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EDITORIAL
SPECIAL ARTICLE
Paediatric Neurology: Past, present, and future
S. G. Youroukos
REVIEW ARTICLES
Assessment of lung function in preschoolers
Vasiliki Avramidou, Elpis Hatziagorou, Ioannis Tsanakas
Τype 2 diabetes mellitus in children and young adults; obesity as a major pathogenetic mechanism
Angeliki A. Makri, Demetrios P. Chiotis
Childhood acute lymphoblastic leukemia: outcome with current therapeutic protocols and modern therapeutic approaches
Maria Abatzidou, Euthymia Rigatou and Sophia Polychronopoulou
Infections in immunocompromised children
Filippou Olga, Kalambalikis Panagiotis, Vliora Christianna
ORIGINAL ARTICLES
A Greek multicenter study comparing the clinical and immunologic phenotypes between adult and juvenile-onset lupus
P. Nalbanti, S. Stefanidou, S. Alfantaki, A. Siamopoulou, M. Trachana, V. Galanopoulou, P. Pratsidou-Gertsi, E. Farmaki, F. Papachristou, A. Garyphallos, F. Kanakoudi-Tsakalidou
The role of exhaled Nitric Oxide (eNO) in modification of treatment among asthmatic children
Elpis Hatziagorou, Vasiliki Avaramidou, Fotis Kirvassilis, Antigoni Mavroudi, Maria Emboriadou, John Tsanakas
CASE REPORTS
Parotid pleomorphic adenoma in childhood: report of a case and review of the literature
Evangelia Tsakiropoulou, George Kontzoglou, Aris Maganaris
Osteomyelitis: a complication of varicella
H. Papadimitriou, M. Argyrou, J. Kavaliotis
Κikuchi-Fujimoto disease in a 12-year old girl with pauciarticular juvenile idiopathic arthritis
E. Spatoula, F. Haliotis, C. Stefanaki, E. Efthimiou, N. Manolaki
Haemophagocytic syndrome: report of three cases
Georgia Avgerinou, V. Papadakis, Kleoniki Roka and Sophia Polychronopoulou
Petersen Michael
Κανακά-Gantenbein Χριστίνα
Κανάριου Μαρία Καπόγιαννης
Καραγκιόζογλου-Λαμπούδη Θωμαή
Καττάμης Αντώνης
Καφρίτσα Παναγιώτα
Κίτσιου-Τζέλη Σοφία
Κοντόπουλος Ελευθέριος
Κόσσυβα Λυδία
Κουρή Αγαθή
Κώσταλος Χρήστος
Μαλαμίτση-Πούχνερ Αριάδνη
Μανουσάκης Εμμανουήλ
Μανταδάκης Ελπιδοφόρος
Μπιτσώρη Μαρία
Μυριοκεφαλιτάκης Νικόλαος
Ντουντουνάκης Σταύρος
Ξεπαπαδάκη Παρασκευή
Παναγιωτοπούλου-Γαρταγάνη Πολυτίμη
Παπαβασιλείου-Συρίγου Αντιγόνη
Παπαγαρουφάλης Κωνσταντίνος
Abstract
S. G. Youroukos
First Department of Paediatrics, University of Athens
“Aghia Sophia” Children’s Hospital
S. G. Youroukos
During the last 50 years paediatric neurology was internationally recognized and established. After 1950 it became clear that paediatricians as well as adult neurologists could not manage efficiently neurological disorders in children. At that time in some major paediatric hospitals of developed countries, the first efforts to organize paediatric neurology units were started. In Greece the first effort to organize such a unit took place in 1951 in the Athens University Department of Paediatrics in “St. Sophia” Children’s Hospital. Since 1968, international societies for paediatric neurology have been formed, while the Hellenic Paediatric Neurology Association was formed in 1992. In the fifties a 3-year-training program was organized in U.S.A. This program was later extended to 5 years. In Europe, the training program proposed by the European Paediatric Neurology Society, is 6.5 years long. In Greece, the program proposed to the Ministry of Health by the committee appointed in 2009, has a duration of 7 years (4 years paediatrics, 6 months neurology, 2.5 years paediatric neurology). Since 2002 paediatric neurology has been recognized in Europe as “subspecialty of paediatrics with strong relation to neurology”. In Greece we have more than 30 paediatric neurologists and 1-2 new ones are expected every year. It is hoped that the ability to provide full training of high level in Greece, will lead to better paediatric neurology services in the country.
Key words: paediatric neurology, training, subspecialty
Αλληλογραφία
Σωτήρης Γ. Γιουρούκος
Θηβών και Λεβαδείας, 11527, Αθήνα
Τηλ.: 2108061714, 6972721996
www.pediatrics-uoa.edu.gr
e-mail: sotel@hol.gr
Correspodence
S. G. Youroukos
Thivon & Livadias, 11527, Athens, Greece
Τel.:+302108061714, +306972721996 www.pediatrics-uoa.edu.gr e-mail: sotel@hol.gr
Κατάλογος Συντομογραφιών
ΚΔ = κετογονική δίαιτα
2DG = 2-δεοξυ-D-γλυκόζη
TCA κύκλος = κύκλος του
κιτρικού οξέος
GNU = μονάδα νευρώνα–νευρογλοίας
GABA = γ-αμινοβουτυρικό οξύ
DON = 6-διαζο-5-οξο-Lνορλευκίνη
AEDs = αντιεπιληπτικά φάρμακα MCT1 = μονοκαρβοξυλικοί
υποδοχείς 1
ATP = τριφωσφορική αδενοσίνη
Lennox (Boston Children’s Hospital), P. Dodge (Mass General Hospital, Boston), S. Carter (New York) και D. Clark (John Hopkins, Baltimore).
S. Prichard (Toronto),
ο Y. Fukuyama (2).
W. Wyllie
R. McKeith
V. Hutinel (έκδοση
(Maladies des enfants: Tome V. Maladies du Systeme Nerveux) (4).
Diseases of the Nervous System in Infancy, Childhood and Adolescence
F. Ford,
(5).
