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Παιδιατρική | Τόμος 74 • Τεύχος 4 • Οκτώβριος - Νοέμβριος - Δεκέμβριος 2011

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Μ. Κανάριου

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Β. Παπαευαγγέλου

Α. Παπαθανασίου

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Α. Συρίγου-Παπαβασιλείου

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Κλινικοεργαστηριακά

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καρκίνο. Επταετής εμπειρία ενός ογκολογικού τμήματος Δ. Δογάνης, Α. Πουρτσίδης, Ε. Λεμπέση, Δ. Μπουχούτσου, Μ. Mπάκα, Μ. Βαρβουτσή, Μ. Φουστούκου, Ε. Κοσμίδη ΕΝΔΙΑΦΕΡΟΥΣΕΣ ΠΕΡΙΠΤΩΣΕΙΣ

παιδιά

Paediatriki

Volume 74 • Number 3 • October-November-December 2011

Trimonthly publication of the Greek Paediatric Society

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CONTENTS

EDITORIAL

SPECIAL ARTICLE

Moral dilemmas in modern medicine

F. Papadellis

REVIEW ARTICLES

Brain glucose metabolism and epilepsy in childhood

S. G. Varlamis, A. Evangeliou

Τhe polymorphisms of vitamin D receptor gene and their relationship with growth

E. Emmanouilidou, A. Galli-Tsinopoulou

Neonatal thrombocytopenia

L. Kossiva, T. Sdogou

ORIGINAL ARTICLES

An animal facilitated active with companion animal in a greek public kindergarten

K. Loukaki, M. Myriokefalitaki, E. Ktenas, D. Kafetzis, P. Koukoutsakis

Clinical and laboratory characteristics of bloodstream infections in children with cancer. A 7-year experience at a single center

D. Doganis, A. Pourtsidis, E. Lempesi, D. Mpouhoutsou, M. Mpaka, M. Varvoutsi, M. Foustoukou, E. Kosmidi

CASE REPORTS

Recurrent bacterial infections: Caroli’s Disease (case report & review of literature)

M. Tzoufi, M. Rogalidou, E. Drimtzia, I. Sionti, M. Argyropoulou, E.V. Tsianos, A. Siamopoulou-Mavridou

Case report and review of clinical and laboratory evidence of leukemia in children with bone pain

Ε. Mantadakis, Α. Valsamidis, Α. Hatzimichail

An infant with severe pulmonary arterial hypertension was treated successfully with sildenafil and bosentan

C. Barbaresou, E. Gaki, H. Tsipou, M. Kazantzi, T. Xatzis

Hypereosinophilic syndrome in a haemodialysis child

M. Mila, M. Baka, A. Mitsioni, A. Zampetoglou, E. Kosmidi, K. Stefanidis

Acute otitis media with intracranial complications

Ε. Papadimitriou, L. Giannopoulou, S. Kouskouvekakis, D. Haniotakis, F. Tsitsopoulos, Ι. Kavaliotis

Περίληψη

Abstract

Sotirios G. Varlamis1 Laboratory of Biochemistry, Medical School of Aristotle University of Thessaloniki, Greece.

Athanasios Evangeliou2 4th Department of Pediatrics, Aristotle University of Thessaloniki, Papageorgiou Hospital, Thessaloniki, Greece.

Brain glucose metabolism and epilepsy in childhood

Sotirios G. Varlamis1 , Athanasios Evangeliou2

Glucose is the main fuel of the brain. Ketone bodies act as an alternative fuel in the case of fasting or diet. Metabolic pathways are related to synaptic activity at the level of neuron - glia. There is strong evidence suggesting that neurometabolic disturbances are related to epileptogenesis, through changes occurring at neurotransmitters’ concentrations. Based on experimental data related to intermediate metabolism disturbances in reluctant epilepsy, dietary treatments contributing to the decrease of seizures with fewer side effects than drugs, have been developed. The ketogenic diet and its variations is the most important applied diet. The use of dietary treatments is mostly applied to children with reluctant epilepsy. However, the type of epilepsy does not affect the effectiveness of said diets. The most likely mechanisms of action for the ketogenic diet are related to glucose metabolism, and one of these, the theory of glycolysis, suggests that bypassing glycolysis can effectively contribute to the reduction of epileptic activity. This conclusion is further reinforced by studies suggesting the increase of lactate during seizures and other studies supporting the antiepileptic actions of 2-deoxyglucose, a glycose analogue, as proved at an experimental level in vitro and in vivo in animal models. Research regarding the pathophysiology of the disease is increasingly oriented towards the field of neurometabolism.

Key Words: Glucose, epilepsy, ketogenic diet, children

Αλληλογραφία

Βαρλάμης Σωτήριος

Λευκωσίας 26, Καλαμαριά 55133, Θεσσαλονίκη

Τηλ: 2310482220

e-mail: sotirisvarlamis@yahoo.gr

Correspodence

Varlamis Sotirios

Lefkosias 26, Kalamaria 551133, Thessaloniki, Greece.

Τel: +30 2310482220

e-mail: sotirisvarlamis@yahoo.gr Εισαγωγή

2DG

GNU = μονάδα νευρώνα–νευρογλοίας

GABA = γ-αμινοβουτυρικό οξύ

DON = 6-διαζο-5-οξο-Lνορλευκίνη

AEDs = αντιεπιληπτικά φάρμακα

MCT1 = μονοκαρβοξυλικοί

υποδοχείς 1

ATP = τριφωσφορική αδενοσίνη

Επίσης,

Magistretti & Pellerin (22)

Pan JW, Williamson Anne, Cavus I, Hetherington HP, Zaveri H, Petroff OAC, Spencer DD. Neurometabolism in human epilepsy. Epilepsia 2008; 49(Suppl. 3): 31-41).

Οι Pan et al (20),

Stafstrom CE, Roopra A, Sutula TP. Seizure suppression via glycolysis inhibition with 2-deoxy-D-glucose (2DG). Epilepsia 2008; 49 Suppl 8: 97-100).

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21. Pfund Z, Chugani DC, Juhász C, Muzik O, Chugani HT, Wilds IB, et al. Evidence for coupling between glucose metabolism and glutamate cycling using FDG PET and 1H magnetic resonance spectroscopy in patients with epilepsy. J Cereb Blood Flow Metab. 2000;20:871-8.

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Abstract

Eleftheria Emmanouilidou

Assimina Galli-Tsinopoulou

4th Department of Pediatrics, Medical School, Aristotle University of Thessaloniki, Papageorgiou General Hospital, Thessaloniki, Greece

Τhe

polymorphisms of vitamin D receptor gene and their relationship with growth

As Vitamin D plays an important role in bone metabolism, it can be considered as a possible regulator of growth during infancy, childhood and adulthood. The biological actions of 1α,25(ΟΗ)2D3 are expressed after its binding with an intracellular receptor, the Vitamin D Receptor-VDR. This review consists of two parts. The first part is a brief description of the protein, the gene and gene polymorphisms of VDR. The second part presents the literature data, regarding the relationship of VDR gene polymorphisms with anthropometric characteristics such as height and weight from the neonatal to the adult life. Most researchers support the existence of a correlation between the VDR gene and growth and final height and there are reports that relate the Fok I polymorphism of VDR gene with idiopathic short stature.

