Editors/ Uniform Requirements for Manuscripts Sub-mitted to Biomedical Journals, (http://www.icmje.org Î·È http:// www.icmje.org/icmje.pdf). OÈ Û˘ÓÙÌ‹ÛÂȘ ÙˆÓ Ù›ÙÏˆÓ ÙˆÓ ÂÚÈÔ‰ÈÎÒÓ Á›ÓÔÓÙ·È Ì ‚¿ÛË ÙÔ Cumulated Index Medicus [List of Journals Indexed in Index Medicus (http://www.nlm.nih.gov/bsd/uniform requirements.html)].
Proesmans W. Bartter syndrome and its neonatal variant. Eur J Pediatr 1997;156:669-679.
™˘ÌÏËڈ̷ÙÈÎfi Ù‡¯Ô˜ ÂÚÈÔ‰ÈÎÔ‡:
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.
Èڛ˜ Û˘ÁÁڷʤ·:
National Institutes of Health Consensus Development Conference. Neurofibromatosis conference statement. Arch Neurol 1988;45:575-578.
¶ÚÔÛ‰ÈÔÚÈÛÌfi˜ Ù‡Ô˘ ¿ÚıÚÔ˘:
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. µπµ§π∞
∫ÂÊ¿Ï·ÈÔ Û ‚È‚Ï›Ô:
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.
™‡ÁÁÚ·ÌÌ· ‹ ÌÔÓÔÁÚ·Ê›·:
Gorlin RJ, Cohen MM, Levin LS. Syndromes of the head and neck. 3rd ed. New York: Oxford University Press; 1990.
¢ËÌÔÛ›Â˘ÛË Û ÙfiÌÔ Ú·ÎÙÈÎÒÓ: 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.
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.™∆O ¢π∞¢π∫∆ÀO ÕÚıÚÔ Û ÂÚÈÔ‰ÈÎfi:
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
ªÔÓÔÁÚ·Ê›·:
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
πÛÙÔÛÂÏ›‰Â˜: Cancer-Pain.org [Webpage, Internet]. New York: Association of Cancer Online Resources, Inc.; 2002: http://www.cancer-pain.org/
A o angstromangstrom calıÂÚÌ›‰·calorie cmÂηÙÔÛÙficentimeter cm2
ÙÂÙÚ·ÁˆÓÈÎfi ÂηÙÔÛÙfisquare centimeter cm3
΢‚ÈÎfi ÂηÙÔÛÙficubic centimeter oC ‚·ıÌfi˜ ∫ÂÏÛ›Ô˘degree Celsius gÁÚ·ÌÌ¿ÚÈÔgram h ÒÚ·hour IU‰ÈÂıÓ‹˜ ÌÔÓ¿‰·international unit kg¯ÈÏÈfiÁÚ·ÌÌÔkilogram l Ï›ÙÚÔliter m ̤ÙÚÔmeter mg¯ÈÏÈfiÁÚ·ÌÌÔmilligram minÏÂÙfiminute mm¯ÈÏÈÔÛÙfiÌÂÙÚÔmillimeter molÁÚ·ÌÌÔÌfiÚÈÔmole n ·ÚÈıÌfi˜number
NS ‹ ª™ÌË ÛËÌ·ÓÙÈÎfinot significant osmÔÛÌÒÏÈÔosmole p Èı·ÓfiÙËÙ·probability SDÛÙ·ıÂÚ‹ ·fiÎÏÈÛËstandard deviation SEÛÙ·ıÂÚfi ÛÊ¿ÏÌ·standard error sec‰Â˘ÙÂÚfiÏÂÙÔsecond U ÌÔÓ¿‰·unit
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 and case reports 1500-2500 words;
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.
1.Steinberg MH, Rodgers GP. Pathophysiology of sickle cell disease: role of cellular and genetic modifiers. Semin Hematol 2001;38:299-306.
2.Bunn FH. Human hemoglobins: sickle hemoglobin and other mutants. In: Stamatoyannopoulos G, Majerus WP, Perlmutter MR, Varmus H, editors. The molecular basis of blood diseases. 3rd ed. Philadelphia: WB Saunders Company; 2001. p. 227-270.
