔÙÚÔ-
›·˜ ·¤Ó·ÓÙÈ ÛÙË ˙ˆ‹ ·ÏÏ¿ Î·È ÛÙÔÓ ·ıÏËÙÈ-
ÛÌfi. √ ·ıÏËÙ‹˜ ı· Ú¤ÂÈ Ó· ÈηÓÔÔÈÂ›Ù·È ÌÂ
fi,ÙÈ ÚÔÛʤÚÂÈ ÛÙËÓ ˘Á›· Î·È ÙÔ Ó‡̷ ÙÔ˘ Ë
ۈ̷ÙÈ΋ ¿ÛÎËÛË Î·È Ó· ·ÚÎÂ›Ù·È Û ·˘Ù¿ fiÙ·Ó
ÙÔ ÛÒÌ· ÙÔ˘ ‰ÂÓ ‰È·ı¤ÙÂÈ ÙȘ ··Ú·›ÙËÙ˜ ‰˘Ó¿ÌÂȘ Î·È ·ÓÙÔ¯¤˜ ÁÈ· ˘„ËϤ˜ ÂȉfiÛÂȘ Î·È ‰È¿ÎÚÈÛË (69).
µÈ‚ÏÈÔÁÚ·Ê›·
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Î·È ÛÙËÓ ÂÓ‰ÔÊϤ‚ÈÔ ˘ÂÏÔÁÚ·Ê›· ‰È·ÈÛÙÒıËÎÂ
‰È¿Ù·ÛË ÙˆÓ Î·Ï‡ÎˆÓ ¯ˆÚ›˜ Ó· Û˘Ó˘¿Ú¯ÂÈ ‰È¿Ù·ÛË Ù˘ ˘¤ÏÔ˘ (∂ÈÎfiÓ·
∂ÈÎfiÓ· 2. ∂Ó‰ÔÊϤ‚ÈÔ˜ ˘ÂÏÔÁÚ·Ê›·, Ô˘ ‰Â›¯ÓÂÈ ‰È¿Ù·ÛË ÙˆÓ Î·Ï‡ÎˆÓ ÙÔ˘ ·ÚÈÛÙÂÚÔ‡ ÓÂÊÚÔ‡ Î·È Î˘Ú›ˆ˜ Ù˘ ¿Óˆ Î·Ï˘ÎÈ΋˜ ÔÌ¿‰·˜ (‚¤ÏÔ˜).
™ÙÂÊ·Ó›‰Ë˜ stefanid@hol.gr
∏ ·¿ÓÙËÛË ·ÎÔÏÔ˘ı› ÛÙË ÛÂÏ›‰· 326
µ’ ¶·È‰È·ÙÚÈ΋
¶·ÓÂÈÛÙËÌȷ΋ ∫ÏÈÓÈ΋, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ
“¶. & ∞. ∫˘ÚÈ·ÎÔ‡”, ∞ı‹Ó·
AÏÏËÏÔÁÚ·Ê›·:
¡›ÎÔ˜ ¶··‰fiÔ˘ÏÔ˜ E-mail: ngp@allergy.gr
– ∞ÏÏÂÚÁÈÔÏfiÁÔ˜
¶·È‰È¿ Î·È ∆¤¯ÓË
¤ÊÂÛË
ÛÔ˘Ì ‰ËÌÈÔ˘ÚÁ›Â˜ ÌÈÎÚÒÓ Î·ÏÏÈÙ¯ÓÒÓ, ·ÏÏ¿ ηÈ, ÁÈ·Ù› fi¯È, Ó· ÚÔÙÚ¤„Ô˘ÌÂ Î·È Ù· ‰Èο Ì·˜ ·È‰È¿ Ó· Û˘ÌÌÂÙ¤¯Ô˘Ó, ·ÚÔ˘ÛÈ¿˙Ô˘Ì ÛÙÔ Ù‡¯Ô˜ ·˘Ùfi ‰È·‰ÈÎÙ˘·ÎÔ‡˜ ÙfiÔ˘˜
Arts and Kids - http://www.artsandkids.com
∆Ô Arts and Kids ¤¯ÂÈ ˆ˜ ÛÙfi¯Ô ÙËÓ ·ÚÔ˘Û›·ÛË Ó·ÚÒÓ
‰È¿, ÙÔ˘˜ ‰›ÓÂÈ ÙË ‰˘Ó·ÙfiÙËÙ· Ó· ÛÙ›ÏÔ˘Ó Â˘¯ÂÙ‹ÚȘ οÚÙ˜, Ó· χÓÔ˘Ó ·˙Ï ·fi ÂÈÎfiÓ˜ Ô˘ ¤¯Ô˘Ó ˙ˆÁÚ·Ê›ÛÂÈ ·È‰È¿, ÂÓÒ ˘¿Ú¯ÂÈ Î·È Ë ‰˘Ó·ÙfiÙËÙ· ˙ˆÁÚ·ÊÈ΋˜ ¿Óˆ Û ÚfiÙ˘· online. µ¤‚·È·, ‰ÂÓ Ï›ÂÈ Ë ¤ÎıÂÛË (gallery) Ë ÔÔ›· ¤¯ÂÈ ÙË ÌÔÚÊ‹ ‚ÈÓÙÂÔ·ÚÔ˘Û›·Û˘ Ì ¢¯¿ÚÈÛÙË ÌÔ˘ÛÈ΋ ˘fiÎÚÔ˘ÛË.
