Congenital Hepatic Fibrosis with Polycystic Kidney Disease: An Unusual Cause of Neonatal Cholestasis

被引：3

作者：

Bharani, Vani ^{[1
]}

Venkatesh, G. Vybhav ^{[2
]}

Saikia, Uma Nahar ^{[3
]}

Thapa, B. R. ^{[4
]}

机构：

[1] PGIMER, Dept Pathol, Chandigarh, Punjab, India

[2] PGIMER, Dept Pediat, Chandigarh, Punjab, India

[3] PGIMER, Dept Histopathol, Chandigarh, Punjab, India

[4] PGIMER, Dept Gastroenterol, Chandigarh, Punjab, India

来源：

INDIAN PEDIATRICS | 2017年 / 54卷 / 07期

关键词：

Autopsy; Cholestatic Jaundice; Neonate; BILIARY ATRESIA;

D O I：

10.1007/s13312-017-1074-6

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

Congenital hepatic fibrosis is characterized by hepatic fibrosis, portal hypertension, and renal cystic disease. Typical presentation of congenital hepatic fibrosis is in the form of portal hypertension, in adolescents and young adults. We present an unusual case of neonatal cholestasis with rapid deterioration within first 4 months of life, who was diagnosed to have congenital hepatic fibrosis with polycystic kidney disease on autopsy.

引用

页码：589 / 592

页数：4

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