3.Vichinsky E. New therapies in sickle cell disease. Lancet 2002;360:629-631.
4.Elion JE, Brun M, Odievre MH, Lapoumeroulie CL, Krishnamoorthy R. Vaso-occlusion in sickle cell anemia: role of interactions between blood cells and endothelium. Hematol J 2004;5 (Suppl 3):S195-S198.
6.de Franceschi L, Baron A, Scarpa A, Adrie C, Janin A, Barbi S et al. Inhaled nitric oxide protects transgenic SAD mice from sickle cell disease-specific lung injury induced by hypoxia/reoxygenation. Blood 2003;102:1087-1096.
7.Moore CM, Ehlayel M, Leiva LE, Sorensen RU. New concepts in the immunology of sickle cell disease. Ann Allergy Asthma Immunol 1996;76:385-400.
8.Ballas SK, Smith ED. Red blood cell changes during the evolution of the sickle cell painful crisis. Blood 1992;79:2154-2163.
9.Walters MC, Nienhuis AW, Vichinsky E. Novel therapeutic approaches in sickle cell disease. Hematology 2002;1:10-34.
10.Brown MD, Wick TM, Eckman JR. Activation of vascular endothelial cell adhesion molecule expression by sickle blood cells. Pediatr Pathol Mol Med 2001;20:47-72.
11.Hebbel RP. Beyond hemoglobin polymerization: the red blood cell membrane and sickle disease pathophysiology. Blood 1991;77:214-237.
12.Ruoslahti E. Integrins. J Clin Invest 1991;87:1-5.
13.Joneckis CC, Ackley RL, Orringer EP, Wayner EA, Parise LV. Integrin alpha 4 beta 1 and glycoprotein IV (CD36) are expressed on circulating reticulocytes in sickle cell anemia. Blood 1993;82:3548-3555.
14.Sultana C, Shen Y, Rattan V, Johnson C, Kalra VK. Interaction of sickle erythrocytes with endothelial cells in the presence of endothelial cell conditioned medium induces oxidant stress leading to transendothelial migration of monocytes. Blood 1998;92:3924-3935.
15.Phelan M, Perrine SP, Brauer M, Faller DV. Sickle erythrocytes, after sickling, regulate the expression of the endothelin-1 gene and protein in human endothelial cells in culture. J Clin Invest 1995;96:11451151.
16.Solovey A, Gui L, Key NS, Hebbel RP. Tissue factor expression by endothelial cells in sickle cell anemia. J Clin Invest 1998;101:1899-1904.
17.Solovey A, Lin Y, Browne P, Choong S, Wayner E, Hebbel RP. Circulating activated endothelial cells in sickle cell anemia. N Engl J Med 1997;337:15841590.
18.Kurantsin-Mills J, Ofosu FA, Safa TK, Siegel RS, Lessin LS. Plasma factor VII and thrombin-antithrombin III levels indicate increased tissue factor activity in sickle cell patients. Br J Haematol 1992;81:539-544.
19.Kaul DK, Nagel RL, Chen D, Tsai HM. Sickle erythrocyte-endothelial interactions in microcirculation: the role of von Willebrand factor and implications for vasoocclusion. Blood 1993;81:2429-2438.
20.Okpala I, Daniel Y, Haynes R, Odoemene D, Goldman J. Relationship between the clinical manifestations of sickle cell disease and the expression of adhesion molecules on white blood cells. Eur J Haematol 2002;69:135-144.
21.Turhan A, Weiss LA, Mohandas N, Coller BS, Frenette PS. Primary role for adherent leukocytes in sickle cell vascular occlusion: a new paradigm. Proc Natl Acad Sci USA 2002;99:3047-3051.
22.Hofstra TC, Kalra VK, Meiselman HJ, Coates TD. Sickle erythrocytes adhere to polymorphonuclear neutrophils and activate the neutrophil respiratory burst. Blood 1996;87:4440-4447.
23.Agarwal G, Wang JC, Kwong S, Cohen SM, Ferrone FA, Josephs R et al. Sickle hemoglobin fibers: mechanisms of depolymerization. J Mol Biol 2002;322:395-412.
