THE CLINICOPATHOLOGICAL CHARACTERISTICS AND OUTCOME IN HEMOLYTIC-UREMIC SYNDROME OF ADULTS

Twenty-eight adults who developed hemolytic-uremic syndrome (HUS) unde rwent a renal biopsy during the period from 1973 to 1993, and the spec imens were analyzed both clinically and pathologically. The observed e tiologic antecedents of HUS were infectious disease in 7 cases, medica tion, mainly due to cytotoxic drugs in 7 cases, post-partum in 7 cases , progressive systematic scleroderma in 1 case and unknown in 6 cases. Histologically, 17 cases were glomerular type, 3 cases were arterial type, 6 cases were mixed type and 2 cases demonstrated cortical necros is. The preceding conditions and the histological findings appeared no t to be related to each other. In the acute phase of the disease, of 1 1 patients who fell into severe acute renal failure (AW) with an incre ase in the serum creatinine level of more than 5 mg/dl, 8 patients nee ded dialysis therapy while 3 patients did not recover from the disease (3 died and 1 was introduced to maintenance dialysis therapy). The in cidence of thrombus in the glomerular capillaries and fibrinoid necros is of the arterioles, the index of the expansion of glomerular subendo thelial lucent zone (Glom-SE-ex), the index of glomerular sclerosing a nd the index of the intimal thickening of the arterioles were higher i n the severe ARF than in the mild ARF. After analyzing the prognostic factors on renal survival, severe ARF, the presence of fibrinoid necro sis, severe glomerular endothelial damage and severe intimal thickenin g of the arterioles appeared to have a significantly adverse effect. M oreover, high Glom-SE-ex and the presence of fibrinoid necrosis also i ndependently influenced renal survival. We thus conclude that the etio logy of HUS in adults demonstrates a greater variation than that in ch ildren. Therefore, it is very difficult to predict the outcome of this disease based on the clinical findings alone, and thus a renal histol ogical examination is considered to help in determining the long-term outcome.