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[21-羟化酶缺乏所致先天性肾上腺皮质增生症:27例典型型患者的最终身高]

[Congenital adrenal hyperplasia due to 21-hydroxylase deficiency: final height in 27 patients with the classical form].

作者信息

Lemos-Marini Sofia H V, Guerra-Júnior Gil, Morcillo André M, Baptista Maria Tereza M, Silva Luciana O, Maciel-Guerra Andréa T

机构信息

Departamento de Pediatria, Ciped, Faculdade de Ciências Médicas, Universidade Estadual de Campinas, SP.

出版信息

Arq Bras Endocrinol Metabol. 2005 Dec;49(6):902-7. doi: 10.1590/s0004-27302005000600008. Epub 2006 Mar 16.

DOI:10.1590/s0004-27302005000600008
PMID:16544012
Abstract

OBJECTIVE

To determine final height (FH) in congenital adrenal hyperplasia (CAH) patients and investigate conditions allowing better height outcome.

METHODOLOGY

13 salt-wasting (SW) and 14 simple virilizing (SV) patients were studied. FH and target height (TH) were transformed into standard deviation score (z). Data were analyzed according to sex, clinical form, age at treatment onset and length of treatment before attaining FH.

RESULTS

zFH (n = 27) was -1.57 +/- 1.01. FH (-1.50 +/- 1.03) was below TH (-0.78 +/- 0.84) (n = 25, p < 0.001). FH has not differed according to sex, clinical form and age at therapy onset although there was a trend towards better FH in SW patients and in early treated cases; there was significant difference (p = 0.018) between patients treated for less than 5 years (-2.49 +/- 1.03) and those accompanied longer than 10 years (-1.21 +/- 0.88) before attaining FH.

CONCLUSIONS

There was a FH impairment and adult height improvement seems to depend mainly on early diagnosis and treatment.

摘要

目的

确定先天性肾上腺皮质增生症(CAH)患者的最终身高(FH),并研究能实现更好身高结局的条件。

方法

对13例失盐型(SW)和14例单纯男性化型(SV)患者进行研究。将FH和靶身高(TH)转换为标准差评分(z)。根据性别、临床类型、开始治疗时的年龄以及达到FH前的治疗时长对数据进行分析。

结果

zFH(n = 27)为-1.57±1.01。FH(-1.50±1.03)低于TH(-0.78±0.84)(n = 25,p < 0.001)。尽管SW患者和早期治疗的病例有FH更好的趋势,但FH在性别、临床类型和治疗开始时的年龄方面并无差异;在达到FH之前,治疗少于5年的患者(-2.49±1.03)与治疗超过10年的患者(-1.21±0.88)之间存在显著差异(p = 0.018)。

结论

存在FH受损情况,成人身高的改善似乎主要取决于早期诊断和治疗。

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Indian J Endocrinol Metab. 2019 May-Jun;23(3):298-306. doi: 10.4103/ijem.IJEM_99_19.
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Growth and reproductive outcomes in congenital adrenal hyperplasia.先天性肾上腺皮质增生症的生长及生殖结局
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