24.Browne PV, Hebbel RP. CD36-positive stress reticulocytosis in sickle cell anemia. J Lab Clin Med 1996;127:340-347.
25.Udani M, Zen Q, Cottman M, Leonard N, Jefferson S, Daymont C et al. Basal cell adhesion molecule/lutheran protein. The receptor critical for sickle cell adhesion to laminin. J Clin Invest 1998;101:2550-2558.
26.El Nemer W, Gane P, Colin Y, Bony V, Rahuel C, Galacteros F et al. The Lutheran blood group glycoproteins, the erythroid receptors for laminin, are adhesion molecules. J Biol Chem 1998;273:1668616693.
27.Ataga KI, Orringer EP. Hypercoagulability in sickle cell disease: a curious paradox. Am J Med 2003;115:721-728.
28.Setty BN, Rao AK, Stuart MJ. Thrombophilia in sickle cell disease: the red cell connection. Blood 2001;98:3228-3233.
29.Papadimitriou CA, Travlou A, Kalos A, Douratsos D, Lali P. Study of platelet function in patients with sickle cell anemia during steady state and vaso-occlusive crisis. Acta Haematol 1993;89:180-183.
30.Wun T, Paglieroni T, Tablin F, Welborn J, Nelson K, Cheung A. Platelet activation and platelet-erythrocyte aggregates in patients with sickle cell anemia. J Lab Clin Med 1997;129:507-516.
31.Carlos TM, Harlan JM. Leukocyte-endothelial adhesion molecules. Blood 1994;84:2068-2071.
32.Imhof BA, Dunon D. Leukocyte migration and adhesion. Adv Immunol 1995;58:345-416.
33.Rybicki AC, Musto S, Swinson G. Increased levels of endothelin-1 in plasma of sickle cell anemia patients. Blood 1991;78:202.
34.Smolinski PA, Offermann MK, Eckman JR, Wick TM. Double-stranded RNA induces sickle erythrocyte adherence to endothelium: a potential role for viral infection in vaso-occlusive pain episodes in sickle cell anemia. Blood 1995;5:2945-2950.
35.Francis RB. Large-vessel occlusion in sickle cell disease: pathogenesis, clinical consequences, and therapeutic implications. Med Hypotheses 1991;35:88-95.
36.Adams R, McKie V, Nichols F, Carl E, Zhang DL, McKie K et al. The use of transcranial ultrasonography to predict stroke in sickle cell disease. N Engl J Med 1992;326:605-610.
37. Hess DC, Zhao W, Carroll J, McEachin M, Buchanan K. Increased expression of ICAM-1 during reoxygenation in brain endothelial cells. Stroke 1994;25:1463-1467.
38.Vichinsky EP, Neumayr LD, Earles AN, Williams R, Lennette ET, Dean D et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med 2000;342:1855-1865.
39.Stuart MJ, Setty BN. Acute chest syndrome of sickle cell disease: new light on an old problem. Curr Opin Hematol 2001;8:111-122.
40.Gladwin MT, Rodgers GP. Pathogenesis and treatment of acute chest syndrome of sickle-cell anaemia. Lancet 2000;355:1476-1478.
41.Maitre B, Habibi A, Roudot-Thoraval F, Bachir D, Belghiti DD, Galacteros F et al. Acute chest syndrome in adults with sickle cell disease. Chest 2000;117:1386-1392.
42.Hebbel RP. Blockade of adhesion of sickle cells to endothelium by monoclonal antibodies. N Engl J Med 2000;342:1910-1912.
43.Stuart MJ, Setty BN. Sickle cell acute chest syndrome: pathogenesis and rationale for treatment. Blood 1999;94:1555-1560.
44.Hammerman SI, Klings ES, Hendra KP, Upchurch GR Jr, Rishikof DC, Loscalzo J et al. Endothelial cell nitric oxide production in acute chest syndrome. Am J Physiol 1999;277:1579-1592.
45.Gross PL, Aird WC. The endothelium and thrombosis. Semin Thromb Hemost 2000;26:463-478.
46.Stuart MJ, Setty BN. Sickle cell acute chest syndrome: pathogenesis and rationale for treatment. Blood 1999;94:1555-1560.
