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21-羟化酶缺陷型先天性肾上腺皮质增生症患儿长期生长轨迹的性别二态性。

Gender dichotomy in long term growth trajectories of children with 21-hydroxylase deficiency congenital adrenal hyperplasia.

机构信息

Paediatric Endocrinology, Manchester Academic Health Sciences Centre, University of Manchester, Manchester, UK.

出版信息

Horm Res Paediatr. 2011;75(3):206-12. doi: 10.1159/000320700. Epub 2010 Oct 21.

Abstract

AIM

To evaluate longitudinal growth in 21-hydroxylase deficiency congenital adrenal hyperplasia (CAH), factors contributing to this and outcome for BMI, weight (Wt) and height (Ht) in adolescence.

METHODS

Multi-level longitudinal models were used to evaluate growth patterns of 45 salt wasters (SW) and 12 non-SW with CAH.

RESULTS

Ht, Wt and BMI growth curves differed between SW and non-SW, and by gender. In contrast to SW and males, non-SW females showed a markedly different pattern with a progressive increase in Wt and BMI SDS over childhood and adolescence and only a slight gain in Ht SDS. BMI SDS remained above 0 after early childhood. Over the 15 years, the growth variables were negatively associated with fludrocortisone (FC) (shorter children were receiving larger doses) but not hydrocortisone (HC) doses nor FC and HC doses in the first year.

CONCLUSION

The growth patterns in these children with CAH were influenced by age, gender, phenotype and FC treatment. There was a trend to increasing BMI from an early age, greater adiposity during childhood and females had disproportionately greater adiposity but shorter stature during adolescence. These patients therefore have a predisposition to obesity in childhood and later life independent of early corticosteroid treatment.

摘要

目的

评估 21-羟化酶缺乏型先天性肾上腺皮质增生症(CAH)的纵向生长情况,探讨影响生长的因素以及青少年时期 BMI、体重(Wt)和身高(Ht)的结果。

方法

采用多水平纵向模型评估 45 名盐皮质激素过多症(SW)和 12 名非 SW 型 CAH 患者的生长模式。

结果

Ht、Wt 和 BMI 生长曲线在 SW 和非 SW 之间以及性别之间存在差异。与 SW 和男性不同,非 SW 女性表现出明显不同的模式,其 Wt 和 BMI SDS 在儿童期和青春期持续增加,而 Ht SDS 仅略有增加。BMI SDS 在幼儿期后仍保持在 0 以上。在 15 年的时间里,生长变量与氟氢可的松(FC)呈负相关(个子矮的孩子接受的剂量更大),但与氢化可的松(HC)剂量或第一年的 FC 和 HC 剂量无关。

结论

这些 CAH 患儿的生长模式受年龄、性别、表型和 FC 治疗的影响。从早期开始就有 BMI 增加的趋势,儿童期肥胖程度增加,而女性在青春期时肥胖程度不成比例地增加,但身高较矮。因此,这些患者在儿童期和以后的生活中存在肥胖的倾向,与早期皮质类固醇治疗无关。

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