Epidemiological, etiological and evolutionary aspects of children cirrhosis in a developing country: experience of the pediatric department of SFAX University hospital, Tunisia

BACKGROUND: Cirrhosis is rare in pediatrics. The children cirrhosis is particular by the ascendancy of biliairy cirrhosis and cirrhosis due to an innate error of metabolism and by the relative frequency of the cases where an etiological treatment is possible. However in developing countries, the children cirrhosis still put problems of etiological diagnosis and of therapeutic coverage. AIM: To study epidemiological and etiological particularities, therapeutic modalities and outcome of cirrhosis in the south of Tunisia. METHODS: We led a retrospective study over 15 years (1990-2004) having allowed to depict 71 cirrhotic children followed in the service of general pediatric department of SFAX University hospital (Tunisia). RESULTS: Our patients divide up into 36 girls and 35 boys. The age of revelation of the disease was variable (15 days to 15 years). Jaundice and hepatomegaly were the most two clinical signs frequently found in the clinical exam. On the etiological plan, biliairy cirrhosis (Extra hepatic biliary atresia, dilatation of choledocal cyst, progressive familial intrahepatic cholestasis...) were the most frequent (40%) followed by metabolic cirrhosis(tyrosinemia type I, Wilson disease...) (17 %) and post-hepatitic cirrhosis (17%). In 27% of cases, no etiology was found. Besides the symptomatic treatment, an etiological treatment was tempted in some cases. No patient benefited from hepatic transplantation. The follow-up of the children cirrhosis was grave because 30 children (42%) died, 19 children are lost-sight and 22 children (31%) are still alive. CONCLUSION: The children cirrhosis pose still in our country of the problem of etiological diagnosis because of the not availability of some specific additional exams and especially problems of coverage for lack of a program of hepatic transplantation.