Executive, Training, Educational, Scientific, Expended Europe, Journal, Committee of
tional Advisers (CNA).
Aicardi, P. Barth, P. Casear, V. Dubowitz, P. Evrard, N. Gordon, B. Hagberg, F. Hanefeld, G. Lyon, B. Neville, και F. Schulte.
του Confederation of European Specialties in Paediatrics (CESP). Όμως το 2009-2010
pean Board of Paediatrics).
(3
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(Interrupter Resistance, Rint),
(Multiple-Breath Washout, MBW),
(Whole Body Plethysmography, WBP)
(Forced oscillation Technique, FOT).
Rint
Abstract
Vasiliki Avramidou
Elpis Hatziagorou
Ioannis Tsanakas
Department of Paediatric Pneumonology, 3rd Department of Paediatrics, Hippokrateion
General Hospital of Thessaloniki, Aristotle University of Thessaloniki
Vasiliki Avramidou, Elpis Hatziagorou, Ioannis Tsanakas
Measuring lung function in preschoolers and infants presents a number of special challenges. The most commonly used pulmonary function tests (PFTs) in this age group include Interrupter Resistance (Rint), Multiple-Breath Washout (MBW), Whole Body Plethysmography (WBP) and Forced Oscillation Technique (FOT), which require only quiet tidal breathing. In addition, adjusted spirometric indices can be used in preschoolers. Spirometry measures flows pulmonary volumes and capacities in cooperative patients. Rint is a representation of airway resistance; its measurements are higher in young asthmatic children, children with history of wheeze and children with cystic fibrosis compared with healthy children. Rint measurements can also evaluate the airways’ response to inhaler medication. The MBW of an inert gas method is used to measure the FRC and the efficiency of ventilation distribution in the lungs. The Lung Clearance Index (LCI) is one of the indices of ventilation distribution and a sensitive index in airway disorders that involve
49 546 42, Θεσσαλονίκη e-mail: tsanakas@hol.gr
Correspodence
Tsanakas Ioannis Constantinoupoleos 49 546 42, Thessaloniki, Greece. e-mail: tsanakas@hol.gr
the peripheral airways. The WBP -either by variable pressure or volume displacementcan measure both thoracic gas volume (FRCpleth) and airways’ resistance (sRaw), which are sensitive indices of obstructive airway diseases, primarily involving more peripheral airways, as well as estimate the response to inhaler medication. Lastly, in FOT an external signal is applied to the respiratory system and its response reflects the total resistanse of the respiratory system (Rrs). FOT can identify bronchial obstruction and response to bronchodilators and bronchoconstrictors.
Key words: lung function, preschoolers, Spirometry, Rint, Multiple-Breath Washout, Whole Body Plethysmography, Forced Oscillation Technique
Breath Washout,
mography, WBP)(5-8)
(Forced Oscillation Technique, FOT).(8)
2.
3.
FRC=CEV / CetSTART - CetEND
CEV: Cumulative Expired Volume tracer gas
Cet: end- tidal concentration
CetEND approx. 1/40 of CetSTART
/ FRC
4.
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Αγγελική
Human Genetics Department,
McGill University Health CenterMontreal Children’s Hospital Research Institute, Montreal, Canada
Τype 2 diabetes mellitus in children and young adults; obesity as a major pathogenetic mechanism
Angeliki A. Makri1, Demetrios P. Chiotis2
Abstract
Angeliki A. Makri1
Human Genetics Department, McGill University Health CenterMontreal Children’s Hospital Research Institute, Montreal, Canada
Demetrios P. Chiotis2
Endocrinology Department - Pediatric Euroclinic
The global epidemic of childhood obesity is closely associated with the increasing incidence of type 2 diabetes in children and young adolescents. Despite the extended research on type 2 diabetes in adult patients, clinical and experimental data in childhood are still limited. Obesity appears to be the major pathogenetic mechanism. Risk factors also include ethnicity, family history, insulin resistance and finally smoking. This modern trend seems to be evolving into a major public health issue, given the significant co-morbidities that almost always accompany the diagnosis of type 2 diabetes mellitus. The extent of the problem will only be appreciated when the obese adolescents develop -during their adulthood- the full spectrum of diabetes clinical consequences.
Key words: Adolescents, childhood obesity, type 2 diabetes mellitus, epidemiology, pathophysiology, risk factors.
McGill University Health Center-Montreal Children’s Hospital Research Institute, 4060 St-Catherine H3Z2Z3, Montreal, Quebec, Canada.
e-mail: angeliki.makri@mail.mcgill.ca
Correspodence
Angeliki Makri
McGill University Health Center-Montreal Children’s Hospital Research Institute, 4060 St-Catherine H3Z2Z3, Montreal, Quebec, Canada.
e-mail: angeliki.makri@mail.mcgill.ca
BMI = Body Mass Index
ADA = American Diabetes Association
AHA = American Heart Association
CDC = Center for Disease Control and Prevention
FPG =
IFG = Ιmpaired Fasting glucose
IGT = Impaired Glucose Tolerance
OGTT = Oral Glucose Tolerance Test
Europe-
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Maria Abatzidou
Euthymia Rigatou
Sophia Polychronopoulou
Paediatric Department of Hematology - Oncology, “Aghia
Sophia” Children’s Hospital.
