Abstract:

Objective  To analyze the etiologic  and clinical characteristics of 26 patients with atypical hemolytic-uremic syndrome (aHUS) in order to improve the prognosis. Methods  Twenty-six patients with aHUS in our hospital from January 2003 to December 2009 were enrolled in this study. Etiology, clinical features and therapeutic response were retrospectively analyzed.  Results  Among the 26 patients, there were 18 patients with idiopathic HUS, 1 with familial HUS and the other 7 with secondary HUS (4  pregnancy-associated HUS, 1 SLE-associated HUS, 1  malignant hypertension-associated HUS and 1  post-trauma-associated HUS). Prodromes of the patients included nausea and vomiting (73%), gross hematuria (65%), fever (50%), skin or mucosa hemorrhage (46%), upper respiratory infection (35%), oliguresis or anuresis (31%), and abdominal pain (31%). 15 patients were treated with plasma exchange(PE), among them 6 with PE and HD,  and 1 with PE plus CVVH and HD. 4 patients were treated with HD alone, and 1 patient was treated with HD and CVVH. 8 patients  received plasma infusion. Among the 18 patients who were included in the outcome analysis, 14 achieved complete response and 4 had partial response; 16 had normal renal function, and 2 progressed to ESRD(end stage renal disease). Conclusions  Most of the patients suffered from idiopathic HUS. Secondary causes include pregnancy, SLE, malignant hypertension and trauma. aHUS represents a syndrome with variable and critical clinical manifestations. Main therapies include plasma exchange and plasma infusion, with poor prognosis.

Key words: Hemolyticuremic syndrome; Atypical hemolytic-uremic syndrome; Plasma exchange