Biliary atresia in Turkish children

“…11,22,23 In a study from Turkey which evaluated 27 patients with BA, delay of the diagnosis resulted in death about half of the cases in the postoperative period, and 30% of the cases underwent liver transplantation. 24 However the Netherlands Study Group on Biliary Atresia reported performance of surgery before two months of age and clearance of jaundice associated independently with 4 year transplantfree survival. 25 In developed countries short term clearance of jaundice can be achieved with Kasai portoenterostomy in approximately 50% to 60% of the children, and one third of the patients can survive with native liver up to the age of 10 years.…”

Section: Discussionmentioning

confidence: 99%

Evaluation of Infants with Neonatal Cholestasis: Experience of a Tertiary Referral Center in Turkey

Gökçebay¹,

Gülerman²,

Arda³

et al. 2015

Turkiye Klinikleri J Med Sci

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ABS TRACT Objective: Neonatal cholestasis can be the initial evidence of a heterogeneous group of diseases of different etiologies. The aim of the study is to evaluate the patients with neonatal cholestasis and analyse the etiologic factors, diagnostic tools and outcome. Material and Methods: Seventy-five patients (65% males, 45% females) between 0-6 months of age with neonatal cholestasis were retrospectively evaluated; analysed for the clinical, laboratory, radiological, scintigraphic data and histopathological findings from liver biopsies. Results: Eighty per cent of the infants' admitted to our hospital because of prolonged jaundice. The onset of the jaundice was in the first week of life in 64% of the cases, but admission time of the referral center was median two months. Biliary atresia (BA) was determined in 21 (28%), neonatal hepatitis (NH) in 54 (72%) of the patients. Biliary atresia group had significantly more frequent acholic stool, relatively lower aspartate aminotransferase, but higher gamma-glutamyl transpeptidase, and serum protein levels. Liver biopsy and hepatobiliary scintigraphy were the most sensitive methods for differentiation BA from NH (p<0.05). During the follow-up period, 13 of the patients (18%) died, whereas cholestasis improved in 14 of the patients with NH (27%) within median 6 months. Survival rate at one year was 45.5% for the patients with BA, and 87% for the patients with NH. So patients with NH had better prognosis (p<0.05). Conclusion: It has been evident that early diagnosis and intervention of treatable causes of neonatal cholestasis have a vital importance.Key Words: Jaundice, neonatal; cholestasis; biliary atresia ÖZET Amaç: Neonatal kolestaz farklı nedenlerle oluşan heterojen bir grup hastalığın ilk bulgusu olabilir. Bu çalışmanın amacı neonatal kolestazlı hastaların değerlendirilmesi ve etyolojik faktör-ler, tanı yöntemleri ve sonuçlarının incelenmesidir. Gereç ve Yöntemler: Neonatal kolestazı olan, yaşları 0-6 ay arasında değişen 75 hasta (%65'i erkek, %45'i kız) geriye dönük olarak değerlen-dirildi; klinik, laboratuvar, radyolojik, sintigrafik verileri ve karaciğer biyopsilerindeki histopatolojik bulguları incelendi. Bulgular: Bebeklerin %80'i uzamış sarılık nedeniyle hastanemize başvurmuştu. Olguların %64'ünde sarılık yaşamın ilk haftasında başlamasına rağmen ortanca başvuru zamanı iki aydı. Hastaların 21'inde (%28) bilier atrezi (BA), 54'ünde (%72) neonatal hepatit (NH) saptandı. Bilier atrezi grubunda akolik dışkı daha sık, aspartat aminotransferaz görece düşük, ancak gama-glutamil transpeptidaz ve serum protein düzeyleri yüksek saptandı. Karaciğer biyopsisi ve hepatobilier sintigrafi BA'nın NH'den ayrımında en duyarlı yöntem-lerdi (p<0,05). İzlem sırasında 13 hasta (%18) kaybedildi, ancak NH'li 14 hastada (%27) kolestaz ortanca 6 ay içinde düzeldi. Bilier atrezili hastaların bir yıllık yaşam oranı %45,5 iken, NH tanılı hastaların %87 idi. Neonatal hepatitli hastaların prognozları daha iyiydi (p<0,05). Sonuç: Erken tanı ile neonatal kolestazın tedavi edilebilir ned...

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Biliary atresia in Turkish children

Cited by 2 publications

References 13 publications

Polymorphisms of the ICAM-1 Gene Are Associated with Biliary Atresia

Polymorphisms of the ICAM-1 Gene Are Associated with Biliary Atresia

Evaluation of Infants with Neonatal Cholestasis: Experience of a Tertiary Referral Center in Turkey

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