Natural Child Project - http://www.naturalchild.com/gallery/
∆Ô Natural Child Project, ÌÈ· ÎÔÈÓˆÊÂÏ‹˜ ÂÙ·ÈÚ›· Ì ÛÙfi¯Ô ÙË Û˘ÓÔÏÈÎfiÙÂÚË ˘ÔÛÙ‹ÚÈÍË ÙˆÓ ·È‰ÈÒÓ, ÂÚÈÏ·Ì‚¿ÓÂÈ ÌÈ· Ôχ ÂӉȷʤÚÔ˘Û· ¤ÎıÂÛË Ì ÂÚÈÛÛfiÙÂÚ˜ ·fi 1.000 ˙ˆÁÚ·ÊȤ˜ ·È‰ÈÒÓ ·’ fiÏÔ ÙÔÓ ÎfiÛÌÔ. ªÔÚ› ηÓ›˜ Ó· ·ÁÔÚ¿ÛÂÈ Î¿ÚÙ˜ ·fi ÙȘ ‰ËÌÈÔ˘ÚÁ›Â˜ ·˘Ù¤˜ Ô˘ Â›Ó·È ÔÌÔÏÔÁÔ˘Ì¤Óˆ˜ ÂÍ·ÈÚÂÙÈΤ˜.
Kids’ Space - http://www.kids-space.org/ ŒÓ· ·ÎfiÌË ›‰Ú˘Ì· Ì ÂÎ·È‰Â˘ÙÈÎfi ¯·Ú·ÎÙ‹Ú·, ÙÔ ∫ids’ Space, ¤¯ÂÈ ˆ˜ ÛÙfi¯Ô ÙË ‚ÂÏÙ›ˆÛË Ù˘ ÂÎ·È‰Â˘ÙÈ΋˜ ÂÌÂÈÚ›·˜ ̤ۈ ‰È·‰ÈÎÙ‡Ô˘, ÙËÓ ·ÔÌ˘ıÔÔ›ËÛË ÙÔ˘ Internet Î·È ÙË ¯Ú‹ÛË ÙÔ˘ ˆ˜ ¿ÍÔÓ· ·ÁÎfiÛÌÈ·˜ Î·È ‰È·ÔÏÈÙÈÛÌÈ΋˜ ÂÈÎÔÈÓˆÓ›·˜. ∂‰Ò ÔÈ ˙ˆÁÚ·ÊȤ˜ Ù˘ ¤Î-
∫ÏÈÓÈÎfi QUIZ
∞¶∞¡∆∏™∏
∏ ‰È¿ÁÓˆÛË Â›Ó·È ÌÂÁ·Î·Ï‡ÎˆÛË ‹ ÓfiÛÔ˜
ÙÔ˘ Puigvert, Ë ÔÔ›· ‚·Û›ÛÙËΠÛÙÔ Ù˘ÈÎfi ‡-
ÚËÌ· Ù˘ ‰È¿Ù·Û˘ ÙˆÓ Î·Ï‡ÎˆÓ, ¯ˆÚ›˜ Ó· Û˘-
Ó˘¿Ú¯ÂÈ ‰È¿Ù·ÛË Ù˘ ˘¤ÏÔ˘ (1-3). ™Â ·ÓÙ›ıÂ-