47.Bellet PS, Kalinyak KA, Shukla R, Gelfand MJ, Rucknagel DL. Incentive spirometry to prevent acute pulmonary complications in sickle cell diseases. N Engl J Med 1995;333:699-703.
48.Hammerman SI, Kourembanas S, Conca TJ, Tucci M, Brauer M, Farber HW. Endothelin-1 production during the acute chest syndrome in sickle cell disease. Am J Respir Crit Care Med 1997;156:280-285.
51.Haynes J Jr, Obiako B. Activated polymorphonuclear cells increase sickle red blood cell retention in lung: role of phospholipids. Am J Physiol Heart Circ Physiol 2002;282:122-130.
52.Morris CR, Kuypers FA, Larkin S, Sweeters N, Simon J, Vichinsky EP et al. Arginine therapy: a novel strategy to induce nitric oxide production in sickle cell disease. Br J Haematol 2000;111:498-500.
53.Kanakoudi-Tsakalidou F, Athanasiou-Metaxa M, Tzimouli V, Koussi A, Taparkou M, Trachana M et al. Effect of cetrizin on circulating indicators of persistent inflammation and endothelial cell activation in patients with sickle cell disease. Ped Res 1999;45:755.
1 3rd Paediatric Clinic, Aristotle University, Ippokration General Hospital, Thessaloniki
2 1st Paediatric Clinic, Aristotle University, Ippokration General Hospital, Thessaloniki
Correspondence:
∂. Karatza-Xifilidou
Ippokration General Hospital 49 Konstantinoupoleos Str. 546 42, Thessaloniki
Date of submission:
03-09-2004
Date of approval: 01-04-2005
Pathophysiology of vaso-occlusive crises in sickle cell disease
E. Karatza - Xifilidou1, M. Athanasiou - Metaxa2
Abstract
Sickle cell disease is caused by a mutation which is characterized by the replacement of glutaminic acid by valine in the 6th position of the ‚-chain of haemoglobin (‚6glu val) and the production of haemoglobin S (·2‚2S). The exclusive characteristic of haemoglobin S, when deoxygenated, is polymerization of the chains and, as a result, production of dehydrated rigid sickle cells. Until the beginning of the past decade, the rigid sickle cells were thought to play the exclusive role in the pathogenesis of the painful thrombotic crises of the disease. Newer studies have proved that thrombotic episodes are the result of the activation of a complex multifactorial mechanism. At the onset of these episodes, stress reticulocytes attach to the endothelium of the vessels in the microcirculatory system and the rigid sickle cells become trapped in an attempt at their removal from circulation. The establishment of the initial thrombus is controlled by: a) factors from the red blood cell membrane, which is damaged by hypoxia, and parallel activation of the plasma coagulation factors, b) factors from the endothelium of the vessels (antagonism between vasopressor and vasodilator factors), c) surface adhesion molecules of the endothelial cells and the stress reticulocytes, which contribute to the initial attachment of reticulocytes to the endothelium, and d) activation and attachment of other blood cells (platelets and white cells). Study of the interaction of all these factors has helped in the understanding of the pathogenesis of vaso-occlusive episodes, resulting in better monitoring, treatment approaches, quality of life and survival of patients suffering from sickle cell disease.
2.Primeau MN, Adkinson NF Jr. Recent advances in the diagnosis of drug allergy. Curr Opin Allergy Clin Immunol 2001;1:337-341.
3.Gruchalla RS. Understanding drug allergies. J Allergy Clin Immunol 2000;105:637-644.
4.Grattan CE. Aspirin sensitivity and urticaria. Clin Exp Dermatol 2003;28:123-127.
5.Torres MJ, Blanca M, Fernandez J, Romano A, Weck A, Aberer W et al. EAACI Interest Group on Drug Hypersensitivity. Diagnosis of immediate allergic reactions to beta-lactam antibiotics. Allergy 2003;58:961-972.
6.Impicciatore P, Choonara I, Clarkson A, Provasi D, Pandolfini C, Bonati M. Incidence of adverse drug reactions in paediatric in/out-patients: a systematic review and meta-analysis of prospective studies. Br J Clin Pharmacol 2001;52:77-83.
