설사와 관련된 성인 용혈성 요독 증후군 환자의 임상상과 혈장교환술의 치료 경과

Diarrea-associated hemolytic uremic syndrome (HUS) is very rare in adults. Few reports are available on clinical features and plasma exchange in adult patients with diarrhea-associated HUS in Korea. We retrospectively examined the records of five adult patients with diarrhea-associated HUS admitted to Samsung Seoul Hospital between January 1995 and December 1997. If the patient had neurologic abnormalities, or there was rapid clinical deterioration, with the hematocrit decreasing below 20%, the platelet count falling below 10,000/mm3, the creatinine concentration increasing above 5.0mg/dl, plasma exchange was begun. There were 4 females and 1 male. Patients ranged in age from 16 to 61 years. All patients presented with diarrhea and abdominal pain, and 3 patients had bloody diarrhea. The mean time between the onset of diarrhea and thrombocytopenia was 4.4±1.9 days(range, 1 to 6). All patients received 7 to 24 plasma exchanges. The mean exchanged plasma volume was 1.1±0.2 times of patients own plasma volume. The pattern of clinical response to plasma exchange was initial normalization of platelet count (8.0±3.8 days), followed by normalization of LDH level(20.2±14.5 days) and creatinine concentration (25.8±13.8 days). Metabolic alkalosis developed in two patients undergoing daily plasma exchange. We successfully managed the metabolic alkalosis with continuous venovenous hemofiltration. The mean duration of hospitalization was 28.8±11.2days(range, 20 to 42). All patients successfully recovered without any sequale. Although this study is based on small case series, we suggested that plasma exchange may improve the outcome in adult diarrhea-associated HUS.