Maria Abatzidou, Euthymia Rigatou and Sophia Polychronopoulou
Currently applied therapeutic protocols in childhood acute lymphoblastic leukemia (ALL) have led to the attribution of remarkable survival rates that exceed 85% in cases of standard and medium risk and 55-60% in cases of high risk patients. This success has been
34, Αμπελόκηποι
Τ.Κ. 115 27,
Τηλ: 6973631032
e-mail: mirellaaba@yahoo.gr
Correspodence
Maria Abatzidou
34 Sinopis St., Ampelokipi 115 27, Athens, Greece
Tel: +306973631032
e-mail: mirellaaba@yahoo.gr
mainly accomplished through precise risk stratification and subsequent individualized treatment planning. In a recent study of our Department, we analyzed the epidemiological, morphological, flow cytometric, cytogenetic and molecular characteristics as well as the final outcome of patients with ALL in our Department since 1999. 136 pediatric patients have been analyzed that all received chemotherapy homogeneously according to ALL-BFM 95-2000 Protocol by two therapy risk groups (Medium Risk Group-MR and High Risk Group-HR) and a median follow up time of 5 years. In groups MR and HR, EFS percentages were 95,3% and 66,7% respectively while in the whole cohort OS and EFS percentages reached 89,6% and 92,5% respectively. However, despite the amazing progress in treatment strategies and prognosis of pediatric patients with ALL, the long term survival rates in relapsed and refractory disease have been consistently low during the past two decades. Early response to treatment remains one of the fundamental prognostic factors that highlights the need for advanced therapeutic strategies, especially in cases of patients that are in high risk of relapse according to contemporary treatment protocols. Novel treatment strategies include new formulations of currently applied chemotherapeutic agents, new antimetabolites and nucleoside analogs, monoclonal antibodies and cellular immunotherapy, as well as new molecular inhibitors. Some of these agents have already been incorporated in contemporary treatment protocols while others remain in early stages of development and evaluation. Future research is oriented towards a more sophisticated understanding of mechanisms that are implicated in leukemogenesis that will allow formulation of treatment agents of great specification and low chemotherapy tolerance. The main goal always remains the improvement of long term outcome and survival of pediatric ALL patients as well as substantial treatment toxicity decrease through careful modification and gradiation of currently applied and novel therapy agents.
Key words: Childhood acute lymphoblastic leukemia, treatment protocols, nucleoside analogs, monoclonal antibodies, molecular inhibitors.
ALL = Acute Lymphoblastic Leukemia
MRD = Minimal Residual Disease
OS = Overall Survival
EFS = Event-free survival
(prednisone, VCR, PEG Asparaginase, doxorubicin).
asparaginase
2.
Wolinella asparaginase.
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Abstract
cystis carinii, CMV
Filippou Olga1, Kalambalikis Panagiotis2, Vliora Christianna1
Filippou Olga1
Vliora Christianna1
Department of Paediatric, General Hospital “Asklipeio
Voulas”
Kalambalikis Panagiotis2
Paediatric Intensive Care
Unit, “Aghia Sophia” Children’s Hospital
The number of immunosuppressed / immunocompromised children, in recent times is constantly rising, mainly due to advances in transplantations, the HIV syndrome and the extended survival of patients with neoplastic diseases. In these patients, infections are the main cause of morbidity and mortality. Symptoms and signs of infection can be very subtle and diagnostics made difficult, as the inflammatory response is moderated by immunosuppression. In this review, the main characteristics of infections caused by fungi and viruses, the modern methods of laboratory confirmation and the recent guidelines for their treatment are summarized. Also diseases caused by Candida, Aspergillus and Pneumocystis carinii, as well as CMV, which has primary importance in transplant recipients, are described in detail. Furthermore, emphasis is given in the defining role of administering prophylaxis to prevent infections, in special patient categories and in the recommendations for the prompt initiation of empirical treatment.
Key words: immunosuppressed children, transplantations, fungal infections, pneumocystis carinii, CMV infection.
Β.
Τ.Κ. 16673
Τηλ.: 2108923751
e-mail: filiolga@hotmail.com
Correspodence
Filippou Olga
1 V. Pavlou St. 166 73, Voula, Attiki, Greece
Tel.: 2108923751
e-mail: filiolga@hotmail.com
Candida Αλληλογραφία
Candida (C. parapsilosis, C. tropicalis, C. krusei, C. glabrata)
Amphotericin B δεοξυχολική (Fungizone)
Amphotericin B λιποσωμιακή (Ambisome)
Amphotericin B
(Fungustatin)
(V-Fend)1
(Noxafil)2
Κασποφουγγίνη (Cancidas)
(Abelcet)
0,6-1,5 mg/kgr/24ωρο, σε 1 δόση IV
3-5 mg/kgr/24ωρο, σε 1 δόση IV
3-5 mg/kgr/24ωρο, σε 1 δόση IV
6-12 mg/kgr/24ωρο, σε 2 δόσεις IV
14 mg/kgr/24ωρο, σε 2 δόσεις IV
400-800 mg/24ωρο POS
50 mg/m²/24ωρο, σε 1 δόση IV.
και κ/α BAL, ΕΝΥ, ή/και υλικού
3) Ιστολογική εξέταση (βιοψία), 4) Ανίχνευση και προσδιορισμός των επιπέδων γαλακτομανάνης (Αg του κυτταρικού τοιχώματος του ασπέργιλλου) στον ορό, BAL, ENY. Η ευαισθησία και ειδικότητα αγγίζουν το 80%, αλλά ποικίλλουν ευρέως, λόγω μη σαφούς καθορισμού
300 mg ανά μήνα με νεφελοποιητή Respigard II® (≥5 ετών).
30 mg/kg/24h PO σε 1 δόση (1-3 μηνών) 45 mg/kg/24h PO σε 1 δόση (4-24 μηνών)
30 mg/kg/24h PO σε 1 δόση (>24 μηνών)
2 mg/kg/24h PO σε 1 δόση (max 100 mg) 4 mg/kg PO
(max 200 mg)
CMV
(62, 63, 64, 65).
1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12.
