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婴儿期先天性肾上腺皮质增生症的治疗反应。

Response to treatment of congenital adrenal hyperplasia in infancy.

作者信息

Young M C, Hughes I A

机构信息

Department of Child Health, University of Wales College of Medicine, Heath Park, Cardiff.

出版信息

Arch Dis Child. 1990 Apr;65(4):441-4. doi: 10.1136/adc.65.4.441.

Abstract

Nine infants with congenital adrenal hyperplasia were started on replacement doses of hydrocortisone (20.6-32.6 mg/m2/day) without receiving a high dose for an initial period first. Plasma adrenal steroid concentrations fell to acceptable levels by 3 months of age. Adequate biochemical control was maintained and satisfactory growth achieved even though the mean dose of hydrocortisone had been reduced to 15 mg/m2/day by the age of 3 years. Inadvertent overtreatment and growth suppression in infants with congenital adrenal hyperplasia may be avoided by using replacement doses from the start, and by permitting the relative dose of hydrocortisone to fall as the body surface area increases during the first year of life.

摘要

九名先天性肾上腺皮质增生症婴儿一开始就接受氢化可的松替代剂量治疗(20.6 - 32.6毫克/平方米/天),未先给予高剂量初始治疗。到3个月大时,血浆肾上腺类固醇浓度降至可接受水平。即使到3岁时氢化可的松平均剂量已降至15毫克/平方米/天,仍维持了充分的生化控制并实现了令人满意的生长。对于先天性肾上腺皮质增生症婴儿,从一开始就使用替代剂量,并随着出生后第一年体表面积增加允许氢化可的松相对剂量下降,可避免意外的过度治疗和生长抑制。

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本文引用的文献

1
A direct radioimmunoassay for 11-deoxycortisol.
Steroids. 1982 Feb;39(2):115-28. doi: 10.1016/0039-128x(82)90080-0.
5
Experience with long-term therapy in congenital adrenal hyperplasia.
J Pediatr. 1974 Jul;85(1):12-9. doi: 10.1016/s0022-3476(74)80277-5.
8
The effect of treatment of final height in classical congenital adrenal hyperplasia (CAH).
Acta Endocrinol Suppl (Copenh). 1986;279:305-14. doi: 10.1530/acta.0.112s305.
10
Management of congenital adrenal hyperplasia.先天性肾上腺皮质增生症的管理
Arch Dis Child. 1988 Nov;63(11):1399-404. doi: 10.1136/adc.63.11.1399.

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