Clinical Evaluation of Syndromic and Nonsyndromic Intrahepatic Bile Duct Paucity / 대한소아소화기영양학회지
Korean Journal of Pediatric Gastroenterology and Nutrition
;
: 178-184, 1999.
Article
in Korean
| WPRIM
| ID: wpr-8055
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Prognosis
/
Pulmonary Valve Stenosis
/
Bile Ducts
/
Bile Ducts, Intrahepatic
/
Biliary Atresia
/
Fibrosis
/
Cholestasis
/
Retrospective Studies
/
Liver Transplantation
/
Alagille Syndrome
Type of study:
Diagnostic study
/
Observational study
/
Prognostic study
Limits:
Humans
/
Infant
Language:
Korean
Journal:
Korean Journal of Pediatric Gastroenterology and Nutrition
Year:
1999
Type:
Article
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