Keywords: vitamin D receptor (VDR), gene polymorphisms of vitamin D receptor, final stature, idiopathic short stature.

e-mail: galtsin@otenet.gr assimina@med.auth.gr

Correspondence

Assimina Galli-Tsinopoulou

Assistant Professor of Pediatric Endocrinology

4th Department of Pediatrics, Medical School, Aristotle University of Thessaloniki, Papageorgiou General Hospital, Thessaloniki, Greece

Τel/Fax: +30 2310-991537

e-mail: galtsin@otenet.gr assimina@med.auth.gr Aλληλογραφίας:

D (Vitamin D Receptor: VDR)

Polymorphisms-SNPs)

BsmI (Bb), TaqI(Tt)

(short polyA stretch, n=13-17).

ApaI (22-25), BsmI (22, 23, 25-27), TaqI (22, 23, 24) και FokI (21, 23, 24, 28).

VDR [1521 bp (G/C), 1012 bp (A/G)].

παραπάνω SNPs, των 1521G/1012A και 1521C/1012G (ομόζυγοι

1012AA, 1521CC/1012GG /ετερόζυγος 1521GC/1012AG).

1521C/1012G.

1521CC/1012GG

Fok I (57).

Fok I (59).

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47. Gomez C, Naves ML, Barrios Y, Diaz JB, Fernandez JL, Salido E, Torres A, Cannata JB. Vitamin D receptor gene polymorphisms, bone mass, bone loss and prevalence of vertebral fracture: differences in postmenopausal women and men. Osteoporos Int 1999; 10:175-182

48. Kikuchi R, Uemura T, Gorai I, Ohno S, Minaguchi H. Early and late postmenopausal bone loss is associated with Bsm I vitamin D receptor gene polymorphism in Japanese women. Calcif Tiss Int 1999; 64:102-106

49. Efstathiadou Z, Kranas V, Ioannidis JPA, Georgiou I, Tsatsoulis A. The Sp1 COLIA1 gene polymorphism, and not Vitamin D receptor or Estrogen receptor gene polymorphisms, determines bone mineral density in postmenopausal Greek women. Osteoporos Int 2001; 12:326-331

50. Kim JG, Kwon JH, Kim SH, Choi YM, Moon SY, Lee JY. Association between vitamin D receptor gene haplotypes and bone mass in postmenopausal Korean women. Am J Obstet Gynecol 2003; 189:1234-1240

51. Garnero P, Munoz F, Borel O, Sornay-Rendu E, Delmas PD. Vitamin D receptor gene polymorphisms are associated with the risk of fractures in postmenopausal women, independently of bone mineral density. The OFELY study. J Clin Endocrinol Metab 2005; 90:4829–4835

52. Qin YJ, Zhang ZL, Huang QR, He JW, Hu YQ, Zhou Q, Lu JH, Li M, Liu YJ. Association of vitamin D receptor and estrogen receptor-α gene polymorphism with peak bone mass and bone size in Chinese women. Acta Pharmacol Sin 2004; 25:462-464

53. Kurabayashi T, Matsushita H, Tomita M, Kato N, Kikuchi M, Nagata H, Honda A, Yahata T, Tanaka K. Association of vitamin D and estrogen receptor gene polymorphism with the effects of long term hormone replacement therapy on bone mineral density. J Bone Miner Metab 2004;22:241-247

54. Nguyen TV, Esteban LM, White CP, Grant SF, Center JR, Gardiner EM, Eisman JA. Contribution of the collagen I_ 1 and vitamin D receptor genes to the risk of hip fracture in elderly women. J Clin Endocrinol Metab 2005; 90:6575–6579

55. Lucotte G, Mercier G and Burckel A. The vitamin D receptor FokI start codon polymorphism and bone mineral density in osteoporotic postmenopausal French women. Clin Genet 1999; 56: 221-224

56. Wynne F, Drummond F, O’Sullivan K, Daly M, Shanahan F, Molloy MG, Quane KA. Investigation of the genetic influence of the OPG, VDR (Fok1), and COLIA1 Sp1 Polymorphisms on BMD in the Irish population. Calcif Tissue Int 2002; 71:26-35

57. Ferrari S, Manen D, Bonjour JP, Slosman D, Rizzoli R. Bone mineral mass and calcium and phosphate metabolism in young men: relationships with vitamin D receptor allelic polymorphisms. J Clin Endocrinol Metab 1999; 84:2043-2048

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59. Windelinckx A, De Mars G, Beunen G, Aerssens J, Delecluse C, Lefevre J, Thomis MAI. Polymorphisms in the vitamin D receptor gene are associated with muscle strength in men and women. Osteoporos Int 2007; 18:1235-1242

60. Lei SF, Wang YB, Liu MY, Mo XY, Deng HW. The VDR, COL1A1, PTH, and PTHR1 gene polymorphisms are not associated with bone size and height in Chinese nuclear families. J Bone Miner Metab 2005;23:501-505

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Lydia Kossiva

Triantafyllia Sdogou

Second department of Paediatrics, University of Athens

“P. & A. Kyriakou” Children Hospital, Athens Greece

Neonatal thrombocytopenia

Kossiva, Triantafyllia Sdogou

During gestation the production of platelets from megakaryocytes starts in the liver and continues in the bone bone marrow. Adequate number of platelets is achieved at the 18th week of gestation. Thrombocytopenia is common among sick neonates, affecting 20-35% of all patients admitted to the neonatal intensive care unit (NICU). While most cases of neonatal thrombocytopenia are mild to moderate and resolve in a short-time period with appropriate therapy, 2.5-5% of NICU patients experience severe thrombocytopenia lasting longer and requiring platelet transfusions.

Keywords: Neonate, thrombocytopenia

e-mail: lydiakossiva@hotmail.com

Correspondence:

Lydia Kossiva

Second Department of Paediatrics, Medical School, University of Athens

“P. & A. Kyriakou” Children’s Hospital, Athens, Greece e-mail: lydiakossiva@hotmail.com

Συντομογραφίες NICU: neonatalintensivecareunit

Abbreviations: NICU: neonatal intensive care unit

Αυτοάνοση

Σ.Wiskott-Aldrich

•

• Κληρονομικές διαταραχές (σ. TAR, σ.Wiskott-Aldrich) Θρομβοπενία

• Πλακουντιακή ανεπάρκεια

• Ασφυξία

• ΔΕΠ

• Περιγεννητικήλοίμωξη (groupBstreptococcus, E. coli, Listeria)

• Συγγενείς λοιμώξεις

• Αλλοάνοση θρομβοπενία

• Θρόμβωση

• Συγγεννής

•

•

•

•

•

Kasabach-Merritt

Bernard-Soulier, Alport) •

Wiskott-Aldrich

1.Sola MC, Del Vecchio A, Rimza LM. Evaluation and treatment of thrombocytopenia in the neonatal intensive care unit. Clin Perinatol 2000;27:655-79

2.Dreyfus M, Kaplan C, Verdy E, et al. Frequency of immune thrombocytopenia in newborns: a prospective study. Immune Thrombocytopenia Working Group. Blood 1997;89:4402-6

3.Roberts I, Murray NA. Neonatal thrombocytopenia: causes and management. Arch Dis Child Fetal Neonatol Ed 2003;88:F 359-F364

4.Bussel JB, Zacharoulis S, Kramer K, et al. Clinical and diagnostic comparison of neonatal alloimmune thrombocytopenia to non-immune cases of thrombocytopenia. Pediatr Blood Cancer 2005;45:176-83

5.Bussel JB, Jumbelic MI, Ancona RJ, et al. Clinical and diagnostic comparison of neonatal alloimmune thrombocytopenia to non-immune cases of thrombocytopenia. Pediatr Blood Cancer 2005;45:176-83

6.Aspr o usb Bussel 2009

7.Mao C, Guo J, Chituwo BM. Intraventricular haemorrhage and its prognosis, prevention and treatment in term infants. J Trop Pediatr 1999;45:237-40

