Abstract

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. To describe the pattern of chronic liver diseases at The Royal Hospital, Muscat during the period January 2005 to January 2007. 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. 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. 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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