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Abstract
P. Nalbanti1
M. Trachana1
P. Pratsidou-Gertsi1
E. Farmaki1
F. Papachristou1
F. Kanakoudi-Tsakalidou1
11st Dept of Pediatrics, Aristotle University, Pediatric Immunology and Rheumatology Referral Center, Thessaloniki
S. Stefanidou2
A. Garyphallos2 24th Dept of Internal Medicine, Aristotle University, Thessaloniki
S. Alfantaki3
A. Siamopoulou3
3Dept of Child Health, University of Ioannina, Ioannina
V. Galanopoulou4
4Rheumatology Unit, N. Papageorgiou Hospital, Thessaloniki, Greece
P. Nalbanti1, S. Stefanidou2, S. Alfantaki3, A. Siamopoulou3, M. Trachana1, V. Galanopoulou4, P. Pratsidou-Gertsi1, E. Farmaki1, F. Papachristou1, A. Garyphallos2, F. Kanakoudi-Tsakalidou1
Background: Differences in the clinical and immunologic phenotype between adult and juvenile-onset SLE (aSLE, jSLE) affect the decision about the “induction therapy” which has to be used in order to shorten the time of disease activity and minimize the risk of cumulative damage overtime. The objective of this study is to compare the clinical and immunologic phenotypes between aSLE and jSLE, in Greek patients at disease onset and 5 yrs thereafter due to paucity of relevant data from Mediterranean countries. Methods: This retrospective study enrolled 66 jSLE and 97 aSLE Greek Caucasian patients, all having been attended in 4 Rheumatology Centers. Demographic data, as well as clinical and immunologic findings at diagnosis and 5 yrs thereafter, were studied.
Results: At the disease onset, the mean (SD) ages were 12,25±0,27 and 33,93±1,32 yrs and the mean follow-up time 6,63±0,59 and 11,6±0,7 yrs, for jSLE and aSLE respectively. Regarding the clinical phenotype, general features, lymphoid hyperplasia (hepatosplenomegaly and/or lymphadenopathy) and blood disorders were more frequent in jSLE patients (p=0,001, p=0,015 and p<0,001 respectively), whereas photosensitivity was commoner in those with aSLE (p=0,033). In contrast with most of the relevant studies in the literature, renal involvement in this study was not found to be significantly commoner in jSLE patients compared to those with aSLE. Nevertheless, frequency of organ/system involvement in jSLE was significantly higher than in aSLE (p=0,009). At the end of 5 yrs, the cumulative number of clinical manifestations and organ involvement was similar in both groups. General symptoms and blood disorders remained significantly commoner in patients with jSLE. Frequency of anti-dsDNA, anti-cardiolipin, anti-Sm, anti-URNP antibodies and low C3 and C4 levels were cumulatively found to be significantly higher in jSLE patients (p<0,01).
Conclusions: The results of our study show that clinical and immunologic phenotypes of jSLE are more severe, disease activity index is high and frequency of organ/system involvement is higher at disease onset, compared with aSLE. These findings suggest that the induction therapy in patients with jSLE has to be more aggressive and probably more specific (i.e. anti B-cell biologics) than in those with aSLE.
Key words: Adult-onset lupus; juvenile-onset lupus; clinical phenotype; immunologic phenotype
2310839291
e-mail: flkan@auth.gr
Correspodence
F. Kanakoudi-Tsakalidou
Emeritus Professor of Paediatrics
2 M. Mpotsari St. 546 43 Thessaloniki, Greece
Tel.: +302310839291
e-mail: flkan@auth.gr
WHO). 10)
γαστρεντερικό (αιμορραγίες,
αγγειίτιδας). 15) Εκδηλώσεις από
Hashimoto. 17)
0,001
Στοματικά
Μυοσκελετικές
0,001 0,007 0,308 0,19 0,377 0,24 0,717 0,757 0,212 0,986 0,056 <0,001 0,271 1 0,475 1 0,202 0,252
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22.Cooper GS, Parks CG, Treadwell EL, St Clair EW, Gilkeson GS, Cohen PL, et al. Differences by race, sex and age in the clinical and immunologic features of recently diagnosed systemic lupus erythematosus patients in the southeastern United States. Lupus 2002; 11: 161-167.
23.Jurencak R, Fritzler M, Tyrrell P, Hiraki L, Benseler S, Silverman E. Autoantibodies in pediatric systemic lupus erythematosus: ethnic grouping cluster analysis and clinical correlations. J Rheumatol 2009; 36: 416-21.
Elpis Hatziagorou1
Vasiliki Avaramidou1
Fotis Kirvassilis1
Antigoni Mavroudi1
John Tsanakas1
3rd Paediatric Department, Aristotle University of Thessaloniki, Hippokration Hospital of Thessaloniki
Maria Emboriadou2
2nd Paediatric Department, Aristotle University of Thessaloniki, AHEPA Hospital
Elpis Hatziagorou1, Vasiliki Avaramidou1, Fotis Kirvassilis1, Antigoni Mavroudi1 , Maria Emboriadou2, John Tsanakas1
Background: Exhaled nitric oxide (eNO) is a sensitive marker of airway inflammation. The aim of the study was to assess the anti-inflammatory effect of inhaled steroids and to compare the combination of low doses of inhaled steroids with LABA (long acting beta agonists) vs. high doses of inhaled steroids among children with persistent asthma. Methods: We studied 52 children (mean age ± SD: 13.3 ± 3.3 years) with moderate asthma, who had not received prophylactic anti-inflammatory treatment before, and were
Αλληλογραφία
Ελπίδα Χατζηαγόρου,
Πλουτάρχου 6 55236,
Θεσσαλονίκη
Τηλ.: 2310892400
Κινητό: 6972851721
e-mail: elpcon@otenet.gr
Correspodence
Elpis Hatziagorou
Ploutarchou 6, 55236, Thessaloniki, Greece
Τel.: +302310892400
Mob: +306972851721
e-mail: elpcon@otenet.gr
on inhaled steroids for two months. Furthermore, we studied 36 children (mean age ± SD: 14.6 ± 3.1 years) with persistent asthma that received either high doses of inhaled steroids or a combination of low doses of inhaled steroids with LABA. Children were evaluated by measuring eNO and FEV1 (spirometry).
Results: Median eNO values were significantly decreased by inhaled steroids among children with moderate asthma (p<0.05). Median eNO values were reduced significantly, by combination of low doses of inhaled steroids with LABA (p=0.00001) and by high dose of inhaled steroids (p=0.00001).
Conclusion: Measurement of eNO is a useful tool to regulate the anti-asthmatic treatment. Inhaled steroids were found to reduce significantly the eNO values. The combination of low doses of inhaled steroids with LABA were found to reduce eNO values significantly; they can be used instead of high dose of inhaled steroids.
