Journal
ANNALS OF TROPICAL PAEDIATRICS
Volume 29, Issue 3, Pages 183-189Publisher
MANEY PUBLISHING
DOI: 10.1179/027249309X12467994693770
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Arab Gulf countries including Oman have a high rate of consanguineous marriage with an associated increased frequency of a variety of genetic disorders including liver diseases. Aim: To describe the pattern of chronic liver diseases at The Royal Hospital, Muscat during the period January 2005 to January 2007. Methods: A retrospective study of all children with chronic liver diseases seen at the Royal Hospital, which is a tertiary referral centre for gastro-enterology and hepatology for the whole of Oman. Results: Seventy-nine patients with chronic liver disease were identified. The consanguinity rate amongst parents was 78%. The mean (SD) age of patients at the time of final diagnosis was 5 years (4). The two most common liver disorders were progressive familial intrahepatic cholestasis (30%) and fibrocystic disease of liver and kidney (21%). One-third of the patients came from the Sharqiya region. Complications included growth retardation (75%), cirrhosis (26%) and portal hypertension (39%). Three children died and three others received living related liver transplant. Conclusion: About half of the chronic liver disorders in Oman are hereditary in origin. At least three children per year will require liver transplant.
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