Childhood sclerosing cholangitis associations in a Tunisian tertiary care hospital: a many-faceted disease

Özet

Sclerosing cholangitis (SC) is a liver disorder affecting children and adults,causing chronic cholestasis and secondary biliary cirrhosis. The purpose ofthis study was to present different associated diseases to SC in a Tunisiantertiary care hospital.Six patients were identified with SC associated with other diseases, fourmales and two females. The first symptom was liver enlargement in all caseswith abnormal liver biochemistry. A moderate increase in AST and ALT levelswas registered in all cases with moderate cholestasis in 4 patients. Three ofthem presented an auto-immune condition. Two patients were diagnosed withauto-immune hepatitis prior to SC and Crohn disease in only one patient.One developed linear IgA bullous dermatosis. Three patients were diagnosedwith Multisystemic Langerhans Cell Histiocytosis (LCH). The primary siteof LCH was the liver associated secondary to insipidus diabetes (one case),mastoiditis (two cases) and chest localization (one case). The outcome ofthose patients was variable with poor prognosis especially for SC secondaryto LCH. No patient underwent liver transplantation.SC is a rare disorder with variable clinical presentations. To our knowledge,this is the first report of this condition in Tunisian and North Africanchildren. Diagnosis and treatment of SC and its associations remains achallenge, especially because there is still no effective medical therapy aimedat preventing disease progression. Pediatric liver transplantation is the onlylife-extending therapeutic alternative for patients with end-stage liver failure.Liver transplantation has not been performed on young children in our country.