Four monthly scientific journal of the Greek Paediatric Society
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A.V.Kozlov, J.C. Duvigneau, S. Dumitrescu, A. Weidinger,
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Volume 80 | Number 2 | May - June - July - August 2017
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EDITORIAL
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088
RESEARCH STUDIES
Longitudinal changes of inflammatory-hormonal response innate-immunity bioenergetics and metabolism in critically ill children with severe sepsis
A.M. Spanaki, T. Tavladaki, H. Dimitriou, E. Blevrakis, A.V. Kozlov, J.C. Duvigneau, S. Dumitrescu, A. Weidinger, G. Briassoulis
100
Oral hygiene habits among 12 years old (grade 6th) school children in elementary schools of western
Soultana Georga, Venetia Sotiri, Dionysis Karakaidos, Chrisavgi Liouli, Eleni Kanavoura, Petros Almagout, Lykourgos Kanaris
112
REVIEW ARTICLES
The child with short stature Sofia Leka-Emiris, Elpis-Athina Vlachopapadopoulou, Stefanos Michalacos
040
Cardiovascular manifestations in children with neurocutaneous syndromes Maria Gogou, Andreas Giannopoulos
120
EPIDEMIOLOGICAL STUDY
(Pro) action plan for HPV vaccination How the Theory of Planned Behavior contributes as a method of planning health interventions Christodoulou Panayiotis
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Congenital polycythemia with elevated erythropoietin levels
Vassiliki-Sotiria Tzotzola, Ioannis Panagiotou, Maria Ambatzidou, Sofia Polichronopoulou
140
Gianotti-Crosti syndrome associated with streptococcal infection Klimi Eleni
144
CASE REPORT IN PICTURES
Cheloids of considerable size in the ear lobe of an adolescent, after ear piercing
Matheos Angelakis
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150
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e-mail:
Kozlov, J.C. Duvigneau, S. Dumitrescu, A. Weidinger,
(Systemic inflammatory response syndrome, SIRS)
με SIRS καθώς και υγιή παιδιά-controls (H). Υλικά/Μέθοδοι: Μελετήθηκαν 62 παιδιά (SS/15, SIRS/20, H/27) στην 1, 3 και 5 ημέρα της νοσηλείας τους. Υπολογίστηκαν τα scores δείκτη
(Body mass index (BMI) z-scores) και βαρύτητας νόσου (PeLOD, APACHE, SOFA). Μετρήθηκαν: καρδιακή συσταλτικότητα (EF, SF), τροπονίνη (Tn), γαλακτικό
κατανάλωση ενέργειας (Energy expenditure, EE) με Gas Module E-COVX, ATP στα λευκά αιμοσφαίρια με τη δοκιμασία λουσιφεράσης (luciferase luminescent assay). Επίσης μετρήθηκαν τα επίπεδα γλουταμίνης και NO2/NO3 με υγρή χρωματογραφία υψηλής
λιπιδίων (TBARS) με
(HPLC),
resistin, Antiponectin
Heat Shock Proteins (HSP) με την ποσοτική ανοσοενζυμική μέθοδο sandwich enzyme-linked immunosorbent assay (ELISA), καθώς και οι ενδοκυττάριες HSP72, HSP90α με κυτταρομετρία ροής (flow cytometry).
Αποτελέσματα:
(p<0.05).
Συμπέρασμα:
A.V. Kozlov
S. Dumitrescu
A. Weidinger
Ludwig Boltzmann Institute for Experimental and Clinical Traumatology in the AUVA, Vienna, Austria
J.C. Duvigneau
University of Veterinary Medicine, Vienna, Austria
Correspondence
Άnna-Maria Spanaki
Κyra tis Ro 10, 71307
Κipoupoli, Ηraklio Crete
Τ. +306944962133
e-mail: spanakam@yahoo. gr
A.M. Spanaki, T. Tavladaki, H. Dimitriou, E. Blevrakis, A.V. Kozlov, J.C. Duvigneau, S. Dumitrescu, A. Weidinger, G. Briassoulis
Introduction: Cell stress induced by severe sepsis (SS) or systemic inflammatory response syndrome (SIRS) presents with acute inflammatory, hormonal, immune and metabolic derangements. Their relation with mitochondrial dysfunction has not been adequately studied.