8.Bassler D Greinacher A, Okascharoen C, et al. Systematic review and survey of the management of unexpected neonatal alloimmune thrombocytopenia. Transfusion 2008;48(1):92-98

9.Stavrou E, McCrae KR. Immune thrombocytopenia in pregnancy. Hematol Oncol Clin North Am. 2009;23(6):1299-316

10. Kaplan C. Neonatal alloimmune thrombocytopenia. Haematologica 2008;93(6):805807

11. Rayment R, Brunskill SJ, Soothill PW, et al. Cochrane Database Syst Rev. 2011. 11;5: CD004226

12. Skogen B, Killie MK, Ahlen MT, et al. Reconsidering fetal and neonatal alloimmune thrombocytopenia with a focus on screening and prevention. Expert Rev Hematol 2010;3 (5): 559-66

13. Kjeldsen-Kragh J, Killie MK, Tomter G, et al. A screening and intervention program aimed to reduce mortality and serious morbidity associated with severe neonatal alloimmune thrombocytopenia. Blood 2007;110:833-839

14. Roberts IA, Muray NA. Thrombocytopenia in the newborn. Curr Opin Pediatr 2003;15:17-23

15. Murray NA. Evaluation and treatment of thrombocytopenia in the neonatal intensive care unit. Acta Paediatr Suppl, 2002; 91:74-81

16. Bussel JB, Marcia MD, Zabusky BA, et al. Fetal alloimmune thrombocytopenia. New Engl J Medicine. 1997; 337:22-6

17. Vinograd CA, Bussel JB. Antenatal treatment of fetal alloimmune thrombocytopenia: a current persective. Haematologica 2010;95:1807-11

18. Althaus K, Greinacher A. MYH9-Related Platelet Disorders. Seminars in Thrormbosis and Hemostasis. 2009;35:189-203

19. Skogen B, Husebekk A, Killie MK, et al. Neonatal alloimmune thrombocytopenia is not what it was. A lesson learned from a large prospective screening and intervention program. Scand J Immunol. 2009;70: 531-34

20. Birchall JE, Murphy MF Kaplan C, et al. European Fetomaternal Alloimmune Thrombocytopenia Study Group. European collaborative study of the antenatal management of feto-maternal alloimmune thrombocytopenia. Br J Haemantol 2003; 122: 275-88

21. Nugent DJ. Immune thrombocytopenic purpura in childhood. Hematology 2006;97103

22. Balduini CL, Cattaneo M, Fabris F, et al. Italian Gruppo di Studio delle Piastrine. Inherited thrombocytopenias: a proposed diagnostic algorithm from the Italian Gruppo di Studio delle Piastrine. Haematologica. 2003;88:582-92

23. Balduini CL, Iolascon A, Savoia A. Inherited thrombocytopenias: from genes to therapy. Haematologica. 2002;87:860-80

24. Geddis AE, Kaushansky K. Inherited thrombocytopenias: toward a molecular understanding of disorders of platelet production. Curr Opin Pediatr. 2004;16: 15-22

25. Ghevaert C, Campbell K, Walton J, et al. Management and outcome of 200 cases of fetomaternal alloimmune thrombocytopenia. Transfusion 2007;47:901-10

26. Josephson CD, Su LL, Christensen RD, et al. Platelet transfusion Practices Among Neonatologists in the United States and Canada: Results of a Survey. Pediatrics 2009;123(1):278-285

An Animal facilitated active with companion animal in a greek public kindergarten

Katerina Loukaki1, Maria Myriokefalitaki2, Eytyxios Ktenas3, Dimitrios Kafetzis1, Petros Koukoutsakis1

In recent years the contribution of pets in the socialization, communication and emotional expression as long as the education of the people and especially children is achieved by specialized methods of intervention referred to as Animal facilitated activities. Children - method: We studied and recorded the reactions of children and staff, at a presence of a pet, for the first time in Greece in the environment of a public Kindergarten. We selected a group of 39 health children who were just enrolled in the program of kindergarten, aged 2,5 - 4 years and a rabbit as a pet. Developed risk questionnaires were

Katerina Loukaki1

Dimitrios Kafetzis1

Petros Koukoutsakis1

2nd Department of Paediatrics, Medicine School, of Athens University, P. & A. Kyriakou Children Hospital

Maria Myriokefalitaki2

Public Nursery School of Municipality of Helioupolis, Athens

Eytyxios Ktenas3

National School of Public Health, Domain of Epidemiology

established concerning the control of socialization, communication, emotional expression and influencal contact the animal had on children. As a method of statistical analysis, in addition to the description of the data, we used the simple analysis of variance measures.

Results: it was found that in the presence of the animal both the socialization and communication but also the emotional expression of children grew gradually from measurement to measurement. The nursery teachers recorded progressively increasing impact of the animal and considered the intervention as very positive regarding the socialization, communication and emotional expression of children.

Conclusion: The implementation of an Animal facilitated activity program in public kindergarten is feasible and effective, giving the children the opportunity of learning, pleasant living and the chance to have a closer contact with nature.

Keywords: companion animal, rabbit, animal facilitated activity, kindergarten

e-mail: loukaki1@otenet.gr

Correspondence:

Katerina Loukaki

Protopapa 29, Helioupolis

16342 Athens

Τel.: +30 2109932295

e-mail: loukaki1@otenet.gr

1. SherpellA.James. Animal - AssistedInterventionsinHistoricalPerspective cited InAnimalAssistedtherapyHandbooktheoreticalfoundationasandGuidelinesforPractice (A.H.Fine, ed) 2ndEdition, 2006 AcademicPressElsevierLondon, pp3-30

2. NimerJ., Lundahl (2006) AnimalAssistedTherapy: Ametanalysis, Anthrozoos, Volume 20, issue3 p.225-238

3. Owens R.&Williams N (1995) A new breed of teachers Pet. Teaching PreK-26, (50-54)

4. BrandleyBays, T., Lighthoot T., Mayer J. (2006) Exotic Pet Behavior: Birds, Reptiles and Small Mammals, Saunders, Elsevier (25-38)

5. Brown-HarcourtFr. (2001) TextbookofRabbitMedicine, AcademicPressVet.R, Butterworth-Heinemann; 1st ed. (45-52)

6. Delta Society. Definitions Development Task Force of the Standards Committee (1992) Generic Terms and Definitions. Handbook for the Animal Assisted Activities and Animal Assisted Therapy, Renton, Delta Society (ed), WA(25-45)

7. Delta Society (2005), Animals in the classroom Handbook (35-55)

8.

2003 (22-48)

9. Melson G.F (2003), Child Development and Human Companion Animal Bond, American Behavioral Scientist, September, vol47, no.1, p.31-39

10. Shalev A., Ben-Mondehai, Dror (1996) Snakes: Interactions with children with disabilities and the elderly-Some psychological consideration, Anthrozoos: volume 9, number 4, pp.182-187

11. Adbill M. N, Juppe D(2000) Pets in Therapy, Ravensdale, Idyll Arbor, Inc., WA, (55-83)

12. Kaminski M., T.Pellino and J.Wish (2002), Children ‘s Health Care, v31, p.321-335

13. Dimitrijevic I (2009) Animal Assisted therapy a new trend in the treatment of children and adults, PsychiatriaDnubina 21(2): 236-41

14. Arken M (1984) There is a real dog in the Classroom. ChildrensEnviromentQualterly 1(3) 23-26

15. ManiI., MaguireJ.H (2009), Small Animal Zoonoses and immunocompromised pet owners. Top Companion Anim. Med 24(4): 164-174

16. Morrison G. (2001) Zoonotic infections from pets. Understanding the risks and treatment. Postgraduate Medicine 110(1): 24-26

17. Condoret A (1983). Speech and Companion Animals: Experiments with normal and disturbed nursery school children, cited in A. Katcher and A.Beck (eds). New Perspectives in our lives with Companion Animals, Philadelphia, PA: University Pennsylvania press (125-185)

18. RudA.G., Beck A.M. (2003) Companioin Animals in Indiana elementary schools, Anthrozoos,16, pp.241-253

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domonas sp (15), Klebsiella sp (10), Staphylococcus aureus (12,

(72%)

Hickman / Broviac (91%)

Staphylococcus coagulase negative (32).