ÛË Ì ÙË ÌÂÁ·Î·Ï‡ÎˆÛË, ÛÙË ÛÙ¤ÓˆÛË Ù˘ ˘Â-
ÏÔÔ˘ÚËÙËÚÈ΋˜ Û˘Ì‚ÔÏ‹˜ ‰È·ÈÛÙÒÓÂÙ·È ÛËÌ·-
ÓÙÈ΋ ‰È¿Ù·ÛË ÙfiÛÔ Ù˘ ˘¤ÏÔ˘, fiÛÔ Î·È ÙˆÓ
ηχΈÓ, ηıÒ˜ Î·È ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù· ÛÙÔ ‰˘Ó·ÌÈÎfi ÛÈÓıËÚÔÁÚ¿ÊËÌ· ÓÂÊÚÒÓ Ì 99mTc
ÌÂÚηÙÔ-·ÎÂÙ˘ÏÔ-ÙÚÈÁÏ˘Î›ÓË (MAG3). ∂ÈϤÔÓ, Ù· ·È‰È¿ Ì ÌÂÁ·Î·Ï‡ÎˆÛË ‰ÂÓ ¤¯Ô˘Ó
Â˘Ú‹Ì·Ù· ·fiÊڷ͢ ÛÙÔ ‰˘Ó·ÌÈÎfi ÛÈÓıËÚÔÁÚ¿ÊËÌ· MAG3. ∏ ‰È¿ÁÓˆÛË Ù˘ ÌÂÁ·Î·Ï‡Îˆ-
Û˘ ÂȂ‚·ÈÒÓÂÙ·È Ì ٷ Ù˘Èο Â˘Ú‹Ì·Ù·
ÛÙËÓ ÂÓ‰ÔÊϤ‚ÈÔ
‚¿-
ÚÔ˜ Ô‡ÚˆÓ ·fi ·Ó·Ú΋ ‰˘Ó·ÙfiÙËÙ· Û˘Ì‡ÎÓˆÛ˘, Èı·ÓÒ˜ ÏfiÁˆ ÂÏ¿ÙÙˆÛ˘ ÙÔ˘ ·ÚÈıÌÔ‡ ÙˆÓ ·Ú·Ì˘ÂÏÈÎÒÓ ÓÂÊÚÒÓˆÓ (2). ∏ Ì·ÁÓËÙÈ΋ Ô˘ÚÔÁÚ·Ê›· (Magnetic Resonance Urography, MRU), ·Ú¤¯ÂÈ ÙË ‰˘Ó·ÙfiÙËÙ· Ï‹„˘ ÙfiÛÔ ·Ó·ÙÔÌÈÎÒÓ fiÛÔ Î·È ÏÂÈÙÔ˘ÚÁÈÎÒÓ ÏËÚÔÊÔ-
ÚÈÒÓ Î·È ÈÛÙ‡ÂÙ·È fiÙÈ ÛÙÔ Ì¤ÏÏÔÓ ı· Û˘Ì‚¿-
ÏÂÈ ÛÙË ‰È¿ÁÓˆÛË ·˘Ù‹˜ Ù˘ Û¿ÓÈ·˜ ·ÓˆÌ·-
Ï›·˜, ηıÒ˜ Î·È ¿ÏÏˆÓ Û‡ÓıÂÙˆÓ Ô˘ÚÔÏÔÁÈÎÒÓ
·ÓˆÌ·ÏÈÒÓ (Riccabona/04).
