Sclerosing cholangitis in children☆,☆☆,★
Section snippets
METHODS
Between 1972 and 1992, we investigated 56 children for cholestatic liver disease and found that they had SC, as shown on cholangiograms displaying typical bile duct abnormalities.12 The diagnosis of SC was established by operative cholangiography in 12 children, percutaneous transhepatic cholangiography or cholecystography in 40 children, and endoscopic retrograde cholangiography in 4. The clinical or radiologic features of some of these children have been previously reported in part.13, 14, 15
Clinical and biochemical findings
(Table I). There were 32 boys and 24 girls. The parents were related in 13 instances. Fifteen children had neonatal cholestatic jaundice starting within the first 2 weeks of life. Among the 41 with later onset, initial signs occurred at a mean age of 5 ± 4 years (range, 3 months to 14 years) and consisted of cholestatic jaundice in 9, hepatomegaly in 28, splenomegaly in 1, and gastric variceal hemorrhage in 1. Abnormalities were found on liver function tests during a routine examination in two
DISCUSSION
Sclerosing cholangitis is being recognized more frequently since the development of percutaneous transhepatic cholangiography and cholecystography. In 1987 a review of 78 pediatric cases showed that SC in children was most often associated with an underlying disease (75%),5 but only 55% of the children reported on here had an associated disease and IBD was relatively uncommon. This difference may reflect referral patterns. Children who had SC without an apparent associated extrabiliary disease
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2018, Journal of AutoimmunityJaundice
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2018, Practical Hepatic Pathology: A Diagnostic Approach: Second Edition
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From the Service d' Hépatologie Pédiatrique, Service de Radiologie Pédiatrique, and Unité de Recherche INSERM U 347, Hôpital de Bicêtre, 94275 Le Kremlin-Bicêtre, France
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Reprint requests: Dominique Debray, MD, Service d'Hépatologie Pédiatrique, Hôpital de Bicêtre, 78 rue du Général Leclerc, 94275 Bicêtre, France
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