7.deShazo RD, Kemp SF. Allergic reactions to drugs and biologic agents. JAMA 1997;278:1895-1906.
8.Cetinkaya F. Sensitivity to local anaesthetics among asthmatic children. Int J Paediatr Dent 2001;11:405408.
9.Macy E, Burchette RJ. Oral antibiotic adverse reactions after penicillin skin testing: multi-year followup. Allergy 2002;57:1151-1158.
10.Aberer W, Bircher A, Romano A, Blanca M, Campi P, Fernandez J et al. European Network for Drug Allergy (ENDA). EAACI Interest Group on Drug Hypersensitivity. Drug provocation testing in the diagnosis of drug hypersensitivity reactions: general considerations. Allergy 2003;58:854-863.
Evaluation of oral challenges for the diagnosis of drug allergy
A. Sofianou - Katsoulis1, G. Du Toit2, G. Lack3
Abstract
Background: Allergic reactions to drugs in the paediatric population are serious and potentially life threatening events that are often poorly diagnosed and managed. This is due to a lack of validated and readily available diagnostic tests, as well as of consensus guidelines for their management.
Methods: This was a retrospective study of 22 patients who underwent provocation tests for the diagnosis of reported drug allergy. The testing was made at St. Mary’s Hospital, London. The tests were predominantly oral challenges with the exception of lidocaine/procaine, for which a skin test was performed. Most of the tests were done to test penicillin allergy (40%). Three quarters were single compared to combined challenges.
Results: Six patients (28%) reacted, five of whom had an immediate and one a delayed reaction. None of them had a reaction upon re-exposure to a suggested medication. The parents of the children who had a negative challenge felt that the outcome was reassuring. For those whose children had a positive outcome, it enhanced their confidence in the use of alternative drugs.
Conclusions: Drug challenges need to be performed at a specialized centre particularly in patients where the history is equivocal. Many of the present diagnostic tests need validation prior to their generalization. Larger cohort studies are also required to establish the long-term prognosis in these cases.
Key words
Drug challenges, drug allergy, adverse events.
1 Paediatric Specialist Registrar, St. Mary’s Hospital, London, UK
2 Consultants in Paediatric Allergy, Department of Paediatric Allergology and Immunology, St. Mary’s Hospital, London, UK
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V. Tsagris1, A. Kitsopoulou2, C. Mihas2, P. Nikolaidou - Karpathiou1
Abstract
Fibromyalgia of childhood is a common musculoskeletal pain disorder characterized by widespread persistent pain, the presence of multiple discrete tender points on physical examination, stiffness, sleep disturbances and fatigue. Its course is unremitting and it has a restrictive effect on the functional ability and psychosocial development of the affected children. Although its exact aetiology remains unknown, it is perceived that a number of factors can contribute to the clinical expression of the disorder. The therapeutic intervention is non-specific and usually multidisciplinary.
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Severe Guillain-Barré syndrome. Outcome in children admitted to a PICU
A. Tsoutsou, D. Gionis, S. Mastroyianni, J. Papadatos
Abstract
Background: Guillain-Barré syndrome (GBS) is a progressive symmetrical polyneuritis with acute onset, evolution over days or weeks and a good outcome in most cases. The aim of this study was to analyze the clinical course and outcome in children with GBS requiring hospitalization in a Paediatric Intensive Care Unit (PICU).
Methods: The medical records of all children admitted to the PICU between January 1986 and January 2003 were reviewed. The clinical and laboratory features, therapy and outcome were recorded.
Results: Twelve children, with GBS, 5 males, mean age 8.04 years (range 22 months - 15 years), were admitted to the PICU. In 8/12 patients a preceding viral illness was reported. In 10/12 patients serological investigations were performed and the aetiological factor was found in 6/10. In addition to weakness and loss of tendon reflexes, 11/12 children presented with cranial nerve palsy and 9/12 had autonomic dysfunction (mainly hypertension). All patients had a severity score (Hughes scale) of 4 or 5. A lumbar puncture was performed in all patients, with positive findings when it was done 7 days or more after the onset of symptoms. Eleven of the 12 patients required mechanical ventilation for a mean period of 18.4 days (range 2-37 days) and 10 were given intravenous immunoglobulin (IVIG). The mean period of PICU hospitalization was 24.8 days (range 2-42 days). None of the patients died, 10 had full recovery but two patients had neurological sequelae.