Clinical and laboratory characteristics of bloodstream infections in children with cancer. A 7-year experience at a single center

Doganis Dimitrios, Pourtsidis Apostolos, Lempesi Evangelia, Bouhoutsou Despina, Baka Margarita, Varvoutsi Maria, Foustoukou Maria, Kosmidi Helen

Abstract

Background-methods: Pediatric cancer patients have an increased risk of potentially lifethreatening infections such as bloodstream infections (BSI). In this study our aim was to

Doganis Dimitrios

MD

Pourtsidis Apostolos

MD

Lebessi Evangelia*

MD

Bouhoutsou Despina

MD

Baka Margarita

MD

Varvoutsi Maria

MD

Foustoukou Maria*

MD

Kosmidis Helen

MD

Oncology Department, Microbiology Department*, «P & A Kyriakou» Children’s Hospital, Athens

evaluate the laboratory and clinical characteristics of children with cancer and BSI who were treated in our department during 7 years. We recorded demographic characteristics, clinical and laboratory findings, treatment variables as well as the outcome and the complications during these episodes. We also correlated all these factors with the type of underlying malignancy: children treated for leukemia and children treated for lymphoma or solid tumors

Results: We evaluated 138 consecutive BSI detected in 58 children treated for leukemia (90 BSIs) and in 41 children treated for lymphoma or solid tumors (48 BSIs). Twenty six children (21 treated for leukemia and 5 treated for lymphoma or solid tumors, p=0.015) had more than one distinct episodes. In patients suffering from Leukemia we detected more frequently gram negative organisms (57%) whereas in patients suffering from lymphoma or solid tumor the majority of isolated organisms were gram positive (64%). The most common organisms isolated were: Escherichia coli (23 episodes, more frequently in leukemia patients), Pseudomonas sp (15), Klebsiella sp (10), Staphylococcus aureus (12, more frequently in lymphoma / solid tumor patients) and Staphylococcus epidermidis (32). In 99 BSIs (72%), children were neutropenic whereas Hickman / Βroviac type catheters were used in 125 BSI (91%) without differences concerning the disease. The incidence of catheter related infection was 2.53 episodes per 1000 catheter days -2.45 and 2.7 in leukemia patients and lymphoma / solid tumor patients respectively, p=ns. Serious medical complications were present in 29 (21%) episodes -in 22 and 7 BSI in leukemia patients and lymphoma / solid tumor patients respectively, p=ns. No death was attributed to infection alone in this group of patients.

Conclusions: There was increased risk for more than one distinct bloodstream infection episodes in children treated for leukemia. Escherichia coli was isolated more frequently in children suffered from leukemia and Staphylococcus aureus in children with lymphoma or solid tumors. The presence of serious medical complications did not differ significantly between the two groups of patients.

Κey words: children, cancer, blood stream infections

Αλληλογραφία

Δογάνης Δημήτριος

Παιδίατρος Ογκολόγος,

Επιμελητής Ογκολογικό Τμήμα, Νοσοκομείο Παίδων

«Π. & Α. Κυριακού»

Καπετάν Πετρούτσου 12,

Αμπελόκηποι, 11523, Αθήνα

Τηλ.: 210 7707775 (εργασίας) e-mail: doganisd@gmail.com

Correspodence

Doganis Dimitrios

Pediatric Oncologist

Oncology Department, «P & A Kyriakou» Children’s Hospital

12 Kapetan Petroutsou st, 115 23 Athens, Greece

Τel.: +30 210 7707775

e-mail: doganisd@gmail.com

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29. Sasse EC, Sasse AD, Brandalise S, Clark OA, Richards S. Colony stimulating factors for prevention of myelosupressive therapy induced febrile neutropenia in children with acute lymphoblastic leukemia. Cochrane Database Syst Rev. 2005;3:CD004139

30. Sung L, Nathan PC, Lange B, Beyene J, Buchanan GR. Prophylactic granulocyte colony-stimulating factor and granulocyte-macrophage colony-stimulating factor decrease febrile neutropenia after chemotherapy in children with cancer: a meta-analysis of randomized controlled trials. J Clin Oncol. 2004;22:3350-3356

31. Creutzig U, Zimmermann M, Lehrnbecher T, Graf N, Hermann J, Niemeyer CM, Reiter A, Ritter J, Dworzak M, Stary J, Reinhardt D. Less toxicity by optimizing chemotherapy, but not by addition of granulocyte colony-stimulating factor in children and adolescents with acute myeloid leukemia: results of AML-BFM 98. J Clin Oncol. 2006;24:4499-4506

32. Santolaya ME, Cofre J, Beresi V. C-reactive protein: Valuable aid for the management of febrile children with cancer and neutropenia. Clin Infect Dis. 1994;18:589-595

33. Opilla M. Epidemiology of bloodstream infection associated with parenteral nutrition. Am J Infect Control. 2008;36:S173.e5-8

34. Sutter D, Stagliano D, Braun L, Williams F, Arnold J, Ottolini M, Epstein J. Polymicrobial bloodstream infection in pediatric patients: risk factors, microbiology, and antimicrobial management. Pediatr Infect Dis J. 2008;27:400-405

35. Van Houten MA, Uiterwaal CS, Heesen GJ, Arends JP, Kimpen JL. Does the empiric use of vancomycin in pediatrics increase the risk for Gram-negative bacteremia? Pediatr Infect Dis J. 2001;20:171-177

36. Stamou SC, Maltezou HC, Pourtsidis A, Psaltopoulou T, Skondras C, Aivazoglou T..Hickman-Broviac catheter-related infections in children with malignancies. Mt Sinai J Med. 1999;66:320-326

37. Mermel LA, Allon M, Bouza E, Craven DE, Flynn P, O’Grady NP, Raad II, Rijnders BJ, Sherertz RJ, Warren DK. Clinical practice guidelines for the diagnosis and management of intravascular catheter-related infection: 2009 Update by the Infectious Diseases Society of America. Clin Infect Dis. 2009;49:1-45

38. Dudgale DC, Ramsey PG. Staphylococcus aureus bacteremia in patients with Hickman catheters. Am J Med. 1990;89:137-41

39. Aquino VM, Pappo A, Buchanan GR, Tkaczewski I, Mustafa MM. The changing epidemiology of bacteremia in neutropenic children with cancer. Pediatr Infect Dis J. 1995;14:140-143

40. Harms D, Gortitz I, Lambrecht W, Kabisch H, Erttmannn R, Janka-Schaub G. Infectious risks of Broviac catheters in children with neoplastic diseases: A matched pairs analysis. Pediatr Infect Dis J. 1992;11:1014-1018

41. Aledo A, Heller G, Ren L, Gardner S, Dunkel I, McKay SW, Flombaum C, Brown AE. Septicemia and septic shock in pediatric patients: 140 consecutive cases on a pediatric hematology-oncology service. J Pediatr Hematol Oncol 1998;20:215-221

42. Hakim H, Flynn PM, Knapp KM, Srivastava DK, Gaur AH. Etiology and clinical course of febrile neutropenia in children with cancer. J Pediatr Hematol Oncol. 2009;31:623-629

