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November 1970

The Hemolytic Uremic Syndrome: Renal Cortical Thrombotic Microangiopathy

Author Affiliations

Los Angeles

From the Childrens Hospital of Los Angeles, and the Department of Pediatrics, University of Southern California School of Medicine.

Arch Intern Med. 1970;126(5):816-822. doi:10.1001/archinte.1970.00310110086012

The hemolytic uremic syndrome is an uncommon but dramatic illness occurring in infants. After a mild prodromal illness, an acute stage develops characterized by hemolytic anemia, bleeding, renal failure, uremia, hypertension, heart failure, and death in up to 40% of the patients. Survivors may have chronic or progressive renal disease in later years.

The characteristic pathologic findings are fibrin microthrombi in glomerular capillaries and arterioles, swelling and fragmentation of glomerular basement membrane, and renal cortical necrosis.

The probable cause is intravascular coagulation leadingto deposition of fibrin in the renal microcirculation.

Hemolysis and persistent thrombocytopenia probably result from cell injury by circulation through the altered glomerular vessels. Treatment includes transfusions, management of oliguria, and dialysis when indicated. The use of heparin sodium to prevent continued intravascular clotting and further glomerular fibrin deposition is undergoing trial.

The hemolytic uremic syndrome (HUS) is an uncommon but dramatic illness usually occurring in young infants,

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