Objectives: To examine the longitudinal inflammatory, hormonal, immune, bioenergy and metabolic changes in children with SS; to compare with those of children with SIRS or healthy controls (H). Methods: Blood samples obtained from 62 children (SS/15; SIRS/20; H/27) on days 1, 3 and 5. Body mass index (BMI) z-scores, PeLOD, APACHE, SOFA scores were calculated. Lactate, Tn, EF and SF were measured. Energy expenditure (EE) was determined by the Gas Module E-COVX, ATP-levels in white blood cells by luciferase luminescent assay, plasma glutamine and NO2/NO3 by HPLC; lipid peroxidation products (TBARS) by a colorimetric test. Intracellular Heat Shock Proteins (HSP72, HSP90α) expressions by flow cytometry; serum HSP72, adiponectin and resistin by ELISA.
A. M. Spanaki
T. Tavladaki
E. Blevrakis
G. Briassoulis
PICU, University of Crete/University Hospital, Heraklion, Greece
H. Dimitriou
Paediatric Haematology
Oncology, University of Crete, Medical School, Heraklion, Greece
A.V. Kozlov
S. Dumitrescu
A. Weidinger
Ludwig Boltzmann
Institute for Experimental and Clinical Traumatology in the AUVA, Vienna, Austria
J.C. Duvigneau
University of Veterinary Medicine, Vienna, Austria
Results: SS patients with increased severity of illness had lower SF, EF, EE, albumin, glutamine, ATP, TBARS and higher levels of lactate, resistin, ΝΟ2 compared to patients with SIRS, SIRS and H (p<0.05). Changes were persisted through day 3 and were ameliorated by day 5. Longitudinal changes were associated with intracellular repression of HSP72, HSP90α and increased levels of extracellular HSP72 (p<0.05) and were related to mortality (p<0.05). Non-survivors were hypometabolic, with lower EF, SF and higher PRISM, PeLOD, resistin, Tn (p<0.05). Plasma glutamine increased after trauma (p<0.04).
Conclusion: SS is characterized from longitudinally stronger intracellular repression of ATP, HPS72, HSP90α, metabolism (EE, albumin, glutamine) and extracellular induction of inflammatory hormones (resistin) and innate immunity proteins signaling acute stress danger (HSP72) compared to SIRS.
Key words: sepsis, SIRS, bioenergy, HSP, resistin, metabolism
(severe sepsis/septic shock, SS)
(Systemic Inflammatory Response
90pg/mL
Hsp72.
adiponectin (Invitrogen, Carlsbad, CA, USA) και resistin (R&D Systems, Abingdon UK)
resistin.
CD33-PeCy5 (phycoerythrin-Cy5) και CD45-PeCy7 (phycoerythrin-Cy7).
(Fixation Buffer, Biolegend)
Hsp70/Hsp72-FITC (Fluorescein Isothiocyanate, Enzo), and Hsp90a-PE phycoerythrin (Enzo Life Sciences, NY, USA). IgG isotype controls
EPICS Coulter (CYTOMICS FC500)
σύμφωνα με την έκφραση CD45. Η MFI
(m)
(Hitachi AminoacidanalyzerL 8900)
Μέτρηση
(Energy Expenditure, EE)
E-COVX (GE Healthcare/Datex-Ohmeda 2000) (5).
Age, years, median (IQR)
Males/Females, n (%)
APACHE II, median (IQR)
APACHE II, probability of death, (%), median (IQR)
PELOD, median (IQR)
SOFA, median (IQR)
Mortality, n (%)
LOS, days, median (IQR)
Temp. Maximum, oC, mean±SE
MAP, mmHg, mean±SE
SF, %, mean±SE
ng/mL, median (IQR)
LDH, U/L, median (IQR)
CRP, mg/dL, mean±SE
ng/mL, mean±SE
adiponectin, ng/mL, mean±SE
Spearman rank correlation.
Lactate, mg/dL mean±SE
SID, mean±SE
Anion Gap, mean±SE
Glucose, mg/dL, mean±SE
Albumin, g/dL, mean±SE
EE, kCal/BMI/d, mean±SE
mHSP72, MFI, mean±SE
nHSP72, MFI, mean±SE
mHSP90α, MFI, mean±SE
nHSP90α, MFI, mean±SE
eHSP90α, ng/mL, mean±SE
Data are expressed as mean±SE (standard error of mean) or median (IQR, interquartile range) for normally distributed values and non-gaussian values, respectively and as n (%) of patients for categorical parameters. ANOVA wit h Welch correction, Games-Howell post hoc tests, p<0.05: * SIRS vs. H, ** SS vs. H, # SS vs. SIRS
PICU, Pediatric Intensive Care Unit; SIRS, systemic inflammatory response syndrome; APACHE 11, Acute Physiologic Assessment and Chronic Health Evaluation II; SOFA, Sequential Organ Failure Assessment; PELOD, Pediatric Logistic Organ Dysfunction; LOS, Length Of Stay; MAP, Mean Arterial Pressure; SF, Shortening Fraction; EE, energy expenditure; ATP, adenosine triphosphate; TBARS, thiobarbituric acid reactive substances; NOx, Total NO Nitrite; n, neutrophil; HSP, Heat-Shock Proteins; MFI, Mean Fluorescence Intensity; SID, Strong Anion Difference; LDH, Lactate Dehydrogenase; CRP, C-Reacting Protein;
θερμοκρασία σώματος (p<0.001) και διάρκεια νοσηλείας (p<0.02).
