Treatment of hemolytic uremic syndrome after acute stage

Objective Hemolytic uremic sy ndrome (HUS) is a common primary disease that can cause acute renal failure in c hildhood.Renal disease is the most important long-term complication in patient s who survived the acute stage of HUS.Use of angiotensin-converting enzyme in hibitors (ACEI) and a restricted protein intake may be beneficial to the patient s.However, it is not established whether such patients should be treated with s teroids and immunosuppressors.The present study aimed to probe into the benefit of using steroid and immunosuppressor in patients after acute stage of HUS. Methods The subjects included 17 patients (aged 9 m onths to 15 years, 12 males, 5 females) with HUS. Thirteen patients recovered f rom the acute stage of HUS, and underment continuative treatment and follow-up .All the patients were treated with ACEI and early restriction of protein intak e.Additionally, 2 children manifested as glomerulonephritis, one was treated wi th triperygium glycosides.Other 11 children who manifested as nephrotic syndrom e were treated with prednisone, among them 5 children had no response or had inc omplete response to prednisone, for these children short-term high dose cycloph osphamide or methylprednisolone pulse treatment were added; in 3 of the children short-term high dose methylprednisolone treatment was applied additionally for membranoproliferative glomerulonephritis and/or focal segmental glomerulosclero sis and crescentic glomerulonephritis.Results Af ter follow-up for 2 months to 8 years, 4 patients with milder disease recovered , their blood pressure, renal function and urinanalysis became normal, but 1 pat ient had recurrence. Among 9 patients with severe disease, 6 maintained normal blood pressure, recovered renal function and urinanalysis, the other 3 patients failed to comply with treatment protocol and died during the 3rd, 9th and 13th m onth. The remainder (4 cases) gave up therapy and died on the 27th to 48th days of the course.Conclusion The treatment applied in this study could improve the prognosis of patients after acute phase of HUS evidently by using the steroid and immuno suppressor according to clinical class ification and pathological findings. It is recommended that triperygium glycosi des is beneficial to children with glomerulonephritis, proteinuria and hematuria after acute stage of HUS. Adjustment of therapeutic schedule based on patholog ical findings after renal biopsy is helpful. To the patients with progressive r enal failure who have no response to the steroid and immunosuppressors, steroid and immunosuppressor should be discontinued and dialysis treatment should be app lied. Protocol compliance is also an important factor.