Key words: Εxhaled nitric oxide, inhaler steroids, long acting beta agonists, asthma, children
(NOS) [2], [3], [4].
2008 #1509}.
(Vitalograph 2120, Vitalograph Ltd, Ennis, Ireland).
Expiratory Flow 25-75: FEF25-75).
SPSS v.19.0
Chicago, Illinois,
Αποτελέσματα
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8. Gustafsson, L.E., et al., Endogenous nitric oxide is present in the exhaled air of rabbits, guinea pigs and humans. Biochem Biophys Res Commun, 1991. 181(2): p. 852-7.
9. Baraldi, E. and J.C. de Jongste, Measurement of exhaled nitric oxide in children, 2001. Eur Respir J, 2002. 20(1): p. 223-37.
10. Standardization of Spirometry, 1994 Update. American Thoracic Society. Am J Respir Crit Care Med, 1995. 152(3): p. 1107-36.
11. Quanjer, P.H., et al., Lung volumes and forced ventilatory flows. Report Working Party Standardization of Lung Function Tests, European Community for Steel and Coal. Official Statement of the European Respiratory Society. Eur Respir J Suppl, 1993. 16: p. 5-40.
12. Quanjer, P.H., et al., Spirometric reference values for white European children and adolescents: Polgar revisited. Pediatr Pulmonol, 1995. 19(2): p. 135-42.
13. Barnes, P.J., Efficacy of inhaled corticosteroids in asthma. J Allergy Clin Immunol, 1998. 102(4 Pt 1): p. 531-8.
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15. van Rensen, E.L., et al., Effect of inhaled steroids on airway hyperresponsiveness, sputum eosinophils, and exhaled nitric oxide levels in patients with asthma. Thorax, 1999. 54(5): p. 403-8.
16. van Grunsven, P.M., et al., Effect of inhaled corticosteroids on bronchial responsiveness in patients with “corticosteroid naive” mild asthma: a meta-analysis. Thorax, 1999. 54(4): p. 316-22.
17. Sont, J.K., et al., Relationship between the inflammatory infiltrate in bronchial biopsy specimens and clinical severity of asthma in patients treated with inhaled steroids. Thorax, 1996. 51(5): p. 496-502.
18. Haley, K.J. and J.M. Drazen, Inflammation and airway function in asthma: what you see is not necessarily what you get. Am J Respir Crit Care Med, 1998. 157(1): p. 1-3.
19. Foresi, A., et al., Inflammatory markers in bronchoalveolar lavage and in bronchial biopsy in asthma during remission. Chest, 1990. 98(3): p. 528-35.
20. Lipworth, B.J., Leukotriene-receptor antagonists. Lancet, 1999. 353(9146): p. 57-62.
21. Toogood, J.H., et al., A graded dose assessment of the efficacy of beclomethasone dipropionate aerosol for severe chronic asthma. J Allergy Clin Immunol, 1977. 59(4): p. 298-308.
22. Kharitonov, S.A., D.H. Yates, and P.J. Barnes, Inhaled glucocorticoids decrease nitric oxide in exhaled air of asthmatic patients. Am J Respir Crit Care Med, 1996. 153(1): p. 454-7.
23. Kraan, J., et al., Dosage and time effects of inhaled budesonide on bronchial hyperreactivity. Am Rev Respir Dis, 1988. 137(1): p. 44-8.
24. Carra, S., et al., Budesonide but not nedocromil sodium reduces exhaled nitric oxide levels in asthmatic children. Respir Med, 2001. 95(9): p. 734-9.
25. Jones, S.L., et al., Exhaled NO and assessment of anti-inflammatory effects of inhaled steroid: dose-response relationship. Eur Respir J, 2002. 20(3): p. 601-8.
26. Di Rosa, M., et al., Glucocorticoids inhibit the induction of nitric oxide synthase in macrophages. Biochem Biophys Res Commun, 1990. 172(3): p. 1246-52.
27. Smith, A.D., et al., Exhaled nitric oxide: a predictor of steroid response. Am J Respir Crit Care Med, 2005. 172(4): p. 453-9.
28. Fahy, J.V. and H.A. Boushey, Effect of low-dose beclomethasone dipropionate on asthma control and airway inflammation. Eur Respir J, 1998. 11(6): p. 1240-7.
29. Fuglsang, G., et al., Effect of salmeterol treatment on nitric oxide level in exhaled air and dose-response to terbutaline in children with mild asthma. Pediatr Pulmonol, 1998. 25(5): p. 314-21.
30. Currie, G.P., et al., Effects of fluticasone plus salmeterol versus twice the dose of fluticasone in asthmatic patients. Eur J Clin Pharmacol, 2003. 59(1): p. 11-5.
31. Aziz, I., A.M. Wilson, and B.J. Lipworth, Effects of once-daily formoterol and budesonide given alone or in combination on surrogate inflammatory markers in asthmatic adults. Chest, 2000. 118(4): p. 1049-58.
32. Pauwels, R.A., et al., Effect of inhaled formoterol and budesonide on exacerbations of asthma. Formoterol and Corticosteroids Establishing Therapy (FACET) International Study Group. N Engl J Med, 1997. 337(20): p. 1405-11.
33. Ducharme, F.M., et al., Addition of long-acting beta2-agonists to inhaled steroids versus higher dose inhaled steroids in adults and children with persistent asthma. Cochrane Database Syst Rev, 2010(4): p. CD005533.
34. McIvor, R.A., et al., Potential masking effects of salmeterol on airway inflammation in asthma. Am J Respir Crit Care Med, 1998. 158(3): p. 924-30.
35. Lipworth, B.J., et al., Effects of adding a leukotriene antagonist or a long-acting beta(2)-agonist in asthmatic patients with the glycine-16 beta(2)-adrenoceptor genotype. Am J Med, 2000. 109(2): p. 114-21.
