Autoimmunologiczne zapalenie wÄ troby u trzech par rodzeństwa

Three pairs of siblings (9-14 years of age) were found in cohort of 198 children with autoimmune hepatitis (AIH) treated in our institution. Jaundice was the first symptom of the disease in one child, the remaining did not present any characteristic symptoms and the diagnosis was based on typical laboratory and patomorphological abnormalities. At diagnosis all children presented with moderate or severe liver fibrosis. All children received standard prednisone and azathioprine therapy. The treatment was successfully discontinued in one child, four subjects continue the therapy and one child died due to liver function decompensation. Our data shows that the presentation, clinical course and treatment outcome of AIH in siblings are the same as in isolated cases of the disease. The presence of AIH in siblings justifies the implementation of screening liver function tests in the families of AIH patients.