ªÂÙ¿ ·fi ÙËÓ ÚÒÙË ÂÚÈÁÚ·Ê‹ Ù˘ ÌÂÁ·-
ηχΈÛ˘ ·fi ÙÔÓ Puigvert ÙÔ 1964, ¤¯Ô˘Ó
ÂÚÈÁÚ·Ê› Ï›Á˜ ÂÚÈÙÒÛÂȘ. ∏ ‰È¿ÁÓˆÛË Û˘Ó‹ıˆ˜ Á›ÓÂÙ·È ÛÙ· Ï·›ÛÈ· ·ÂÈÎÔÓÈÛÙÈÎÔ‡ ÂϤÁ¯Ô˘ ·È‰ÈÒÓ Ì ԢÚÔÏԛ̈ÍË ‹ Ì ÚÔÁÂÓ-
ÓËÙÈο ‰È·ÁÓˆṲ̂ÓË ˘‰ÚÔÓ¤ÊÚˆÛË (4,5). ™˘Ó‹ıˆ˜ Â›Ó·È ÂÙÂÚfiÏ¢ÚË (80%) Î·È ‰È·ÈÛÙÒÓÂÙ·È ÈÔ Û˘¯Ó¿ ÛÙ· ·ÁfiÚÈ· (90%) (5). ∏ ·ÈÙÈÔÏÔÁ›· Ù˘ Â›Ó·È ¿ÁÓˆÛÙË, ¤¯ÂÈ fï˜ ·Ô‰Ôı› Û ηı˘ÛÙ¤ÚËÛË Ù˘ ·ӷ˘ÏÔÔ›ËÛ˘ ÙÔ˘ ¿Óˆ ÙÌ‹Ì·ÙÔ˜ ÙÔ˘ ÂÌ‚Ú˘˚ÎÔ‡ Ô˘ÚËÙ‹Ú· ÌÂÙ¿ ÙË Û‡Ó‰ÂÛË ÙÔ˘ ÂÌ‚Ú˘˚ÎÔ‡ ÎÔÌ‚›Ô˘ ÛÙÔÓ ÌÂÙ¿ÓÂÊÚÔ (2). ™˘¯Ó¿ ˘¿Ú¯ÂÈ ÔÈÎÔÁÂÓÂȷ΋ ÂÈ‚¿Ú˘ÓÛË Î·È ¤¯ÂÈ ÂÚÈÁÚ·Ê› ÎÏËÚÔÓÔÌÈÎfiÙËÙ· Ì ÙÔÓ ˘ÔÏÂÈfiÌÂÓÔ ÛˆÌ·ÙÈÎfi ¯·Ú·ÎÙ‹Ú· (6). ∏ ÌÂÁ·Î·Ï‡ÎˆÛË Â›Ó·È Û˘Ó‹ıˆ˜ Ì›· ÌÂÌÔӈ̤ÓË ·ÓˆÌ·Ï›·, fï˜, ¤¯ÂÈ ‰È·ÈÛÙˆı› Î·È Û ·È‰È¿ Ì ۇÛÙÔÈ¯Ô ÌÂÁ·Ô˘ÚËÙ‹Ú· (5), ÏÂÈÔ΢ÛÙÈÎfi ‰˘ÛÏ·ÛÙÈÎfi ÓÂÊÚfi (6) ‹ Ì ۇӉÚÔÌÔ Schinzel-Giedion (ÚÔ˚Ô‡Û· Ó¢ÚÔÂÎÊ˘ÏÈÛÙÈ΋ ÓfiÛÔ˜ Ì ÔÙÈΤ˜ Î·È ÔÛÙÈΤ˜ ‚Ï¿‚˜) (7). ∏ ÚfiÁÓˆÛË Â›Ó·È Î·Ï‹. √È ·ÛıÂÓ›˜ ¤¯Ô˘Ó Ê˘ÛÈÔÏÔÁÈ΋ ÓÂÊÚÈ΋ ÏÂÈÙÔ˘ÚÁ›·, fï˜, Ë ·ÓˆÌ·Ï›· ·˘Ù‹ ıˆÚÂ›Ù·È fiÙÈ Úԉȷı¤ÙÂÈ Û ԢÚÔÏÔÈÌÒÍÂȘ Î·È ÓÂÊÚÔÏÈı›·ÛË. ŒÙÛÈ, ÔÈ ·ÛıÂÓ›˜ Ì ÙÔ Úfi‚ÏËÌ· ·˘Ùfi Ú¤ÂÈ Ó· ·Ú·ÎÔÏÔ˘ıÔ‡ÓÙ·È Û˘ÛÙËÌ·ÙÈο, ÙfiÛÔ ÎÏÈÓÈο, fiÛÔ Î·È ˘ÂÚ˯ÔÁÚ·ÊÈο. ∏ Û¿ÓÈ· ·˘Ù‹ ·ÓˆÌ·Ï›· ÙÔ˘ Ô˘ÚÔÔÈËÙÈÎÔ‡ ÁÈ· ÙÔ˘˜ ÂÚÈÛÛfiÙÂÚÔ˘˜ ·È‰È¿ÙÚÔ˘˜ Â›Ó·È Ì›· “ͯ·Ṳ̂ÓË ‰È¿ÁÓˆÛË” [1]. ∆· ·ÂÈÎÔÓÈÛÙÈο Â˘Ú‹Ì·Ù· Â›Ó·È Ù˘Èο Î·È Ú¤ÂÈ Ë ÌÂÁ·Î·Ï‡ÎˆÛË Ó· Û˘ÌÂÚÈÏ·Ì‚¿ÓÂÙ·È ÛÙË ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË ÙÔ˘ ÓÂÔÁÓÔ‡ Ì ÚÔÁÂÓÓËÙÈ΋ ˘‰ÚÔÓ¤ÊÚˆÛË. ¶ÚfiÛÊ·Ù· ¤¯ÂÈ ‰È·ÈÛÙˆı› ·Ó·Óˆ̤ÓÔ ÂӉȷʤÚÔÓ ÁÈ· ÙË ÌÂÁ·Î·Ï‡ÎˆÛË, ÁÈ·Ù› Ì ÙË ÛˆÛÙ‹ ‰È¿ÁÓˆÛË ·ÔʇÁÔÓÙ·È ÔÈ ÂÚÈÙÙ¤˜ ÂÍÂÙ¿ÛÂȘ Î·È ¯ÂÈÚÔ˘ÚÁÈΤ˜ ÂÂÌ‚¿ÛÂȘ. µÈ‚ÏÈÔÁÚ·Ê›·