43. Roilides E, Sidi V, Gompakis N, Tsivitanidou M, Katsaveli A, Koliouskas D. Patterns and outcome of septicemia in neutropenic children with cancer in a Greek hospital. Infection. 1998;26:189-191

44. Rubio M, Palau L, Vivas JR, del Potro E, Diaz-Mediavilla J, Alvarez A, Martinez R, Picazo JJ. Predominance of Gram-positive microorganisms as a cause of septicemia in patients with hematological malignancies. Infect. Control Hosp. Epidemiol. 1994;15:101–104

45. Santolaya ME, Villarroel M, Avendaño LF, Cofré J. Discontinuation of antimicrobial therapy for febrile, neutropenic children with cancer: Prospective study. Clin Infect Dis. 1997;25:92-97

46. Santolaya ME, Alvarez AM, Avilés CL, Becker A, Cofré J, Enríquez N, O’Ryan M, Payá E, Salgado C, Silva P, Tordecilla J, Varas M, Villarroel M, Viviani T, Zubieta M. Prospective evaluation of a model of prediction of invasive bacterial infection risk among children with cancer, fever, and neutropenia. Clin Infect Dis. 2002;35:678–683

47. Gray J, Gossain S, Morris K. Three-year survey of bacteremia and fungemia in a pediatric intensive care unit. Pediatr Infect Dis J. 2001;4:416–421

48. Wisplinghoff H, Seifert H, Tallent SM, Bischoff T, Wenzel RP, Edmond MB. Nosocomial bloodstream infections in pediatric patients in United States hospitals: epidemiology, clinical features and susceptibilities. Pediatr Infect Dis J. 2003;22:686-691

49. Falagas ME, Kazantzi MS, Bliziotis IA. Comparison of utility of blood cultures from intravascular catheters and peripheral veins: a systematic review and decision analysis. J Med Microbiol. 2008;57:1-8

(έτη)

ΠΑΘΟΓΟΝΑ (gram)

(19%)

ΛΕΥΧΑΙΜΙΑ: 58*

ΟΛΛ: 44 ΟΜΛ: 13

1

5 6/12 33:25 21* (36%)

25

(41%)

(43%)

Gram pos 68 (50%)

Gram neg 68 (50%)

Escherichia coli : 23

Pseudomonas sp:15

Klebsiella sp : 10

Str pneumonia : 6

S aureus : 12

CNS: 32

Candida sp : 2

: 24

(0.1-410)

(0-96.4)

(72%)

39 οC

36/90 (40 %)

38/90 (42%)

Gram positive 38 (43%)

Gram negative 51 (57%)

Escherichia coli : 20

Pseudomonas sp:8

Klebsiella sp : 8

Str pneumonia : 5

S aureus : 3

CNS : 19

Candida sp : 1

Άλλο : 17

9

0.7 (0.1-410)

0 (0-95.9)

73 (81%)

64/73 (88%

5 μέρες

7

30 (2-715)

58 (0-598)

2/90 (2%)

ΣΥΜΠΑΓΕΙΣ ΟΓΚΟΙ /ΛΕΜΦΩΜΑΤΑ: 41*

ΝΒΛ: 10

ΛΕΜΦΩΜΑ: 8

(Burkitt 6, Aναπλαστικό 2)

WILMS: 4

EWING: 4

ΟΣΤΕΟΣΑΡΚΩΜΑ: 3

ΡΜΣ: 2

ΟΓΚΟΙ ΚΝΣ: 2

ΑΛΛΟΙ ΤΥΠΟΙ: 8

48 ΕΠΕΙΣΟΔΙΑ

4 8/12 21:20 5* (12%) ΠΡΩΤΗΣ

21/48 (44%)

22/48 (46%)

Gram positive 30 (64%)

Gram negative 17 (36%)

Escherichia coli : 3

Pseudomonas sp:7

Klebsiella sp : 2

Str pneumonia : 1

S aureus : 9

CNS : 13

Candida sp : 1 Άλλο : 7 5 1.35 (0.1-29) 26.1 (0-96.4) 26 (54%) 21/26 (81%) 2.5 μέρες 14.4 106 (7-1000 54 (3-231) 1/48 (2%)

65/138 (47%) 46/90 (51%)

12/138 (9%) 7/90 (8%) 5/48 (10%)

81/138 (59%) 64/90 (71%) 17/48 (35%) 0.000

10/138 (7%) 6/90 (7%) 4/48 (8%)

10/138 (7%) 2/90 (2%) 8/48 (17%) 0.0056

Abstract

Meropi Tzoufi1

Maria Rogalidou1

Ecaterini Drimtzia1

Irini Sionti1

Antigone Siamopoulou-Mavridou1

Depts of Child Health of the University of Ioannina Medical School, Ioannina, Greece

Maria Argyropoulou2 Clinical Radiology

Epameinondas V. Tsianos3 Internal Medicine

Recurrent bacterial infections, Caroli’s Disease

Meropi Tzoufi1, Maria Rogalidou1, Ecaterini Drimtzia1, Irini Sionti1 , Maria Argyropoulou2 , Epameinondas V. Tsianos3 , AntigonSiamopoulou-Mavridou1

Caroli’s disease is a rare congenital disorder characterized by cystic dilatation of the large intrahepatic bile ducts. The most frequent complications due to biliary stasis are cholelithiasis, cholangitis and sepsis as well as an increased risk of cholangiocarcinoma. The clinical course can be asymptomatic for the first 5-20 years or patients may have a history of intermittent abdominal pain, pruritus and/or symptoms of cholangitis. It is rarely diagnosed in childhood.

A 12-year old boy with isolated Caroli’s disease is described. This child presented since the age of 2 years, 4 episodes of recurrent bacterial infections (interestingly remained in between asymptomatic for over 10 years). The diagnosis was made during the last hospi-

Tηλ: 6944773233

e-mail: rogalidoum@yahoo.com

Correspondence: Maria Rogalidou, Department of Pediatrics, University Hospital of Ioannina, 45500, Greece

Tel +30 6944773233

e-mail: rogalidoum@yahoo.com

talization, when he presented, during the period of one month, two episodes of fever and slight abdominal pain, symptoms suspected of cholangitis. The diagnosis was made on the base of radiological findings: U/S, CT, MRI and especially with MRCP, in relation with the clinical picture. Since then he has been followed-up systematically for ten years and remains in good clinical condition without further relapses and with unchanged radiological findings. This is particularly rare for the specific disease.

In Conclusion: Caroli’s disease although a rare disease, should be included in differential diagnosis of children with recurrent episodes of fever especially when symptoms from gastrointestinal system and even mild are present. In addition the benign course of the disease is very rare, but not a non-existent development.

Key words: Caroli’s disease, recurrent bacterial infections, childhood

NYHA: New York Heart Association ΜΕΝΝ:

New York Heart Association

WHO: World Health Organization

QP/Qs: pulmonary blood flow\ systemic blood flow

Rp: resistance pulmonary

Φ.Τ.:

(10, 16).

