Clinical Evaluation of Syndromic and Nonsyndromic Intrahepatic Bile Duct Paucity.
- Author:
Soo Jin HAN
1
;
Bo Hwa CHOI
;
Kyung Hoon KANG
;
Kyung Mo KIM
Author Information
1. Department of Pediatrics, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
- Publication Type:Original Article
- Keywords:
Alagille syndrome;
Nonsyndromic intrahepatic bile duct paucity;
Neonatal cholestasis
- MeSH:
Alagille Syndrome;
Bile Ducts;
Bile Ducts, Intrahepatic*;
Biliary Atresia;
Cholestasis;
Diagnosis, Differential;
Fibrosis;
Hepatitis;
Humans;
Infant;
Liver Transplantation;
Prognosis;
Pulmonary Valve Stenosis;
Retrospective Studies
- From:Korean Journal of Pediatric Gastroenterology and Nutrition
1999;2(2):178-184
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
PURPOSE: The aims of this study were to evaluate the clinical manifestations and prognosis of the syndromic and nonsyndromic intrahepatic bile duct paucity (IHBDP). METHODS: We studied histology of 42 infants with neonatal cholestasis. Fourteen patients were diagnosed as IHBDP. We evaluated the clinical manifestations, courses and prognosis retrospectively. RESULTS: Underlying disease of the 42 infants with neonatal cholestasis were biliary atresia in 23, intrahepatic bile duct paucity in 14 (Alagille syndrome in 4 and nonsyndromic IHBDP in 10), neonatal hepatitis in 5 infants. The mean ratio of the bile ducts per portal tract was 0.087 (range: 0~0.5). The manifestations in 4 patients with Alagille syndrome demonstrated as follows: characteristic face in 3, chronic cholestasis in 4, posterior embryotoxon in 2, vertebral anomalies in 2, peripheral pulmonary stenosis in 2. One of 4 patients of Alagille syndrome improved cholestasis and the other 3 patients were remained their cholestasis and growth retardation. All patients of the nonsyndromic IHBDP were idiopathic. Seven out of 8 patients of nonsyndromic IHBDP showed improvement of cholestasis, and one patient received liver transplantation due to cirrhosis. CONCLUSION: This study suggested that IHBDP should be considered in the differential diagnosis of neonatal cholestasis. The outcome of idiopathic IHBDP was better than predicted.