Conclusions: Guillain-Barré syndrome has a better outcome in children than in adults. Mechanical ventilation is not a risk factor for an adverse outcome.
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Risks of sports activities in individuals with Down syndrome and atlantoaxial instability
H. Hatzissevastou - Loukidou1, M. Badouraki2, P. Kambouridou1, E. Sotiridou1
Abstract
Background: The prevalence of atlantoaxial instability (AAI) among persons with Down syndrome (DS) ranges from 10% to 40% and is related to age and sex. The aim of this study was to investigate AAI in non-institutional people with DS in Northern Greece and to monitor them annually.
Methods: A total of 171 persons with DS, aged 0-40 years, were enrolled in the study. The patients were examined clinically in relation to the presence or absence of discomfort in the neck area and spinal cord compression. Eighty one per cent of those aged 3 years and older were screened radiographically at the cervical spine. A lateral neck X-ray was made in the neutral position, and in flexion when necessary. An atlantoaxial distance of greater than 4 mm for children under 15 years of age and greater than 3 mm for people over 15 years is suggested as the cut off point for AAI. All the X-rays were examined by the same radiologist.
Results: The prevalence of AAI was high (42%) in the group of people with DS, and it was more frequent in boys (56%) and in those under 15 years (63%). Local symptoms were reported by 9.3%. Investigation by computed tomography (CT) suggested that none of these had spinal cord compression.
Conclusions: The results of this study support the guidelines of Committee on Sports of American Academy of Pediatrics (AAP) which recommend screening of children over 3 years old with DS by cervical spine radiography at the initial visit to the paediatrician, and provision of guidance to the families about the participation of the children in sports (i.e., avoidance of extreme flexion or overstretching of the neck) and cervical X-ray screening before they start participation in sports activities. The physician should take a careful history and perform a thorough physical examination, looking for evidence of neurological signs and symptoms of spinal cord compression (easy fatiguability, difficulties in walking, abnormal gait, neck pain, limited neck mobility, torticollis or head tilt, incoordination and clumsiness, sensory deficits, spasticity, hyperreflexia, clonus, extensorplantar reflex, other upper motor neuron signs and changes in bowel or bladder control). Any findings on the physical examination should be investigated by repetition of cervical spine radiography, and further screening by MRI.
Key words
Down syndrome, atlantoaxial instability, atlantoaxial subluxation, atlantoaxial dislocation, X-ray, magnetic resonance imaging (MRI).
1 1st Paediatric Clinic of Aristotle University, “Ippokration” General Hospital, Thessaloniki 2 Paediatric Department of Radiology, “Ippokration” General Hospital, Thessaloniki
Correspondence: H. Hatzissevastou-Loukidou Laboratory of Cytogenetics, 1st Paediatric Clinic of Aristotle University, “Ippokration” General Hospital, 49 Konstantinoupoleos Str., 546 42, Thessaloniki
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Juvenile hyaline fibromatosis. The first case report in Greece
O. Papandreou1, A. Klimentopoulou1, N. Poniros2, A. Konstantinidou3, A. Xypolita - Zachariadi1, N. Rachman4, G. Chroussos1
Abstract
Juvenile hyaline fibromatosis is a rare connective tissue disease with autosomal recessive inheritance. The gene is located on the 4q21 chromosome. It usually presents during the first years of life with multiple painless subcutaneous nodules, hyalinous cutaneous lesions of various sizes, gingival hypertrophy, limb contractures and low bone density. The visceral organs are not affected. Mental development is normal. The diagnosis is confirmed by biopsy of the lesions. There is no specific treatment other than numerous operations for functional and aesthetic reasons. The case is described of a 4 year-old female with failure to thrive, limb contractures, rigid torsus, gingival hypertrophy, subcutaneous nodules, and cutaneous nodules and macules especially on the head, fingers and toes, causing considerable disability. Administration of Botox injections (Botulin toxinum A) has temporarily improved the patient’s quality of life, rendering her able to chew and speak again, as before.
Key words
Nodules, macules, fibromatosis.