(AST, ALT,

CEA, CA19-9,

1.Caroli JR, Soupault J, Kossakowski L, Plocker M, Paradowska: La dilatation polykystique congenitale des voies biliares intraphepatiques: essai de classification. Semin Hop Paris 1958; 34: 488-95

2.Taylor AC, Palmer KR. Caroli’s disease Eur J Gastroenterol Hepatol 1998; 2: 105-108

3.Veigel MC, Focht JP, Rodriguez MG, Zinati , Shao L, Moore CA et al. Fibrocystic liver disease in children. Ped Radiol 2009;39:317-27

4.Miwala F, Segev D, Thuluvath P. Caroli’s disease and outcomes after liver transplantation. Liver Transplantation 2008;14:11-7

5.Yoshizawa K, Kiyosawa K, Yabu K, Usada S, ShimizuZ, Fujimori Y et al. Caroli’s disease in three siblings. Gastroent Jpn 1992; 27: 780-4

6.Tsuchida Y, Sato T, Sango K, Etoh T, Hata K, Terawaki K et al Evaluation of long- term results of Caroli’s disease: 21 years observation of a family with autosomal “dominant” inheritance, and review of the literature. Hepatogastroenterology 1995; 42:175-181

7.Parada La, Hallen M, Hagestrand I, Tranberg KG, Johansson B. Clonal chromosomal abnormalities in congenital bile duct dilatation (Caroli’s disease). Gut 1999;45:780-2

8.Jung G, Benz-Bohm G, Kugel H, Keller KM, Querfield U. MR cholangiography in children with autosomal recessive polycystic kidney disease. Pediatr Radiol 1999; 29: 463-466

9.Pirson Y, Lannoy N, Peters D, Geubel A, Gigot JF, Breunning M. Isolated polycystic liver disease as a distinct genetic disease, unlinked to polycystic kidney disease 1 and polycystic kidney disease. Hepatology 1996; 23: 249-252

10. Itai Y, Ebihara R, Eguchi N, Saida Y, Kurosaki Y, Minami M. Hepatobiliary cysts in patients with autosomal dominant polycystic kidney disease: prevalence and CT findings. AJR 1995; 164: 339-342

11. Mousson C, Rabec M, Cercueil JP, Viret JS, Hillon P, Rifle G. Caroli’s disease and autosomal dominant polycystic kidney disease: a rare association? Nephrol Dial Transplant 1997; 12: 1481-1483

12. Desmet V. Pathogenesis of ductal plate abnormalities. Mayo Clin Proc 1998; 73: 80-9

13. Summerfield JA, Nagufuchi Y, Sherlock S, Cadafalch J, Scheuer P. Hepatobiliary fibropolycystic diseases. A clinical and histological review of 51 patients. J Hepatol 1986; 2:141-156

14. Bernstine J. What is Caroli’s disease? Gastroenterology 1975; 68: 417-419

15. Gupta AK, Gupta A, et al Caroli’s Disease. Indian Journal of Pediatrics 2006;73:233-5

16. Hussain SZ, Bloom DA, Tolia V: Caroli’s disease diagnosed in a child by MRCP. Clin Imag 2000; 24: 289-291

17. Keramidas D.C, Kapouleas G.P, Sakellaris G. Case Report. Isolated Caroli’s disease presented as an exophytic mass in the liver. Ped Surg Internat 1998; 13: 177-179

18. Fagundes-NetoU, Schettini ST, Wehba J, Pinus J, Patricio FR. Caroli’s disease in childhood: report of two new cases. JPediatr Gastroenterol Nutr 1983 ; 2: 708-711

19. Todani T, Watanabe Y, Narusue M, Tabuchi K, Okajima K. Congenital bile duct cysts: Classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. Am J Surg. 1977 ;134 (2):263-9

20. Krausé D, Cercueil JP, Dranssart M, Cognet F, Piard F, Hillon P: MRI for Evaluating Congenital Bile Duct Abnormalities. J Comput Assist Tomogr 2002;24: 541-552

21. Guy F, Cognet F, Dranssart M, Cercueil JP, Conciatori L, Krause D. Caroli’s disease: magnetic resonance imaging features. Eur Radiol 2002;12:2730-6

22. Ros E, Navarro S, Bru C, Gilabert R, Bianchi L, Bruguera M. Ursodeoxycholic acid treatment of primary hepatolithiasis in Caroli’s syndrome. Lancet. 1993;342:404-6

23. Kassahun WT, Kahn T, Wittekind C et al. Caroli’s disease: liver resection and liver transplantation. Experience in 33 patients. Surgery 2005;138:888-98

24. Espinoza R, San Martin S, Court F, et al. hepatic resection in localized Caroli disease.

Rev Med Chil. 2003;131:183-9

25. Mabrut JY, Partensky C, Jaeck D, Oussoultzoglou E, Baulieux J, Boillot O et al Congenital intrahepatic bile duct dilatation is a potentially curable disease: long-term results of a multi-institutional study. Ann Surg.2007;246:236-45

26. Habib S, Shakil O, Couto OF, Demetris AJ, Fung JJ, Marcos A et al. Caroli’s disease and orthotopic liver transplantation. Liver Transpl. 2006;12:416-21

ΤΚΕ mm/1st h

CRP(mg/L)

AST (IU/L)

ALT (IU/L)

γGT (IU/L

Αμυλάση (IU/L)

Χολερυθρίνη / άμεση (mg/dl)

Ουρία (mg/dl)

Κρεατινίνη (mg/dl)

Χρόνοι πήξης (PT, APTT, FIB)

Ανοσοσφαιρίνες, C3, C4, ANA, anti DNA

Αντισώματα για EBV, HIV, CMV, HBV, HCV, HAV, Echo, Coxsakies

Αντισώματα για for Yersinia, Bartonella, Toxoplasma, Yersinia Borellia, Leishmania, Mycoplasma pneumonia, Chlamydia psittaci, echinococcus, entamoeba histolytica, leptospira, rickettsiae

Κ/ες αίματος, ούρων,

CEA, α1AT, CER, aFP, CA19-9

Mantoux:

Ε. Mantadakis

Α. Valsamidis

Α. Hatzimichail

Case report and review of clinical and laboratory evidence of leukemia in children with bone pain

The authors present a 5-year-old boy with mild skeletal complaints for a month that was diagnosed with acute lymphoblastic leukemia (ALL) despite a normal physical examination and minimally affected complete blood count (mild anemia and lymphocytosis without blasts). Children with ALL and musculoskeletal complaints may have a deceptively normal physical examination at presentation, absence of circulating blasts, and few if any abnormalities on the initial complete blood count. The relevant medical literature is reviewed and clinical and laboratory pointers to leukemia are discussed. These include bone tenderness or pain, especially if nocturnal and non-articular, anemia of any degree, low-normal leukocytes with relative lymphocytosis, low-normal platelet counts, especially when associated with elevated erythrocyte sedimentation rate and increased serum LDH. Care is needed in the clinical and laboratory evaluation of children with leukemia and skeletal complaints in order not to miss the underlying diagnosis, misdiagnose juvenile arthritis and/or administer corticosteroids.

