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.
展开▼