1 1st Paediatric Clinic of the University of Athens, “Aghia Sophia” Children’s Hospital, Athens
2 Plastic Surgery Clinic, “Aghia Sophia” Children’s Hospital, Athens
3 Laboratory of PathologyAnatomy, “Aghia Sophia” Children’s Hospital, Athens
4 Institute of Cancer Research, Surrey, UK
Correspondence: Ourania Papandreou 1st Paediatric Clinic of the University of Athens, “Aghia Sophia” Children’s Hospital
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1 Neonatology Department
¡∏π, Hippokration General Hospital, Thessaloniki
2 Neonatology Department, University Hospital of Larissa
3 Molecular Biology and Cytogenetics Center, “Leto” Maternity Clinic, Athens
Correspondence: Alexandros Andreou
15 Heimonidou Str. 551 33, Kalamaria, Thessaloniki
∂-mail: aalex@the.forthnet.gr
Date of submission:
27-09-2004
Date of approval: 29-03-2005
22q11.2 micro-deletion syndrome in a family with variation in the phenotype. Prenatal diagnosis
A.
Andreou1, V. Alexandropoulou - Tsikrika2, A. Sakellaropoulou1, M. Goudesidou2, L. Florentin - Arar3
Abstract
A family is described in which chromosome deletion 22q11.2 was identified in three of its members, with a great heterogeneity in the phenotype. The mother had velo-pharyngeal insufficiency with nasal voice, the third child had DiGeorge syndrome with severe congenital cardiopathy, hypocalcaemia, and aplasia of the thymus gland and died of severe sepsis, and the fourth child had micrognathia and transient hypocalcaemia. The diagnosis was established first in the third child and subsequently in the mother. On the fourth child the diagnosis was made prenatally, after amniocentesis. In all cases the diagnosis was made with the FISH technique.
Key words
22q11 deletion syndrome, familial appearance, prenatal diagnosis, FISH technique.
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The effect of postnatal administration of steroids on the developing brain
H. Bouza, C. Papagaroufalis
Abstract
Postnatal steroid therapy has been widely used for the prevention and treatment of chronic lung disease (bronchopulmonary dysplasia) in preterm infants. Recently, the meta-analyses of the Cochrane Collaboration suggest that the risks of therapy may outweigh the possible benefits, especially if treatment is started before 96 hours of age. The meta-analyses show that treatment at 714 days of life seems to have the best benefit to risk ratio. However, since steroids may have neurotoxic effects on the developing brain, neonatologists must continue to search for alternative therapies for infants with chronic lung disease. Meanwhile, despite the limitations of steroid treatment, the European Association of Perinatal Medicine, the American Academy of Pediatrics and the Canadian Pediatric Society guidelines provide the most realistic and balanced advice on its use for the clinical neonatologist trying to clarify a confusing situation.
Key words
Corticosteroids, neonate, brain.
Correspondence:
2nd
E-mail: bnicu@paidon-agiasofia.gr
Date of submission: 21-06-2004
Date of approval: 17-06-2005
2nd Neonatal Intensive Care Unit, “Aghia Sophia” Children’s Hospital, Athens
Helen Bouza
Neonatal Intensive Care Unit, “Aghia Sophia” Children’s Hospital, 115 27, Athens
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1st Paediatric Clinic, Aristotle University of Thessaloniki, “Ippokration” General Hospital, Thessaloniki
Correspondence: H. Hatzissevastou-Loukidou Laboratory of Cytogenetics, 1st Paediatric Clinic, Aristotle University of Thessaloniki, “Ippokration” General Hospital, Thessaloniki 49 Konstantinoupoleos Str., 546 42, Thessaloniki
Date of submission: 05-11-2004
Date of approval: 29-03-2005
Genetic preventive testing and ethical issues
H. Hatzissevastou - Loukidou
Abstract
In the absence of clearly beneficial treatments or effective preventive strategies for many childhood diseases, the use of new genetic technology for newborn screening, carrier testing and testing for susceptibility to late-onset conditions must be considered carefully. Paediatricians will need to evaluate critically the diagnostic and prognostic implications of testing. Additional research and education in this developing area of medicine are encouraged.