Key words: Leukemia, juvenile arthritis, skeletal complaints, differential diagnosis

Τηλ.: 25510-74411

Fax: 25510-30340

e-mail: emantada@med.duth.gr

Correspodence:

Elpis Mantadakis, MD

Assistant Professor of Pediatrics Democritus University of Thrace and University General District Hospital of Alexandroupolis 6th Kilometre AlexandroupolisMakris 68 100 Alexandroupolis, Thrace, Greece

Tel: +30 25510-74411

Fax: +30 25510-30340

E-mail: emantada@med.duth.gr

επιχρίσματος

80%

Silverstein

1. Pui CH, Relling MV, Downing JR. Acute lymphoblastic leukemia. N Engl J Med. 2004; 350: 1535-48

2. Smith OP, Hann IM. Clinical features and therapy of lymphoblastic leukemia in Pediatric Hematology (Third edition). Arceci RJ, Hann IM, Smith OP (eds). Blackwell Publishing, 2006, pages 450-81

3. Schaller J. Arthritis as a presenting manifestation of malignancy in children. J Pediatr. 1972; 81: 793-7

4. Bradlow A, Barton C. Arthritic presentation of childhood leukaemia. Postgrad Med J. 1991; 67: 562-4

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6. Andersson Gäre B, Fasth A, Andersson J, Berglund G, Ekström H, Eriksson M, Hammarén L, Holmquist L, Ronge E, Thilen A. Incidence and prevalence of juvenile chronic arthritis: a population survey. Ann Rheum Dis. 1987; 46: 277-81

7. Andersson Gäre B, Fasth A. Epidemiology of juvenile chronic arthritis in Southwestern Sweden: a 5-year prospective population study. Pediatrics 1992; 90: 950-8

8. Kaipiainen-Seppanen O, Savolainen A. Changes in the incidence of juvenile rheumatoid arthritis in Finland. Rheumatology (Oxford) 2001; 40:928-32

9. von Koskull St, Truckenbrodt H, Holle R, Hormann A. Incidence and prevalence of juvenile arthritis in an urban population of southern Germany: a prospective study. Ann Rheum Dis. 2001; 60: 940-5

10. Berntson L, Andersson Gäre B, Fasth A, Herlin T, Kristinsson J, Lahdenne P, Marhaug G, Nielsen S, Pelkonen P, Rygg M; Nordic Study Group. Incidence of juvenile idiopathic arthritis in the Nordic countries. A population based study with special reference to the

validity of the ILAR and EULAR criteria. J Rheumatol. 2003; 30: 2275-82

11. Pruunsild C, Uibo K, Liivamägi H, Tarraste S, Talvik T, Pelkonen P. Incidence of juvenile idiopathic arthritis in children in Estonia: a prospective population-based study. Scand J Rheumatol. 2007; 36: 7-13

12. Riise ØR, Handeland KS, Cvancarova M, Wathne KO, Nakstad B, Abrahamsen TG, Kirkhus E, Flatø B. Incidence and characteristics of arthritis in Norwegian children: a population-based study. Pediatrics. 2008; 121: e299-306

13. Ehrmann Feldman D, Bernatsky S, Houde M. The incidence of juvenile rheumatoid arthritis in Quebec: a population data-based study. Pediatr Rheumatol Online J. 2009 Nov 19; 7: 20

14. Smith MA, Gloeckler Ries LA, Gurney JG, Ross JA. Leukemia: SEER pediatric monograph. In: Ries LAG, Smith MA, Gurney JG, et al. (eds). Cancer incidence and survival among children and adolescents: United States SEER Program 1975-1995, National Cancer Institute, SEER Program. NIH Pub No. 99-4649. Bethesda, MD, 1999

15. Gallagher DJ, Phillips DJ, Heinrich SD. Orthopedic manifestations of acute pediatric leukemia. Orthop Clin North Am. 1996; 27: 635-44

16. Wallendal M, Stork L, Hollister JR. The discriminating value of serum lactate dehydrogenase levels in children with malignant neoplasms presenting as joint pain. Arch Pediatr Adolesc Med. 1996; 150: 70-3

17. Silverstein MN, Kelly PJ. Leukemia with osteoarticular symptoms and signs. Ann Intern Med. 1963; 59: 637-45

18. Chudwin DS, Ammann AJ, Cowan MJ, Wara DW. Significance of a positive antinuclear antibody test in a pediatric population. Am J Dis Child. 1983; 137: 1103-6

19. Jones OY, Spencer CH, Bowyer SL, Dent PB, Gottlieb BS, Rabinovich CE. A multicenter case-control study on predictive factors distinguishing childhood leukemia from juvenile rheumatoid arthritis. Pediatrics. 2006; 117: e840-4

20. Blatt J, Penchansky L, Horn M. Thrombocytosis as a presenting feature of acute lymphoblastic leukemia in childhood. Am J Hematol 1989; 31: 46-9

21. Alter BP, Weiner MA, Harris MB. Erythrocyte characteristics in childhood acute leukemia. Am J Pediatr Hematol Oncol. 1989; 11: 8-15

22. Dallman PR, Siimes MA. Percentile curves for hemoglobin and red cell volume in infancy and childhood. J Pediatr. 1979; 94: 26-31

23. Marwaha RK, Kulkarni KP, Bansal D, Trehan A. Acute lymphoblastic leukemia masquerading as juvenile rheumatoid arthritis: diagnostic pitfall and association with survival. Ann Hematol. 2010; 89: 249-54

24. Jonsson OG, Sartain P, Ducore JM, Buchanan GR. Bone pain as an initial symptom of childhood acute lymphoblastic leukemia: association with nearly normal hematologic indexes. J Pediatr. 1990; 117(2 Pt 1): 233-7

25. Ostrov BE, Goldsmith DP, Athreya BH. Differentiation of systemic juvenile rheumatoid arthritis from acute leukemia near the onset of disease. J Pediatr. 1993; 122: 595-8

26. Cabral DA, Tucker LB. Malignancies in children who initially present with rheumatic complaints. J Pediatr. 1999; 134: 53-7

27. Gaynon PS, Lustig RH. The use of glucocorticoids in acute lymphoblastic leukemia of childhood. Molecular, cellular, and clinical considerations. J Pediatr Hematol Oncol. 1995; 17: 1-12

28. Révész T, Kardos G, Kajtár P, Schuler D. The adverse effect of prolongedprednisolone pretreatment in children with acute lymphoblastic leukemia. Cancer. 1985; 55: 1637-40

Abstract

Haricleia Barbaresou1

Eleni Gaki1

Maria Kazantzi1

T. Xatzis1

Intensive Care Unit “Agia Sofia”

General Children’s Hospital, Athens, Greece

Keywords: infant, pulmonary hypertension, bosentan, sildenafil Xαρίκλεια

Haroula Tsipou2

Doctor

bosentan.

sildenafil

An infant with severe pulmonary arterial hypertension was treated successfully with sildenafil

and bosentan

Haricleia Barbaresou1, Eleni Gaki1, Haroula Tsipou2 , Maria Kazantzi1 , T. Xatzis1

Pulmonary hypertension is a devastating disease for children and adults. Attempting to change the course of this disease various new therapeutic agents have been introduced in the last decade. Dual endothelin receptor antagonist bosentan is one of them, as its efficacy has been shown in many studies carried out in adult patients. Lately, the same results are emerging from studies in children. Here we present the case of an infant with pulmonary hypertension due to congenital heart disease that was successfully treated with administration of sildenafil and bosentan.

Τηλ.: 6972260155

e-mail: haroula_tsipou@yahoo.com ctsipou@med.uoa.gr

Correspondence

Haroula Tsipou

14, Pavlou Mela St., 15343, Agia Paraskevi Tel.: +30 6972260155

e-mail: haroula_tsipou@yahoo.com ctsipou@med.uoa.gr

NYHA: New York Heart Association

ΜΕΝΝ:

NYHA: New York Heart Association

WHO: World Health Organization

QP/Qs: pulmonary blood flow\ systemic blood flow

Rp: resistance pulmonary

Φ.Τ.:

(World Health Organization/New York Heart Association

1.Humbert, M., Morrell NW, Archer SL, Stenmark KR, MacLean MR, Lang IM, et al., Cellular and molecular pathobiology of pulmonary arterial hypertension. J Am Coll Cardiol 2004;43(12 Suppl): S13-S24

2.Jeffery, T.K. and J.C. Wanstall, Pulmonary vascular remodeling: a target for therapeutic intervention in pulmonary hypertension. Pharmacol Ther 2001;92:1-20