Evangelia Tsakiropoulou
George Kontzoglou
Aris Maganaris
Department of Otorhinolaryngology, “Hippokratio”General Hospital of Thessaloniki, Greece.
Parotid pleomorphic adenoma in childhood: report of a case and review of the literature
Evangelia Tsakiropoulou, George Kontzoglou, Aris Maganaris
Background: Salivary gland tumors in childhood are rare. The most common tumors of the parotid gland in children are in order haemangioma, lemphangioma and pleomorphic adenoma.
Case presentation: We present the case of an 11-year old boy with 7-month history of a solitary, painless swelling of the left parotid area. Investigation studies included ultrasound and magnetic resonance imaging of the left parotid area. The fine needle aspiration cytology under ultrasound guidance was suggestive of pleomorphic adenoma. Superficial parotidectomy was performed and the histology of the surgical specimen confirmed the diagnosis of the preoperative cytology. The facial nerve was identified and preserved. No complications were noticed during the early postoperative period and 1 year later no signs of tumor recurrence exist.
Conclusion: Due to the low incidence of pleomorphic adenoma in childhood, diagnosis and treatment remain a challenge for both pediatricians and otorhinolaryngologists.
Key words: Pleomorphic adenoma, parotid, parotid tumors, parotidectomy, salivary glands
τηλ: 2310321677
ltsak@hotmail.com
Correspodence
Tsakiropoulou Evangelia
3 Kapetan Kotta St.
Thessaloniki
tel: 2310321677
ltsak@hotmail.com
1. Bentz BG, Hughes CA, Ludemann JP, et al. Masses of the Salivary Gland Region in Children. Arch Otolaryngol Head Neck Surg 2000; 126:1435-1439.
2. Krolls SO, Trodahl JN, Boyers RC. Salivary gland lesions in children: survey of 430 cases. Cancer 1972; 30:459-469.
3. Orvidas LJ, Kasperbauer JL, Lewis JE, et al. Pediatric Parotid Masses. Arch Otolaryngol Head Neck Surg 2000; 126:177-184.
4. Garcia CJ, Flores PA, Arce JD, et al. Ultrasonography in the study of salivary gland lesions in children. Pediatr Radiol 1998; 28:418–425.
5. Chong GC, Beahrs OH, Chen ML, et al. Management of parotid gland tumors in infants and children. Mayo Clin Proc 1975; 50:279-283.
6. Nagler RM, Laufer D. Tumors of the major and minor salivary glands: review of 25 years of experience. Anticancer Res 1997; 17:701-707.
7. Ellies M, Schaffranietz F, Arglebe C, et al. Tumors of the Salivary Glands in Childhood and Adolescence. J Oral Maxillofac Surg 2006 ; 64:1049-1058.
8. Dalati T, Hussein MR. Juvenile pleomorphic adenoma of the cheek: a case report and review of literature. Diagnostic Pathology 2009; 4:32.
9. Faktorovich EG, Crawford JB, Char DH, et al. Benign mixed tumor (pleomorphic adenoma) of the lacrimal gland in a 6-year-old boy. Am J Ophthalmol 1996; 122: 446-447.
10. Rodriguez KH, Vargas S, Robson C, et al. Pleomorphic adenoma of the parotid gland in children. Int J Pediatr Otorhinolaryngol 2007;71: 1717-1723.
11. Ikeda K, Katoh T, Ha-Kawa SK, et al. The usefulness of MR in establishing the diagnosis of parotid pleomorphic adenoma. Am J Neuroradiol 1996; 17:555-559.
12. Cajulis RS, Gokaslan ST, Yu GH, et al. Fine needle aspiration biopsy of the salivary glands: a five-year experience with emphasis on diagnostic pitfalls. Acta Cytol 1997; 41:1412-1420.
13. Rehberg E, Schroeder HG, Kleinsasser O. Chirurgie bei gutartigen Parotistumoren individuell angepasste oderstandardisierte radikale Eingriffe? Laryngorhinootologie 1998;77:283–288.
14. Bullerdiek J, Wobst G, Meyer-Bolte K, et al. Cytogenetic subtyping of 220 salivary gland pleomorphic adenomas: correlation to occurrence, histological subtype, and in vitro cellular behavior. Cancer Genet Cytogenet 1993; 65: 27-31.
15. Stennert E, Wittekindt C, Klussmann JP, et al. Recurrent pleomorphic adenoma of the parotid gland: a prospective histopathological and immunohistochemical study. Laryngoscope 2004; 114: 158-163.
16. Carew JF, Spiro RH, Singh B, et al. Treatment of recurrent pleomorphic adenomas of the parotid gland. Otolaryngol. Head Neck Surg 1999; 121: 539-542.
Συγγενείς
H. Papadimitriou
M. Argyrou
J. Kavaliotis
Pediatric Department, Infectious Diseases Hospital, Thessaloniki
Varicella is a common viral infection which is highly contagious with high morbidity, at least before the introduction of the vaccine. Serious complications are rare in previously healthy people. The case presented is about a child with osteomyelitis of lower limp after varicella. One month after treatment the patient was in very good clinical condition. The aim of the presentation is to remind this rare but serious, if not treated in time, varicella complication.
Key words: Varicella, Complications, Arthritis, Osteomyelitis.
2313308719
e-mail: kavagr@hotmail.com
Correspodence
J. Kavaliotis
Pediatric Department, Infectious Diseases Hospital
13 Gr. Lampraki St. 546 38 Thessaloniki, Greece
Tel. 2313-308719
e-mail: kavagr@hotmail.com
Cierny-Mader
Cierny-Mader
1. Ziebold C, Von Kries R, Lang R, Weigl J, Schmitt H. Severe Complications of varicella in previously healthy children in Germany. A 1-year Survey. Pediatrics 2001, 108:79.
2. Kavaliotis J, Sakellaropoulou A, Siskow A, Mataki Th. Varicella in children. Analysis of 1920 patients. 22th ESPID, Tampere-Finland 26-28/05/2004.
