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RESEARCH PRODUCT

Endotheliotropic (hemolytic) nephroangiopathy and its various manifestation forms (thrombotic microangiopathy, primary malignant nephrosclerosis, hemolytic-uremic syndrome).

subject

description

In hemolytic-uremic syndrome (HUS) various lesion types in the kidneys are observed in childhood. These are designated as the “glomerular type=G type” (essentially identical with thrombotic microangiopathy), as “arterial type=A type” (essentially identical with primary malignant nephrosclerosis (PMN)) or as “mixed type=G/A type”. With reference to characteristic cases, the most important morphological findings are described. In adults, in whom other diagnostic terms are used besides HUS, the same nephropathological types are observed. In the two phases of life, there occurs in addition renal cortical necrosis in subtotal nephroangiothrombosis. The G type and A type also have a different clinical valence. An acute renal failure with hemolytic anemia and mostly slight elevation of blood pressure (HUS in the narrower sense) corresponds predominantly to the G type, and a malignant hypertension with renal insufficiency of delayed onset and lower tendency to hemolysis (primary malignant nephrosclerosis) corresponds predominantly to the A type. Mixed forms also occur clinically. In early childhood (up to 5 years old), the G type predominates; in late childhood and in adulthood, the A type predominates. This is a disease complex with various forms of clinical and pathomorphological manifestation, but with a common denominator of simultaneous erythrocyte damage (fragmentocytosis, hemolysis) and endothelial damage, the latter preferentially in the kidneys. For this reason, “endotheliotropic (hemolytic) nephroangiopathy” with various subforms is suggested as collective term for the kidney damage.