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因21-羟化酶缺乏所致经典型先天性肾上腺皮质增生症患者的生长情况

Growth in patients with classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

作者信息

Dörr Helmuth G

机构信息

Division of Pediatric Endocrinology, University Hospital for Children and Adolescents, Erlangen, Germany.

出版信息

Horm Res. 2007;68 Suppl 5:93-9. doi: 10.1159/000110587. Epub 2007 Dec 10.

Abstract

BACKGROUND

One important goal in the management of children with classic congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is to achieve normal growth. Reviewing available data published over the last few years on growth and height outcomes in CAH patients, it becomes evident that an acceptable height can be achieved by many CAH patients. However, linear growth and final adult height may be stunted in some patients due to factors related to the timing of diagnosis, the age at therapy onset, the start of therapy, the adequacy of metabolic control, the quality of therapy, patient compliance and the experience of the treating physician. In children with CAH who have a poor height prognosis, additional treatment options should be considered.

CONCLUSIONS

Treatment of children with CAH requires individualized approaches to prevent long-term growth failure.

摘要

背景

对于因21-羟化酶缺乏导致的经典型先天性肾上腺皮质增生症(CAH)患儿,管理的一个重要目标是实现正常生长。回顾过去几年发表的关于CAH患者生长和身高结局的现有数据,很明显许多CAH患者能够达到可接受的身高。然而,由于诊断时间、治疗开始年龄、治疗起始、代谢控制的充分性、治疗质量、患者依从性以及治疗医生的经验等因素,一些患者的线性生长和最终成人身高可能会受到阻碍。对于身高预后较差的CAH患儿,应考虑其他治疗选择。

结论

CAH患儿的治疗需要个体化方法以防止长期生长失败。

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