3.Barst, R.J., McGoon M, Torbicki A, Sitbon O, Krowka MJ, Olschewski H, et al., Diagnosis and differential assessment of pulmonary arterial hypertension. J Am Coll Cardiol 2004; 43(12 Suppl):S40-S47

4.Channick, R.N., Simonneau G, Sitbon O, Robbins IM, Frost A, Tapson VF,et al., Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebo-controlled study. Lancet 2001;358(9288):1119-1123

5.Galie, N., Hoeper MM, Humbert M, Torbicki A, Vachiery JL, Barbera JA et al., Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J 2009;30(20):2493-2537

6.Haworth, S.G., The management of pulmonary hypertension in children. Arch Dis Child 2008;93(7):620-625

7.Maiya, S., Hislop AA, Flynn Y, Haworth SG., Response to bosentan in children with pulmonary hypertension. Heart 2006;92(5):664-670

8.Rosenzweig, E.B., Ivy DD, Widlitz A, Doran A, Claussen LR, Yung D, et al., Effects of long-term bosentan in children with pulmonary arterial hypertension. J Am Coll Cardiol 2005;46(4):697-704

9.Brancaccio, G., Toscano A, Bevilacqua M, Di Chiara L, Parisi F., Bosentan and sildenafil: should the combination therapy be a valid alternative in childhood to prostacyclin infusion? Pediatr Transplant 2007;11(1):110-112

10. van Loon, R.L., Hoendermis ES, Duffels MG, Vonk-Noordegraaf A, Mulder BJ, Hillege HL, et al., Long-term effect of bosentan in adults versus children with pulmonary arterial hypertension associated with systemic-to-pulmonary shunt: does the beneficial effect persist? Am Heart J 2007;154(4):776-782

11. Ivy, D.D., Doran A, Claussen L, Bingaman D, Yetman A., Weaning and discontinuation of epoprostenol in children with idiopathic pulmonary arterial hypertension receiving concomitant bosentan. Am J Cardiol 2004;93(7):943-946

12. Li, A.M., Yu CC, Tsang T, So HK, Wong E, Chan D et al., The six-minute walk test in healthy children: reliability and validity. Eur Respir J 2005;25(6):1057-1060

13. Garofano, R.P. and R.J. Barst, Exercise testing in children with primary pulmonary hypertension. Pediatr Cardiol 1999;20(1):61-64

1

2

2

1

Hypereosinophilic syndrome in a haemodialysis child

M. Mila

Abstract

M. Mila1

A. Mitsioni1

A. Zampetoglou1

K. Stefanidis1

Νephrology Department ”P.&A. Kyriakou” Children’s Hospital, Athens, Greece

M. Baka2

E. Kosmidi2

Oncology Department ”P.&A. Kyriakou”Children’s Hospital, Athens, Greece

Hypereosinophilic syndrome is a rare and heterogeneous group of disorders defined by blood eosinophilia for longer than six months, signs and symptoms of organ involvement, and lack of evidence for known causes of eosinophilia, such as allergic, parasitic or malignant disorders.

A 16-year-old boy with end stage renal insufficiency due to focal segmental glomerulosclerosis is described, who six months after the onset of haemodialysis presented with marked leucocytosis, eosinophilia, and thrombocytopenia, together with cough and fever at the end of each dialysis session. Extensive laboratory investigation including echocardiogram, abdominal ultrasound, immunological tests, serology and myelogram, has been negative. The chest plain radiograph and CT showed bilateral reticular opacities and bronchoalveolar lavage (BAL) specimen examination revealed the presence of more than 80% eosinophils, establishing the diagnosis of acute eosinophilic pneumonia. After a month of treatment with prednizolone dramatical improvement of the clinical, radiological and hematological abnormalities was noted. The patient remained stable on low-dose prednisolone and continued his haemodialysis sessions uneventfully.

1, M. Baka2, A. Mitsioni1, A. Zampetoglou1, E. Kosmidi2, K. Stefanidis1

e-mail: p.n@aglaiakyriakou.gr

Correspodence

Mila Maria

Νephrology Department ”P.&A.

Kyriakou” Children’s Hospital, Livadias 11527, Athens, Greece e-mail: p.n@aglaiakyriakou.gr

Conclusion: Although mild peripheral blood eosinophilia is common among haemodialysis patients, full-blown hypereosinophillic syndrome with organ involvement is rather unusual. Together with appropriate treatment, careful investigation and close follow-up are necessary in such cases, for definite exclusion of clonal haematological disease.

Key words: eosinophilia, dialysis, children

(IgG: 1435mg/dL, IgA: 224mg/dL, IgM: 92mg/dL, C3: 153mg/dL, C4: 47,9mg/dL)

(CD4+: 43%, CD8+: 35,2%,

Παράσιτα (έλμινθες, εχινόκοκκος) Πρωτόζωα (αμοιβάδα, τοξόπλασμα,) Βακτηρίδια (οστρακιά, φυματίωση, Borrelia burgdorferi, χλαμύδια) Μύκητες (κοκκιδιοϊδομύκωση)

Αντισπασμωδικά Αντιβιοτικά Αλλοπουρινόλη Αντι-ρευματικά

Άσθμα Αλλεργικές δερματίτιδες (έκζεμα) Δερματικά φλεγμονώδη νοσήματα (ερπητόμορφος δερματίτιδα, ερυθρά πιτυρίαση)

Ηωσινοφιλική περιτονεϊτιδα (eosinophillic fasciitis, συνδρ. Schulman)

Αντίδραση σε συμπληρώματα διατροφής (συνδρ. ηωσινοφιλίας-μυαλγίας) Αυτοάνοσα νοσήματα

Αγγειίτιδες

Ρευματοειδής αρθρίτιδα

Συστηματικός ερυθηματώδης λύκος Δερματομυοσίτιδα

Σκληροδερμία

Ηπατίτιδα χολοστατική

Φλεγμονώδη νοσήματα εντέρου

Μεταστατικές κακοήθειες

Λέμφωμα Hodgkin

Χρόνιες μυελοϋπερπλαστικές διαταραχές Συμπαγείς όγκοι

Σύνδρομο Wiskott-Aldrich

Υπερ-IgE

IgA

Νόσος Addison Υποϋποφυσισμός

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Ε. Papadimitriou

L. Giannopoulou

S. Kouskouvekakis

D. Haniotakis

F. Tsitsopoulos

Ι. Kavaliotis

Pediatric Department

Hospital of Infectious diseases

Thessaloniki

Acute otitis media with intracranial complications

Ε. Papadimitriou, L. Giannopoulou, S. Kouskouvekakis, D. Haniotakis, F. Tsitsopoulos, Ι. Kavaliotis

A 10 year old boy, was admitted to our department for mastoiditis and meningitis, following a 7 day history of acute otitis media. During his hospitalization the patient also developed subdural abscess as well as osteomyelitis of temboral bone. The aim of this case report is to remind the complications of acute otitis media if it is inadequately or improperly treated.

Key words: otitis media, mastoiditis, osteomyelitis

Τηλ.: 2313308719

E-mail: kavagr@hotmail.com

ΟΝΠ:

237/104mg/dl.

Κ/α

Gram

CT

Correspondence

J. Kavaliotis

Pediatric Department

Hospital of Infectious diseases 13 Gr. Lambraki St. 54638 Thessaloniki, Greece

Phone: +30 2313308719

E-mail: kavagr@hotmail.com

Εργαστηριακώς: Αριθμός

12670 κκχ (Π 89%), ΤΚΕ 75, CRP 5,89,

ONΠ: κύτταρα 450 κκχ, (Π 75%), Glu 46, λεύκωμα 93, γαλακτικό οξύ 22,15,

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15. Γκέλης

2008:1-44

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