[Thrombotic thrombocytopenic purpura in children]

An analysis of 4 cases of the thrombotic thrombocytopenia in children of 4 to 10 years of age is performed. The disease was characterized by fever, purpura, headache and abdominal pains, arterial hypertension, microangiopathic haemolytic anemia, thrombocytopenia, increase of blood urea and serum creatinine, micro-haematuria and proteinuria. The duration of the disease was from 4 days to 7 months. Anuria, gangrene of the ears, scrotum, penis and soft tissues of legs and feet were registered in a 5-year-old patient with a fulminant disease. The cause of death of other patients was heart failure with acute lung oedema, brain haemorrhages and haemorrhagic pancreonecrosis. The diagnosis of the thrombotic thrombocytopenia was confirmed by the finding in the autopsy material of thrombotic microangiopathy of small arteries, veins, arterioles, venules and capillaries in kidneys and other organs and tissues. Kidney damage in fulminant disease is complicated by segmentary cortical necrosis, in a more prolonged disease--by glomerulosclerosis or mesangio-capillary glomerulonephritis.