A 23-year old male patient had eight distinct episodes of hemolytic uremic syndrome (HUS) between 8.5 and 15 years of age, five of them accompanied by hypocomplementemia. In the further course, severe hypertension, renal insufficiency as well as protein-losing enteropathy due to intestinal lymphangiectasia developed, whilst hypocomplementemia persisted. The association of recurrent HUS with hypocomplementemia and intestinal lymphangiectasia may represent a new association within a subgroup of the atypical HUS.
Autoimmune hepatitis type 1 in a 8-year old girl is described. The diagnosis was established using International Autoimmune hepatitis group scoring system. In addition to characteristic histologic features of autoimmune hepatitis (periportal hepatitis, piecemeal necrosis and rozettes) prominent centrilobular necrosis was discovered. As an isolate finding in autoimmune hepatitis, this type was described only in five cases. In our unique case centrilobular necrosis is a very important parallel finding previously not detected in autoimmune hepatitis. Some experimental studies suggest that cytocins present in inflammatory cell infiltrate in the liver play a pathologic role in autoimmune liver cell damage.
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