Maria PALABOUGİOUKİ, Nikolaos GOMBAKİS, Ioannis KYRİAKİDİS, Maria STAMOU, Timoleon VYZANTİADİS, Tatiani MOUDİOU, Eleni VASİLEİOU, Maria HATZİSTİLİANOU, Athanasios TRAGİANNİDİS

Hemoglobinopatisi olan bir bebekte biliyer atrezi komplikasyonu olarak kandidemi

Hem biliyer atrezi, hem de hemoglobinopatilerde, kan dolaşımı enfeksiyonu sıklığı daha fazladır. Burada, ekstrahepatik biliyer atrezisi ve “sickle-cell” hastalığı ve alfa-talasemi için çift heterozigot olan Nijerya kökenli bir kız bebeği sunuyoruz. Çocuğa yaşamının altıncı haftasında Kasai hepatoportoenterostomi uygulandı. Halen profilaktik antibiyotik tedavisi almakta olmasına rağmen, hepatoportoenterostomiden iki ay sonra kan dolaşımı enfeksiyonları ortaya çıkmıştır: birincisi kandida albikansa bağlı iken, bunu Escherichia coli’e bağlı bakteriyemi izlemiştir. Sadece cilt ile sınırlı kalan üçüncü bir enfeksiyon Acinetobacter spp’e bağlı olarak gelişmiştir. Bu yazıda biliyer atrezi ve anormal hemoglobini olan hastalarda, tedavi seçenekleri, predispozan faktörler ve kan dolaşımı enfeksiyonlarının patofizyolojisi tartışılmıştır.

Candidemia complicating biliary atresia in an infant with hemoglobinopathy

Both biliary atresia and hemoglobinopathies have been associated with a higher incidence of bloodstream infections. We hereby present the case of a female infant of Nigerian descent with extrahepatic biliary atresia and double heterozygocity for sickle cell disease and alpha-thalassemia. Kasai hepatoportoenterostomy was performed in the child’s sixth week of life. Bloodstream infections occurred two months post-hepatoportoenterostomy, even though the infant was still in prophylactic antibiotic treatment: the first was due to Candida albicans and was followed by bacteremia due to Escherichia coli. A third infection, confined to the skin only, was due to Acinetobacter spp. Treatment options, predisposing factors, and the pathophysiology of bloodstream infections in patients with biliary atresia and aberrant hemoglobin are discussed herein.

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1. Jiang CB, Lee HC, Yeung CY, Sheu JC, Chang PY, Wang NL, et al. A scoring system to predict the need for liver transplantation for biliary atresia after Kasai portoenterostomy. Eur J Pediatr 2003;162: 603−6. [CrossRef]
2. de Marie S. Diseases and drug-related interventions affecting host defence. Eur J Clin Microbiol Infect Dis 1993;12 Suppl 1: S36−41. [CrossRef]
3. Bezerra JA, Spino C, Magee JC, Shneider BL, Rosenthal P, Wang KS, et al. Use of corticosteroids after hepatoportoenterostomy for bile drainage in infants with biliary atresia: the START randomized clinical trial. JAMA 2014; 311: 1750−9.
4. Wildhaber BE, Majno P, Mayr J, Zachariou Z, Hohlfeld J, Schwoebel M, et al. Biliary atresia: Swiss national study, 1994-2004. J Pediatr Gastroenterol Nutr 2008;46: 299−307.
5. de Vries W, de Langen ZJ, Aronson DC, Hulscher JB, Peeters PM, Jansen-Kalma P, et al. Mortality of biliary atresia in children not undergoing liver transplantation in the Netherlands. Pediatr Transplant 2011;15: 176−83.
6. Anyaegbu CC, Okpala IE, Akren’Ova YA, Salimonu LS. Peripheral blood neutrophil count and candidacidal activity correlate with the clinical severity of sickle cell anaemia (SCA). Eur J Haematol 1998; 60: 267−8. [CrossRef]
7. Baskin MN, Goh XL, Heeney MM, Harper MB. Bac teremia risk and outpatient management of febrile patients with sickle cell disease. Pediatrics 2013; 131: 1035−41.
8. Savlov D, Beck CE, DeGroot J, Odame I, Friedman JN. Predictors of bacteremia among children with sickle cell disease presenting with fever. J Pediatr Hematol Oncol 2014; 36: 384−8. [CrossRef]
9. Ramakrishnan M, Moïsi JC, Klugman KP, Iglesias JM, Grant LR, Mpoudi-Etame M, et al. Increased risk of invasive bacterial infections in African people with sickle-cell disease: a systematic review and meta-analysis. Lancet Infect Dis 2010;10: 329−37. [CrossRef]
10. Wang JL, Chang CH, Young-Xu Y, Chan KA. Systematic review and meta-analysis of the tolerability and hepatotoxicity of antifungals in empirical and definitive therapy for invasive fungal infection. Antimicrob Agents Chemother 2010; 54: 2409−19. [CrossRef]