PMID: 2503928Jan 1, 1989Paper

Fatal familial cholestatic syndrome in Greenland Eskimo children. A histomorphological analysis of 16 cases

Virchows Archiv. A, Pathological Anatomy and Histopathology
K OrnvoldH Poulsen

Abstract

We report the first detailed study of hepatic morphology in 28 biopsies from 16 Greenland Eskimo children with fatal familial cholestatic syndrome. The changes were categorized as early, intermediate and late. In the early stage, until 5 months of age, changes were restricted to zone 3, consisting of cholestasis and rosette formation without fibrosis. In the intermediate stage, from 5 to 14 months, cholestasis persisted and rosette formation increased, both with further extension into zone 2. Perisinusoidal fibrosis developed, first in zone 3 and later in zone 1. The late stage, from 17 to 60 months, showed a further increase in cholestasis and rosette formation, and fibrosis of zones 3 and 1 in nearly all biopsies. Portal to portal and portal to central fibrosis was evident with resulting cirrhosis in 2 of 7 patients. The morphological features can be summarized as pure cholestasis with prominent rosette formation followed by zone 3 fibrosis, zone 1 fibrosis, and, cirrhosis. Other characteristics are the virtual absence of inflammation and the lack of anatomical abnormalities such as paucity of bile ducts. The changes and their progression resemble those of Byler disease. Clinical and biochemical features are also largely simi...Continue Reading

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Citations

Sep 12, 2000·Current Gastroenterology Reports·K M Emerick, P F Whitington
Feb 1, 1996·Pediatric Clinics of North America·P F Whitington
Apr 18, 2002·Biochimica Et Biophysica Acta·Ronald Oude Elferink, Albert K Groen
Sep 16, 2014·PloS One·Md Abu ChoudhuryJohn Aaskov
Jul 1, 2016·World Journal of Transplantation·Ashley MehlTrevor W Reichman
Nov 28, 2000·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·L W KlompR H Houwen
Jan 1, 1996·Journal of Clinical Ultrasound : JCU·N R PatelE Thomas
Sep 24, 2004·Pediatric and Developmental Pathology : the Official Journal of the Society for Pediatric Pathology and the Paediatric Pathology Society·A S Knisely

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