Αυξημένα επίπεδα γαλακτικού (p<0.001), resistin (p<0.05), SOFA (p<0.02), APACHEII (p<0.002) και μειωμένα SF (p<0.01) καταγράφηκαν και την 3η ημέρα
(p<0.001) και
SOFA (p<0.001), APACHE II (p<0.001) και μειωμένη
Βιβλιογραφία
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e-mail: paidpent@yahoo.gr
Correspondence
Soultana Georga
Dodekanisou 1 Agia Varvara, 12351
Greece
Τ. +302132073070+306944623737
e-mail: paidpent@yahoo.gr
Soultana Georga, Venetia Sotiri, Dionysis Karakaidos, Chrisavgi Liouli, Eleni Kanavoura, Petros Almagout, Lykourgos
Kanaris
Abstract
Introduction: Good oral-health is a pillar of prevention of many diseases oral and non oral, and its preservation is a responsibility of the paediatrician as well.
Aim: To record the oral-hygiene habits of grade 6th school children (12 years old) in Western Athens.
Materials - Methods: In cooperation with the Municipality of St. Barbara and following parental permission, a preventive dental check-up was performed in our Hospital for 145 students (out of 274, participation of 53%, where boys=50.3%, Greek children=74.5%, immigrants=6.2%, Roma=19.3%). Students answered an anonymous questionnaire regarding their oral-hygiene habits.
Results: In our results 35.8% of students had at least one decayed tooth, 3 in 10 had fillings and 9 in 10 microbial-dental plaque. The 53.8% of participants brushes their teeth daily (half of them twice/day, mostly girls) while 35.8% whenever they remember, or at the urging of their parents. All use fluoride toothpaste while the use of fluorinated solutions was mentioned in 23.4% of children and dental-flossing in 2.7%. Girls have better oral-hygiene level and habits than boys {DI-s(Μ)=0,98 vs DI-s(F)=0,9). The 55% visit the dentist on the occasion of a problem, 13.1% for preventive reasons yearly, while for 7% was their first visit. Almost all children were aware that sugar cause tooth decay. Frequent consumption of sweets and processed carbohydrates (≥3 times/week)was reported by 72.4% (22% daily), while soft drinks/packaged juices consumption (≥3times/week) was reported by 53.7% (15% daily).
Conclusions: The oral-hygiene habits and dental monitoring of students is inadequate, while a high percentage has adopted poor nutritional standards which exacerbate oral-health. Paediatricians should be able to properly inform students and their parents regarding the necessity of preventative dental check-ups and proper oral hygiene.
Soultana Georga
Dionysis Karakaidos
Chrisavgi Liouli
Eleni Kanavoura
Lykourgos Kanaris
Department of Pediatrics, General Hospital of Nikaia-Piraeus “Agios
Panteleimon” - General Hospital of Western Attica “Agia Varvara”, Athens, Greece
Venetia Sotiri
Petros Almagout
Depatment of Dentistry, General Hospital of Western Attica “Agia Varvara”, Athens, Greece
Key words: Oral hygiene, cariogenic foods, schoolchildren, Athens
2)
3)
5)
p=0,0271).
κατά το βούρτσισμα των δοντιών (Αγόρια=13 vs Kορίτσια=3, p=0,008) καθώς
p=0,027). Οι διαπιστώσεις αυτές
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Κ.
e-mail: elpis.vl@gmail.com
Correspondence
Elpis-Athina Vlachopapadopoulou
Thivon & Livadias, 11527
Athens, Greece
Τ. +302132009851
F. +302132009531
Κ. +306932247228
e-mail: elpis.vl@gmail.com
Sofia Leka-Emiris, Elpis-Athina Vlachopapadopoulou, Stefanos Michalacos
Abstract
Background: Short stature is a term applied to a child whose height is 2 standard deviations (SD) or more below the mean for children of the same sex and chronologic age (and ideally of the same racial-ethnic group). Short stature may be either a variant of normal growth or caused by a disease process.
