[Epidemiological, etiological and evolutionary aspects of children cirrhosis in a develping country: Experience of the pediatric department of SFAX University hospital, Tunisia]

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. To study epidemiological and etiological particularities, therapeutic modalities and outcome of cirrhosis in the sooth of Tunisia. 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]. 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-op of the children cirrhosis was grave becaose 30 children [42%] died, 19 children are lost-sight and 22 children [31%] are still alive. 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

mucoviscidose chez l'enfant: Experience d'un service Tunisien de pediatrie generale

Cystic fibrosis was regarded a long time as exceptional in the Nord Africaine population and in particular in Tunisia what was at the origin of the ignorance of its various diagnostic and 'therapeutic aspects in our country. Nevertheless, with the development of the means of the diagnosis, several cases of cystic fibrosis were diagnossd these last years what will pose true problems of assumption of responsibility of these children like illustrates it well our experiment in the pediatric department of Sfax university hospital. In 10 years going of 1991 to 2000 we reported 7 cases of cystic fibrosis in the peditric department of Sfax university hospital. Our patients are 3 boys and 4 girls. The age of revelation of the disease varied from 3 months to 14 years with 4 years and 10 months an average age. All our patient had a respiratory symptomatology in the Foreground. The evolution was marked by the death of 4 patients at an average age of 5 years and half whereas the 3 surviving patients for the moment are more or less balanced on the nutritional level, digestive and respiratory. Cystic fibrosis is not exceptional in our area, its assumption of responsibility therapeutic is difficult, and it requires the collaboration of several experts and a good compliance of the child and of his family