【摘要】 目的探讨溶血性尿毒症综合征(HUS)患儿渡过急性期后如何促进肾功能的修复、延缓肾损害进程,探讨HUS急性期后的治疗方法。方法分析1993年至2005年我科收治的17例HUS患儿的临床资料。13例接受急性期后治疗患儿除用血管紧张素转化酶抑制剂(ACEI)和限制蛋白质摄入外,参照中华医学会儿科学分会肾脏病学组制定的“小儿肾小球疾病的临床分类、诊断及治疗”(方案),按临床分型、对泼尼松治疗的反应及病理类型拟定治疗方案。2例临床表现为肾小球肾炎的患儿中1例应用雷公藤多甙。11例表现为肾病综合征的患儿均用泼尼松;其中5例泼尼松治疗不缓解或部分缓解者,加用环磷酰胺冲击(4例)或甲泼尼龙冲击(1例)治疗;3例因肾组织病理改变为膜增殖+/-局灶节段性肾小球硬化、新月体形成,加用甲泼尼龙冲击。结果随访2个月~8年,轻型4例(1例复发1次)血压、血尿素氮(BUN)、血肌酐(Cr)及尿常规均正常。重型9例中6例血压、BUN、Cr、尿常规正常;3例持续尿检异常,肾功能不全,且没有坚持治疗,分别于病程的第3、9和13个月死亡。4例(均为重型)放弃治疗者分别于病程的第27~48天死亡。结论对渡过急性期的HUS患儿依据其临床分型和肾病理改变参照肾脏病学组制定的方案治疗有望改善预后。除急性期病情轻重、治疗的合理性影响预后外,患儿家长对治疗的依从性也是一个重要因素。更多还原

【Abstract】 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.更多还原