Methods: Review of the literature during the recent years, focusing on clinical, laboratory and imaging data that aid in distinguishing normal from pathological short stature.
Results - Conclusions: The two most common causes of short stature are familial (genetic) short stature and delay of growth and puberty (CDGP) because of the idiosystasy, which are normal variants of growth. These growth patterns can often be distinguished from one another, but some children have features of both. Almost any serious systemic disease can cause growth failure. Systemic disorders or processes that may present with growth failure and/or delayed puberty include undernutrition, glucocorticoid therapy, gastrointestinal disease (especially Crohn disease and celiac disease), and renal disease. A variety of genetic syndromes and congenital malformations are associated with short stature. Turner syndrome is particularly important, as shortness and/or absence of pubertal development may be the presenting feature, with or without other characteristic clinical features. Most of these syndromes can be recognized by characteristic clinical features. These include Noonan, Russell-Silver, and Down syndromes. Endocrine diseases represent 1-5% of known etiologies of short stature. The detailed and targeted clinical and laboratory approach will lead to the correct diagnosis and the proper management.
Key words: short stature, target height, growth velocity, bone age, skeletal dysplasias, chronic disease, genetic syndromes
Sofia Leka-Emiris
Elpis-Athina Vlachopapadopoulou
Stefanos Michalacos
Dept. of Endocrinology
- Growth and Development, The Hospital for sick Children (P&A. Kyriakou) Athens-Greece
•
•
•
Συντομογραφίες:
χαμηλό
SD:
ΟΗ:
GH:
IGF:
SHOX: Short
HOmeoboX gene
Whitehouse (TW2) (16).
μετάλλαξη του SHOX γονιδίου (Short stature HOmeoboX gene).
o H
Madelung (Madelung deformity) παρατηρείται
o
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Correspondence
Maria Gogou
Dimitriou Nika 44, 60 134, Katerini, Greece e-mail: mariaangogou@ gmail.com
Maria Gogou, Andreas Giannopoulos
Abstract
Cardiovascular manifestations are encountered in a variety of neurocutaneous syndromes, even from childhood and their appearance depends on the underlying pathophysiological mechanism. A significant proportion of children with tuberous sclerosis present cardiac rhabdomyomas, which are associated with hemodynamic complications, arrhythmias and valvular dysfunction. Besides, frequent heart complications in children with neurofibromatosis type 1 are left ventricle hypertrophy, arterial hypertension, diastolic dysfunction, as well as regurgitation or stenosis of valves and complications from peripheral vessels. On the contrary, the cardiovascular involvement in other neurocutaneous syndromes (ataxia-telangiectasia, Sturge-Weber syndrome, incontinentia pigmenti, von Hippel-Lindau disease) has not been systematically studied, but according to the literature data, heart problems in these cases usually affect isolated patients.
Key words: neurocutaneous syndromes, phakomatosis, tuberous sclerosis, neurofibromatosis, heart, cardiovascular manifestations, children
Maria Gogou
Andreas Giannopoulos 2nd Department of Pediatrics, Aristotle University of Thessaloniki, Thessaloniki, Greece
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e-mail: Christodp17@ gmail.com
Correspondence
Christodoulou Panayiotis
How the Theory of Planned Behavior contributes as a method of planning health interventions
Christodoulou Panayiotis
Summary
The knowledge of the studies of human behavior in the field of psychology are now being used in the study of human behavior towards health and disease. The Theory of predesigned conduct is a cognitive theory which determines the reaction of the patient based on his convictions, influence of important people of his entourage and his reactions against it. Especially the properties of the latter to make an innovative theory to examine the response of patients to diseases need to be designed as a prevention model. In this context, the THPS is useful to investigate the attitudes of young women towards vaccination for the human papilloma virus (HPV), which is one of the most important carcinogens (cancer of the cervix of the uterus). To this end, the usefulness of a questionnaire is examined and then used for interviewing a young girl. The results of the interviews show that the most important factor that complicates the disease is the lack of knowledge on how HIV transmits. So the goal is to expand prevention for greater use of the vaccine.
Key words: HPV, THPS, cervix cancer, vaccination
(Curson and Butcher, 1992).
(DiMatteo, 2011).
(Koop, 1983).
(Conner and Sparks,
(Fern
ndez-Esquer,
(Norman and Conner, 1996).
(Armitage and Conner, 2001).
and Kenny, 1986, Terry & O’Leary, 1995).
(Langer, 1975, Lerner, 1977).
