PMID: 3758943Sep 1, 1986Paper

Nonsyndromatic paucity of interlobular bile ducts: light and electron microscopic evaluation of sequential liver biopsies in early childhood

Hepatology : Official Journal of the American Association for the Study of Liver Diseases
E I KahnH Aiges

Abstract

Liver biopsies and/or autopsy specimens from 17 children (ages 1 week to 5 years) with nonsyndromatic paucity of the interlobular bile ducts were studied by light and electron microscopy. Initial biopsies were obtained before 90 days of age from all patients, and two or more specimens were available from nine. No specific underlying condition was found in nine infants. The remaining cases were associated with Down's syndrome (n = 2), hypopituitarism (n = 2), cystic fibrosis (n = 1), alpha 1-antitrypsin deficiency (n = 1), cytomegalovirus (n = 1) and Ivemark syndrome (n = 1). Before 90 days of age, portal changes included duct paucity and fibrosis. Lobular changes were nonspecific, consisting of cholestasis, giant cell transformation, extramedullary hematopoiesis and perisinusoidal fibrosis. Duct paucity, portal fibrosis and perisinusoidal fibrosis persisted after 90 days. Cholestasis was mild or no longer apparent. Portal changes before 90 days of age appear to be sufficiently distinctive to microscopically distinguish nonsyndromatic from syndromatic paucity. Electron microscopic findings suggest that paucity in nonsyndromatic patients may result from a primary ductal insult: ultrastructural studies revealed bile duct destructi...Continue Reading

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Citations

Jul 21, 1998·The Anatomical Record·A M CostaL C Pôrto
Jan 1, 1995·Digestive Diseases and Sciences·S BerezinS M Schwarz
Mar 29, 2002·Clinics in Perinatology·Saul J Karpen
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Aug 2, 2002·Journal of Pediatric Gastroenterology and Nutrition·William F Balistreri
Oct 29, 2003·Journal of Pediatric Gastroenterology and Nutrition·Vered Yehezkely-SchildkrautRaanan Shamir
Jul 1, 1996·Archives of Disease in Childhood·P LykavierisM Hadchouel
Jul 1, 1989·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·E KahnF Daum
Feb 1, 1996·Pediatric Clinics of North America·C F McEvoy, F J Suchy
Feb 21, 2013·Fetal and Pediatric Pathology·Zelal KahramanerEsra Ozer
Apr 1, 1996·Journal of Gastroenterology and Hepatology·I NakanoY Nakanuma
May 1, 1996·Journal of Gastroenterology and Hepatology·H H ChiuY H Ni
May 1, 1992·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·M BrugueraJ Rodés
Sep 18, 2018·International Journal of Surgical Pathology·Mukul VijMohamed Rela
Jun 6, 2018·European Journal of Pediatrics·Babu Lal MeenaSeema Alam
Aug 23, 2019·Fetal and Pediatric Pathology·Suravi MohantyMarjorie Maria Anne Correa

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