1.Redman JF, Neeb AD. Congenital megacalycosis: a forgotten diagnosis? Urology 2005;65:384-385.
2.Kasap B, Kavukcu S, Soylu A, Turkmen M, Secil M. Megacalycosis: report of two cases. Pediatr Nephrol 2005;20:828-830.
3.Alivanis P, Giannikouris I, Arvanitis A, Volanaki M, Zervos A, Paliuras C. Bilateral congenital megacalycosis in a young male patient. Nephrol Dial Transplant 2006;21:1446-1447.
4.Hill LM, Macpherson T, Romano L, Gahagan S. Prenatal sonographic findings of fetal megacalycosis. J Ultrasound Med 2002;21:1179-1181.
5.Vidal Company A, Gonzalvez Pinera J, Ruiz Cano R, Gutierrez Junquera C, Lillo Lillo M. Megacalycosis
diagnosed during the study of antenatal hydronephrosis. An Esp Pediatr 2001;54:74-77.
6.Lam AH. Familial megacalyces with autosomal recessive inheritance. Pediatr Radiol 1988;19:28-30.
7.Mandell GA, Snyder HM 3rd, Heyman S, Keller M, Kaplan JM, Norman ME Association of congenital megacalycosis and ipsilateral segmental megaureter. Pediatr Radiol 1987;17:28-33.
8.Perera Soler R, Ruiz Gonzalez AP, Molini Menchon N, Garcia Nieto VM. Unilateral multicystic dysplastic kidney and contralateral megacalycosis. An unusual association. An Pediatr 2004;60:473-476.
9.Minn D, Christmann D, De Saint-Martin A, Alembik Y, Eliot M, Fischbach M, et al. Further clinical and sensorial delineation of Schinzel-Giedion syndrome: report of two cases. Am J Med Genet. 2002 1;109: 211-217.