(Manstead,
(Kashima, Gallois and McCamish, 1993)
(Bentler and Speckart, 1979, Ouellette and Wood,1998, Triandis, 1977, Verplanken, 2006),
(Terry and O’Leary, 1995)
(Terry, Hogg and White, 1999).
(Armitage and Conner,
(Vaidakis,
(Tota, 2011).
(Kahn, 2009).
(70%
(Mammas et al.,
(Gerend and Shepherd, 2012), (Bennett et al., 2012).
Ερωτηματολόγιο σελίδα 3 (ΕΑΠ, 2017)
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e-mail: vtzotzola@gmail.com
Correspondence
Vassiliki-Sotiria Tzotzola
Τ. +306947070973
e-mail: vtzotzola@gmail. com
Vassiliki-Sotiria Tzotzola, Ioannis Panagiotou, Maria Ambatzidou, Sofia Polichronopoulou
Abstract
Introduction: Polycythemia is a rare disease in childhood. Relative polycythemia should firstly be excluded (i.e. dehydration). In case of absolute polycythemia, normal or low levels of erythropoietin are found in primary polycythemia, while elevated levels in the secondary one. The cornerstone of therapy is phlebotomy, though there are additional therapeutic approaches that can be applied in certain cases.
Method/Results: A 4 year old girl was diagnosed with polycythemia (red blood cells 9.530.000/ μl, Hg 17.7 gr/L, Ht 60.8%), while she was tested for a febrile infection. The elevated erythropoietin levels ruled out the primary polycythemia’s causes (i.e. polycythemia vera). Moreover, the normal value of the partial pressure of Ο2 when the Hb saturation is 50% (P50), as well as further investigation ruled out secondary polycythemia’s causes due to hypoxia (i.e. pneumonopathy, hemoglobinopathy, methylhemoglobulinemia, etc.). To conclude, a disorder of hypoxia sensing pathway was suspected as the underlying cause of this polycythemia.
Protein von Hippel-Lindau (VHL) plays a central role in this pathway, as mutations of the tumor suppressor VHL promote the overexpression of the erythropoietin’s gene (EPO). In our patient, molecular test for VHL gene mutations was performed, but it proved to be negative. The patient is satisfactorily treated with phlebotomies.
Conclusion: A detailed investigation for the causes of polycythemia is mandatory. By this approach, a more detailed therapeutic management, follow up and prognosis of the patient could be determined while it is important to note that elevated serum erythropoietin levels do not exclude congenital erythrocytosis. The latter treatment goal is to avoid thromboembolic or hemorrhagic events, maintaining a satisfactory quality of life for the patient.
Key words: polycythemia; erythrocytosis; erythropoietin
Vassiliki-Sotiria Tzotzola
Ioannis Panagiotou
Maria Ambatzidou
Sofia Polichronopoulou
Department of Hematology-Ongology “Agia Sophia” Children’s Hospital
περιελάμβανε:
Γενική αίματος: ερυθρά αιμοσφαίρια 9.530.000/μl, Hg 17.7 gr/L, Ht 60.8%, MCV 63.8 fl, RDW 26.8, ΔΕΚ 3.63%,
αιμοσφαίρια 7.580/μl, ουδετερόφιλα 29.5%, λεμφοκύτταρα 62.2%, ηωσινόφιλα 0.1%, μονοπύρηνα 7.4%, αιμοπετάλια 162.000/μl. Στο επίχρισμα
(υποχρωμία, ανισοκυττάρωση,
• Μεθαιμοσφαιρίνη 0.65% (κφ)
• 2,3-Διφωσφογλυκερινικό οξύ: κφ.
• Έλεγχος ιστικής οξυγόνωσης:
αιμοσφαιρίνης είναι 50% (P50), ήταν φυσιολογική.
• Βιοχημικοί
duced Factor-HIF).
Συντομογραφίες: ΕΠΟ:
VHL: von HippelLindau
PHD: Prolyl Hydroxylase Domain
HIF: Hypoxia induced factor
P50:
2.
2.1.
2.1.1
2.1.2
2.2.
2.2.1.
Chuvash)
Βιβλιογραφία
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e-mail:
Correspondence
Klimi Eleni
Τ. +302107010529
e-mail: klimel2015@in.gr
Klimi Eleni
Abstract
Summary: We present a case of a two year old girl with clinical symptoms typical of GianottiCrosti syndrome associated with a proven streptococcal infection, a rather rare cause of this syndrome.
Key words: Gianotti-Crosti syndrome, streptococcal infection
Klimi Eleni
Thriassio Hospital
Elefsina
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Correspondence
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Dipla
Great Ormond Street
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