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特发性肾病综合征男孩长期环孢素和类固醇治疗后的生长情况。

Growth in boys with idiopathic nephrotic syndrome on long-term cyclosporin and steroid treatment.

机构信息

Assistance publique-Hôpitaux de Paris, Hôpital Robert Debré, Service de Néphrologie Pédiatrique, Paris, France.

出版信息

Pediatr Nephrol. 2009 Dec;24(12):2393-400. doi: 10.1007/s00467-009-1266-y. Epub 2009 Aug 11.

Abstract

Although steroid-free remission can usually be achieved with cyclosporin A (CsA) in patients with steroid-dependent nephrotic syndrome (SDNS), some CsA-treated patients require long-term steroid therapy. Data on growth in these patients are scarce. Sixty-four boys with SDNS receiving long-term CsA and steroid therapy were retrospectively analyzed. During the 10-year follow-up period, height standard deviation score (HSDS) remained in the normal range in 47 patients but was below -2 SD in 17 patients. The occurrence of growth retardation was influenced by height at diagnosis and the number of relapses. Thirty patients were followed for at least 3 years before and after age 12. The decrease in HSDS per year of disease in patients older than 12 years was twice that observed in children younger than 12. However, adult height was < or = -2 SD in only two of the 14 patients reaching adult height, reflecting potential catch-up growth during late puberty. Careful monitoring of growth is recommended, given than up to 25% of patients experienced severe growth retardation during the course of their disease.

摘要

尽管环孢素 A(CsA)可使大多数激素依赖型肾病综合征(SDNS)患者达到无激素缓解,但一些 CsA 治疗的患者仍需要长期激素治疗。关于这些患者生长的数据较为缺乏。本研究回顾性分析了 64 例接受长期 CsA 和激素治疗的 SDNS 男孩。在 10 年的随访期间,47 例患者的身高标准差评分(HSDS)保持在正常范围内,但 17 例患者的 HSDS 低于-2SD。生长迟缓的发生受诊断时的身高和复发次数的影响。30 例患者在 12 岁后至少随访 3 年。12 岁以上患者的 HSDS 每年下降值是 12 岁以下儿童的两倍。然而,在达到成年身高的 14 例患者中,仅有 2 例患者的成年身高<-2SD,提示青春期后期可能存在追赶性生长。鉴于 25%的患者在疾病过程中出现严重生长迟缓,建议密切监测生长情况。

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