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类固醇敏感型肾病综合征的生长与内分泌功能

Growth and endocrine function in steroid sensitive nephrotic syndrome.

作者信息

Rees L, Greene S A, Adlard P, Jones J, Haycock G B, Rigden S P, Preece M, Chantler C

机构信息

Evelina Children's Hospital, London.

出版信息

Arch Dis Child. 1988 May;63(5):484-90. doi: 10.1136/adc.63.5.484.

Abstract

Longitudinal height data and physical development were assessed in 29 boys and 12 girls taking long term steroid treatment for steroid sensitive nephrotic syndrome. Growth in both boys and girls, assessed by changes in height standard deviation score (delta Ht SDS), worsened significantly with chronological age. There was a significant negative correlation between delta Ht SDS and duration of treatment in boys, but not in girls. There was no correlation between delta Ht SDS and relapse rate or the use of cyclophosphamide. In the boys, Ht SDS decreased significantly only after the age of 10 years and was associated with delay in the appearance of secondary sexual characteristics. Eight adolescent boys were assessed endocrinologically by an overnight hormone profile. Blunting of the pulsatility of growth hormone and gonadotrophins was seen in six. Normal profiles were seen in two subjects who were both off steroid treatment at the time of study. Abnormal endocrine function in adolescent boys treated long term for steroid sensitive nephrotic syndrome corresponded with the clinical picture of delayed onset of puberty, which accounted for severe growth retardation in a substantial proportion of subjects.

摘要

对29名男孩和12名女孩进行了纵向身高数据和身体发育评估,这些儿童因类固醇敏感性肾病综合征正在接受长期类固醇治疗。通过身高标准差评分变化(ΔHt SDS)评估,男孩和女孩的生长情况均随实际年龄显著恶化。男孩的ΔHt SDS与治疗持续时间之间存在显著负相关,而女孩则不存在。ΔHt SDS与复发率或环磷酰胺的使用之间没有相关性。在男孩中,Ht SDS仅在10岁以后显著下降,并且与第二性征出现延迟有关。对8名青春期男孩进行了夜间激素谱的内分泌评估。6名男孩出现生长激素和促性腺激素脉冲性减弱。在研究时均已停用类固醇治疗的2名受试者中观察到正常的激素谱。长期接受类固醇敏感性肾病综合征治疗的青春期男孩的内分泌功能异常与青春期延迟的临床表现相符,这在相当一部分受试者中导致了严重的生长迟缓。

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