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特发性肾病综合征患儿最终结局的长期展望及类固醇治疗结果

The long-term outlook to final outcome and steroid treatment results in children with idiopathic nephrotic syndrome.

作者信息

Dinçel Nida, Yılmaz Ebru, Kaplan Bulut İpek, Hacıkara Şükriye, Mir Sevgi

机构信息

a Department of Pediatric Nephrology , Ankara Children Health and Research Hematology Oncology Education Research Hospital , Ankara , Turkey .

b Department of Pediatric Nephrology , Dr. Behcet Uz Children Diseases Teaching and Research Hospital , Izmir , Turkey , and.

出版信息

Ren Fail. 2015;37(8):1267-72. doi: 10.3109/0886022X.2015.1073051. Epub 2015 Aug 27.

Abstract

Idiopathic Nephrotic Syndrome (INS) was defined as combination of a nephrotic syndrome and non-specific histological abnormalities of the kidney. Among these abnormalities, minimal change nephrotic syndrome (MCNS) is the most common. We report our experience with MCNS; its clinical course, treatments and outcomes. One-hundred twenty children (66 male, 54 female) with MCNS, admitted to Nephrology Department between 1987-2009 was assessed. Their clinical presentations, treatment and disease courses were reviewed. The mean duration of follow-up was 11.5 ± 1.9 years. Initially, all patients given prednisone 2 mg/kg/ day single dose per four weeks a followed by eight weeks of the same daily dose given every other day. After week 12, prednisone was progressively tapered off at the rate of 0.5 mg/kg per 15 daily intervals until complete discontinuation had been achieved by week 16. Steroid resistance was accepted as no achievement of remission following four weeks of prednisone 2 mg/kg/day followed by three intravenous pulses of corticosteroids. At the end of the initial steroid treatment, 106 (88.3%) patients were determinate steroid responsive while 14 (11.7%) patients were steroid resistance. Thirty-eight patients underwent biopsy. At the end of study recovery rate was increased from 88.3% to 94.1%. In conclusion, most of patients entered remission by our therapy end of follow up time. With the support of our satisfactory results among the whole study group, long-term prednisolone treatment still remains valid.

摘要

特发性肾病综合征(INS)被定义为肾病综合征与肾脏非特异性组织学异常的组合。在这些异常中,微小病变肾病综合征(MCNS)最为常见。我们报告了我们在MCNS方面的经验,包括其临床病程、治疗方法和结果。对1987年至2009年间入住肾病科的120例MCNS患儿(66例男性,54例女性)进行了评估。回顾了他们的临床表现、治疗情况和疾病病程。平均随访时间为11.5±1.9年。最初,所有患者给予泼尼松2mg/kg/天,每四周单剂量给药一次,随后八周给予相同的每日剂量隔日给药。在第12周后,泼尼松以每15天0.5mg/kg的速率逐渐减量,直到在第16周完全停药。激素抵抗被定义为在给予泼尼松2mg/kg/天四周后未达到缓解,随后给予三次静脉注射糖皮质激素脉冲治疗。在初始激素治疗结束时,106例(88.3%)患者被确定为激素敏感,而14例(11.7%)患者为激素抵抗。38例患者接受了活检。在研究结束时,缓解率从88.3%提高到了94.1%。总之,大多数患者在随访结束时通过我们的治疗进入缓解期。鉴于我们在整个研究组中取得的满意结果,长期泼尼松龙治疗仍然有效。

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