3. Grier D, Feinstein K. Osteomyelitis in hospitalized children with chickenpox. Imaging findings in four cases. AJR 1993, 161: 643-646.
4. Krogstad P. Osteomyelitis and Septic Arthritis. In Feigin, Cherry, Demmler, Kaplan. Textbook of pediatric infectious diseases, 5th edition, Saunders:London, 2004:711-726.
5. Madder/. C, Pennick J. A clinical staging system for adult osteomyelitis. Contemp Orthop 1985, 10:17-37.
6. Symeonides P. Bone and joint infectious diseases. In P. Symeonides, ed. Orthopedics, 1st ed. Thessaloniki 1986:207-217.
7. Lazarini L, Mader J, Calhoun J. Osteomyelitis in long bones. J Bone Joint Surg Am 2004, 86: 2305-2318.
8. Al-Fifi, McDonald J. Group A streptococcus osteomyelitis and septic arthritis following variccella: case report and review of the literature. Ann Saudi Med 1998, Vol 18, No 5,1.
9. Koturoglu G, Kurugol Z, Cetin N, Hizarcioglou, Vardar F, Helvaci M, et al. Complications of varicella in healthy children in Izmir, Turkey. Pediatr Intern 2005, 47:296-299.
E. Spatoula1
F. Haliotis1
E. Efthimiou1
N. Manolaki1
C. Stefanaki2
2nd Department of Pediatrics1 and Department of Pathology2, “Aghia Sophia” Children΄s Hospital, Athens, Greece.
Κikuchi-Fujimoto disease in a 12-year old girl with pauciarticular juvenile idiopathic arthritis
E. Spatoula1, F. Haliotis1, C. Stefanaki2, E. Efthimiou1, N. Manolaki1
Kikuchi-Fujimoto disease is a benign, self limited disease, rare in children, of unknown aetiology. Clinically it manifests as cervical lymphadenopathy with or without fever. Diagnosis is established by characteristic histological findings on affected lymph nodes biopsy. It has been associated with infectious agents and autoimmune diseases especially systemic lupus erythematosus. We describe a case of Κikuchi–Fujimoto disease in a 12-year old girl with pauciarticular persistent juvenile idiopathic arthritis, who presented with arthritis of the right knee 18 months ago. Non steroidal anti-inflammatory medication resulted in full remission for the last 12 months. Lymphadenopathy was accompanied with fever, joint pain, malaise and weight loss. Excision biopsy of cervical lymph nodes confirmed Kikuchi- Fujimoto disease. There was complete recovery and over a 14-month
τηλ: 6945423996
e-mail: elspat10@yahoo.gr
Correspodence
Eleni Spatoula
2nd Department of Pediatrics“Aghia Sophia” Children΄s Hospital, Athens, Greece.
5 Dervenakion St., N. Chalkidona Athens, 143 43 tel: +306945423996
e-mail: elspat10@yahoo.gr
KFD = Νόσος Kikuchi-Fujimoto
JIA = juvenile idiopathic arthritis
SLE = systemic lupus erythematosus
follow up period no recurrence was noted in contrast to three other reported cases of Kikuchi-Fujimoto disease in children with systemic juvenile idiopathic arthritis which were complicated with hemophagocytic lymphohistiocytosis. To our knowledge this is the first case of Kikuchi-Fujimoto disease in a child with pauciarticular persistent juvenile idiopathic arthritis. Kikuchi-Fujimoto disease should be included in the differential diagnosis of cervical lymphadenopathy with or without fever and the patient should be closely followed up either for complications if he has an autoimmune disease or for manifestations of an autoimmune disease later on.
Key words: histiocytic necrotizing lymphadenitis, Kikuchi-Fujimoto disease, juvenile idiopathic arthritis, cervical lymphadenopathy, hemophagocytic lymphohistiocytosis
Kikuchi-Fujimoto (Kikuchi-Fujimoto Disease, KFD)
(1)
Fujomoto (2).
CMV, EBV, HSV, VZV,
Yersinia enterocolitica
Sjogren.(8)
Parvo B19,
Pasteurella multocida (7).
1. Kikuchi M. Lymphadenitis showing focal reticulum cell hyperplasia with nuclear debris and phagocytes. Acta Hematol Jpn 1972; 35: 379-380.
2. Fujimoto Y, Kozima Y, Yamaguchi K. Cervical subacute necrotising lymphadenitis: a new clinicopathologic entity. Naika 1972; 20: 920-927.
3. Ramanan AV, Wynn RF, Kelsey A, Baildam E M. Systemic juvenile idiopathic arthritis. Kikuchi’s disease and haemophagocytic lymphohistiocytosis-is there a link? Case report and literature review. [Letter to the editor] Rheumatology 2003; 42(4): 596-598.
4. Οliveira S, Destri UBW, Vasquez LCO, Ferman S, Romano S, Sztajnbok FR. Systemic juvenile idiopathic arthritis associated with Kikuchi΄s disease. [Abstr.] Ann Rheum Dis 2000; 59(9): 731.
5. Singh YP, Agarwal V, Krishnani N, Misra R. Enthesitis-related arthritis in Kikuchi-Fujimoto disease. Mod Rheumatol. 2008; 18(5): 492-495.
6. Ohta A, Matsumoto Y, Ohta T, Kaneoka H, Yamaguchi M. Still’s disease associated with necrotizing lymphadenitis (Kikuchi’s disease): report of 3 cases. J Rheumatol. 1988; 15(6): 981-983.
7. Wong VK, Campion-Smith J, Khan M, Smith S. Kikuchi disease in association with Pasteurella multocida infection. Pediatrics 2010; 125: 679-682.
8. Οgata S, Bando Y, Saito N, Katsuoka K, Ishii M. Kikuchi-Fujimoto disease developed into autoimmune disease: a report of two cases. Mod Rheumatol 2010; 20(3): 301-305.
9. Μahajan T, Merriman RC, Stone MJ. Kikuchi – Fujimoto disease (histiocytic necrotizing lymphadenitis): report of a case with other autoimmune manifestations. Proc (Bayl Univ Med Cent) 2007; 20: 149-151.
