Causes and clinical features of 20 patients with hemolytic uremic syndrome / 中华肾脏病杂志

ABSTRACT

Objective To analyze the causes and clinical features of 20 patients with hemolytic uremic syndrome (HUS) in order to improve the prognosis. Methods Twenty patients with HUS hospitalized in our department during July 1998 to December 2004 were enrolled in this study. The etiology, clinical features, individualized therapy and prognosis were retrospectively analyzed. Results These 20 HUS patients (18 HUS patients complicated with ARF) accounted for 2.48% of total patients with acute renal failure (ARF) in our hospital. There were 16 females and 4 males with mean age of (49.11±19.85) years. Five patients were idiopathic HUS and the other 15 were secondary HUS (10 SLE-associated HUS, 2 pregnancy-associated HUS, 1 APS-associated HUS, 1 renal arterioles sclerosis-associated HUS and 1 drug-associated HUS). Eighteen cases had ARF and 15 had nephrotic syndrome. Hypertension was found in 17 patients, among them 4 had malignant hypertension. Twelve patients had gross hematuria and the other 8 had microscopic hematuria. Diarrhea was found only in 1 patient. At onset, mean serum creatinine was (504.40±381.10) μmol/L and 24-h proteinuria was (5.0±2.6) g. Renal biopsy was pedormed in 16 patients. Fourteen patients received hemopurification therapy 2 patients plasma exchange (PE); 8 patients PE combined with CVVHDF and /or HD; 4 patients CVVHDF and HD. Seven cases were treated with intravenous immunoglobulin (IVIg). Patients with SLE-associated HUS received the corticosteroids and immunosuppressants. Low or middle dosage of corticosteroids( 10-40 mg/d) was administered in patients with idiopathic HUS. For patients with APS, low molecular weight heparin was used. HUS patients were followed-up for average (46.0±32.8) months. During follow-up, 4 patients died, 11 recovered from renal insufficiency, 4 progressed to end stage renal failure of whom 2 depended on dialysis and 1 lost. The survival rates of SLE-associated HUS and none-SLE-associated HUS were 70% and 90%, and renal survival rates were 50% and 60% respectively, which were not significantly different between these two groups. Conclusions Most of the patients are secondary HUS. SLE-associated HUS is the main type of secondary HUS. The prognosis of SLE-associated HUS is poor. PE and IVIg are main therapy. Low dosage of corticosteroids can reduce relapse of HUS. Immunosuppressants can improve the prognosis.