4-7 ™ÂÙÂÌ‚Ú›Ô˘ 2006 30th UMEMPS Congress
Damascus,
Contact: Eng Rasha Hasan Syria
Tel.: 00963 9274 5746
Fax: 00963 1123 13326
E-mail: info@umemps30-sy.com
Website: www.umemps30-sy.com
14-16 ™ÂÙÂÌ‚Ú›Ô˘ 2006 5th International Neonatal Hematology and Brescia, Immunology Meeting Italy
Contact: Gaetano Chirico
Tel.: 39-0-303-995-219
Fax: 39-0-303-700-817
E-mail: gaechir@tin.it
18-21 ™ÂÙÂÌ‚Ú›Ô˘ 2006 38th Congress of the International Society Geneva, of Paediatric Oncology Switzerland
Contact: Sandrine Haemmerli
Tel.: 41 223 399 648
Fax: 41 223 399 601
E-mail: siop2006@mci-group.com
21-23
Hotel Grecotel Athens Imperial ¶ÏËÚÔÊÔڛ˜:
∆ËÏ./Fax: 210 7794023
√ÚÁ¿ÓˆÛË: PRC Congress & Travel,
∆ËÏ.: 210 7711673
Fax: 210 7711289
E-mail: secretariat@prctravel.gr
Ù˘ ∂ÏÏËÓÈ΋˜
•ÂÓÔ‰Ô¯Â›Ô Aldemar Olympian Village
¢ÈÔÚÁ¿ÓˆÛË Û˘Ó‰ڛԢ: Firstevent
Prousis 3 st, 10440 Athens, Greece
Tel.:+30 2108228950
Fax: +30 2108228901
E-mail: info@firstevent.gr
Website: www.firstevent.gr
π·ÙÚÈ΋ ™¯ÔÏ‹, ¶·ÓÂÈÛÙ‹ÌÈÔ ∞ıËÓÒÓ
ª. ∞Û›·˜ 75, 11527 ∞ı‹Ó·
∆ËÏ.: 210-7462201
Fax: 210-7462105
5-8 √ÎÙˆ‚Ú›Ô˘ 2006
Advances in Paediatric Gastroenterology, Cappadocia, Hepatology & Nutrition Turkey
Turkish - Greek Joint Postgraduate Course endorsed by ESPGHAN
¶ÏËÚÔÊÔڛ˜: ∞ÏÂÍ¿Ó‰Ú· ¶··‰ÔÔ‡ÏÔ˘,
¶Úfi‰ÚÔ˜ ∂∂¶°∏¢
∆ËÏ.: 210 77 26 355
E-mail: papadop5@otenet.gr
∂ÁÁڷʤ˜: Prof. Ender Pehlivanoglou, Marmara University, Istanbul
Fax: 90 212 296 5481
E-mail: e.pehlivanoglu@tnn.net
7-10 √ÎÙˆ‚Ú›Ô˘ 2006 40th Meeting of the European Society of Palermo, Paediatric Nephrology Italy
¶ÏËÚÔÊÔڛ˜: E.DI.PO. s.r.l.Via della Libertà,
103 - 90143 Palermo
∆ËÏ.: ++39 091 340255
Fax: ++39 091 340440
E-mail: edi.po@tiscali.it Website: www.espn2006.org/
29-31 √ÎÙˆ‚Ú›Ô˘ 2006 International Conference on Childhood Cancer Tehran, Contact: Dr. Alireza Mosavi-Jarrahi Iran
∆el.: 98 2 122 481 011
Fax: 98 2 122 481 010
E-mail: rmosavi@yahoo.com
8-11 √ÎÙˆ‚Ú›Ô˘ 2006
European Academy of Paediatrics – CESP 2006 Barcelona, Centre Convencions Internacional Barcelona Spain
¶ÏËÚÔÊÔڛ˜: Kenes International / Europaediatrics 2006
17 Rue du Cendrier
PO Box 1726, CH-1211
Geneva 1, Switzerland
Tel: +41 22 908 0488
Fax: +41 22 732 2850
E-mail: europaediatrics@kenes.com Website: www.kenes.com/europaediatrics
9-10 √ÎÙˆ‚Ú›Ô˘ 2006 ∞ÓÙÈÌÂÙÒÈÛË ·È‰ÈÒÓ Ì ¢È·Ù·Ú·¯‹ ∂ÏÏÂÈÌÌ·ÙÈ΋˜ ∞ı‹Ó·
¶ÚÔÛÔ¯‹˜-ÀÂÚÎÈÓËÙÈÎfiÙËÙ·˜ ·fi
∆ËÏ.: 210 7211845
º·Í: 210 7215082
E-mail: info@epsyype.gr Website: www.epsyype.gr
28 √ÎÙˆ‚Ú›Ô˘ - 18th International Congress on Pediatrics Tehran, 2 ¡ÔÂÌ‚Ú›Ô˘ 2006
Contact: Dr Gh. Valizadeh Iran
Tel.: 98 2 166 428 998
Fax: 98 2 166 923 054
E-mail: pedcong@tums.ac.ir