10. Child fatality associated with pathological features of histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto disease). Pediatr Pathol Lab Med 1998;18:79-88
11. Lee HY, Huang YC, Lin TY, Huang JL, Yang CP, Tsun H, et al. Primary Epstein-Barr virus infection associated with Kikuchi’s disease and hemophagocytic lymphohistiocytosis. A case report and review of the literature. J Microbiol Immunol Infect 2010; 43 (3): 253257.
12. Kim YM, Lee YJ, Nam SO, Park SE, Kim JY, Lee EY. Hemophagocytic syndrome associated with Kikuchi’s disease. J Korean Med Sci 2003;18(4): 592-594.
Georgia Avgerinou
Vasilios Papadakis
Kleoniki Roka and Sophia Polychronopoulou
Paediatric Department of Hematology-Oncology, “Aghia
Sophia” Children’s Hospital, Athens
Georgia Avgerinou, V. Papadakis, Kleoniki Roka and Sophia Polychronopoulou
Τhree children with Haemophagocytic lymphohistiocytosis (2 Males – 1 Female) with median age of 6 years are being presented. The patients were admitted to the hospital with prolonged fever, pancytopenia, hepatoslenomegaly and increased serum ferritin levels. The diagnosis was based on internationally established diagnostic criteria, present at the onset of the illness. All patients received therapy according to the HLH-2004 protocol. Two patients are in remission, alive more than ten months from diagnosis while, the third patient died due to Multiple Organ System Failure. Awareness of the clinical presentation and of the diagnostic criteria of HLH is crucial in establishing HLH diagnosis promptly and initiating life-saving combination therapy.
Key words: Haemophagocytosis, diagnosis, treatment, child
Αλληλογραφία
Γεωργία Αυγερινού
Αργοναυτών 4, 19005,
Μάκρη
τηλ: 6944944535
e-mail: g.avgerinou@yahoo.gr
Correspodence
Georgia Avgerinou
4 Argonauton St., 19005
Nea Makri
tel: +306944944535
e-mail: g.avgerinou@yahoo.gr
[2, 3, 5, 6, 7, 10, 11, 12, 13].
Συντομογραφίες
HLH = Haemophagocytic lymphohistiocytosis
ΑΦΣ = Αιμοφαγοκυτταρικό
TG = Triglycerides
NK = Natural Killer
ΜΕΘ = Μονάδα Εντατικής
FHL = Familial Haemophagocytic Lymphohistiocytosis
MAS = Macrophage Activation Syndrome IL-2
g/dL, PLT 50 k/uL), TG 626 mg/dL,
(SGOT 696 U/L, SGPT 283 U/L),
(Na 118 mmol/L), LDH ορού 3082 U/L,
1. Πυρετός
2. Σπληνομεγαλία
3. Κυτταροπενίες (να
*Αιμοσφαιρίνη <9 g/dL (νεογνά <4
*Αιμοπετάλια <10 k/uL
*Ουδετερόφιλα (ANC) < 1000
4. Υπερτριγλυκεριδαιμία και /ή υποϊνωδογοναιμία:
*Τριγλυκερίδια
5. Αιμοφαγοκυττάρωση
<10 g/dL)
(SGOT 176 IU/L, SGPT 75 IU/L),
[2, 3, 5, 6, 7, 16, 22].
[16, 22, 23].
[5, 6, 19, 20, 22].
[6, 13, 17, 18].
(X-linked lymphoproliferative syndrome: XLP),
celli (type2),
DiGeorge
Chediac-Higashi,
Ommen’s [5, 6, 15, 21].
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2.
2011;28:1-7.
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8. Eiichi Ishii, Ikuyo Ueda, Ryutaro Shirakawa, Ken Yamamoto, Hisanori Horiuchi, Shouichi Ogha et al. Genetic subtypes of familial hemophagocytic lymphohistiocytosis: correlations with clinical features and cytotoxic T lymphocyte/natural killer cell functions. Blood, 1 May 2005, Vol 105, No.9, pp3442-3448.
9. Juan Mayordomo-Colunga, Corsino Rey, Soledad Gonza’lez and Andres Concha (2008). Multiorgan failure due to hemophagocytic syndrome: A case report. Cases journal 2008, 1: 209.
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16. Henter JI, Neresmo I, (1997). Neuropathologic findings and symptoms in twenty three children with hemophagocytic lymphohistiocytosis. J. Pediatr 1997;130: 358-365.
17. Imashuku S, Kuriyama T, Ishii E,et al. Requirement for etoposide in the treatment of Epstein-Barr virus–associated hemophagocytic lymphohistiocytosis, J Clin Oncol 2001;2665-2673.
18. Ueda I, Ishii E, Morrimoto A, Ogha S, Sako M, Imashuku S. Correlation between phe-
notypic heterogeneity and gene mutational characteriostics in familial hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2006;46: 482-488.
19. Schneider EM, Lorentz I, Muller-Rosenberger M, Steinbach G, Kron M, Janka-Schaub GE. Hemophagocytic lymphohistiocytosis is associated with deficiencies of cellular cytolysis but normal expression of transcripts relevant to killer-cell-induced apoptosis.Blood 2002;100:2891-8.
20. Henter JI, Elinder G, Soder O, Hansson M, Andersson B, Andersson U. Hypercytokinemia in familial hemophagocytic lymphohistiocytosis. Blood 1991;78: 2918-22.
21. Aleman K, Noordzij JG, de Groot R, et al. Reviewing Omenn syndrome. Eur J Pediatr2001;160:718-725.
22. Χ. Κουτσαυτίκη, Ε. Μάντζιου, Ν. Μυριοκεφαλιτάκης (2009). Αιμοφαγοκυτταρική Λεμφοϊστιοκυττάρωση. Παιδιατρική 2009;72: 89-96.
23. Παπαδάκης Βασίλειος. Αιμοφαγοκυτταρικά Σύνδρομα. Ημερίδα
Αιματολογίας; 2009. Σελ. 69-78.
