Adult minimal-change disease: Clinical characteristics, treatment, and outcomes
Adult minimal-change disease: Clinical characteristics, treatment, and outcomes
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DOI：
10.2215/cjn.03531006
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发表时间：
2007-05-01
影响因子：
9.8
通讯作者：
Appel, Gerald
中科院分区：
文献类型：
--
作者：
Waldman, Meryl;Crew, R. John;Appel, Gerald
在成人原发性肾病综合征病例中，微小病变（MCD）占10 - 15%。很少有系列研究在成人中研究这种疾病。对95名患有MCD并在单一转诊中心就诊的成人进行了回顾性分析。检查的是目前的特点，每天与隔日类固醇的反应，二线药物的反应，复发模式，疾病和治疗的并发症，急性肾功能衰竭（ARF）的存在，和结果数据。65例患者接受每日和23日接受隔日类固醇最初。每日和隔日治疗患者之间的缓解、至缓解时间、复发率或至复发时间无差异。超过四分之一的患者对类固醇耐药。73%的患者至少复发一次; 28%的患者经常复发。一个显着比例的频繁复发的患者成为类固醇依赖。二线药物用于类固醇依赖、类固醇抵抗或频繁复发。没有哪一种药剂被证明是上级的。与类固醇耐药患者相比，类固醇依赖患者二线药物的缓解率更高，并且类固醇依赖患者的缓解更可能完全。ARF发生在24例患者中，他们往往是老年人和高血压，血清白蛋白较低，蛋白尿比那些没有ARF。随访时，ARF发作患者的血清肌酐高于无ARF患者。4例患者进展为ESRD。这些患者不太可能对类固醇有反应，更可能在重复肾活检时出现FSGS。在该转诊MCD人群中，每日和隔日类固醇治疗的反应相似。二线药物在类固醇依赖患者中的反应更大。ARF发生在相当数量的成人MCD患者中，并可能留下残余肾功能不全。少数患者进展为ESRD。
Minimal-change disease (MCD) counts for 10 to 15% of cases of primary nephrotic syndrome in adults. Few series have examined this disease in adults. A retrospective review was performed of 95 adults who had MCD and were seen at a single referral center. Examined were presenting features, response to daily versus alternate-day steroids, response to second-line agents, relapse patterns, complications of the disease and therapy, presence of acute renal failure (ARF), and outcome data. Sixty-five patients received daily and 23 received alternate-day steroids initially. There were no differences in remissions, time to remission, relapse rate, or time to relapse between daily- and altemate-day treated patients. More than one quarter of patients were steroid resistant. At least one relapse occurred in 73% of patients; 28% were frequently relapsing. A significant proportion of frequently relapsing patients became steroid dependent. Second-line agents were used for steroid dependence, steroid resistance, or frequent relapses. No single agent proved superior. There were more remissions with second-line agents in steroid-dependent patients compared with steroid-resistant patients, and remissions were more likely to be complete in steroid-dependent patients. ARF occurred in 24 patients; they tended to be older and hypertensive with lower serum albumin and more proteinuria than those without ARF. At follow up, patients with an episode of ARF had higher serum creatinine than those without ARF. Four patients progressed to ESRD. These patients were less likely to have responded to steroids and more likely to have FSGS on repeat renal biopsy. In this referral MCD population, response to daily and alternate-day steroids is similar. Second-line agents give greater response in patients who are steroid dependent. ARF occurs in a significant number of adult MCD patients and may leave residual renal dysfunction. Few patients progress to ESRD.