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KMID : 0816119990020020178
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Korean Journal of Pediatric Gastroenterolology and Nutrition
1999 Volume.2 No. 2 p.178 ~ p.184
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Clinical Evaluation of Syndromic and Nonsyndromic Intrahepatic Bile Duct Paucity
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Han Su-Jin
Choi Bo-Hwa
Kim Kyung-Mo
Kang Kyung-Hoon
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Abstract
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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.
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KEYWORD
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Alagille syndrome, Nonsyndromic intrahepatic bile duct paucity, Neonatal cholestasis,
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