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难以控制的先天性肾上腺皮质增生症患儿:是否需要持续皮下给予氢化可的松治疗。

The child with difficult to control Congenital Adrenal Hyperplasia: is there a place for continuous subcutaneous hydrocortisone therapy.

机构信息

Developmental Endocrinology Research Group, UCL Institute of Child Health, London, UK.

出版信息

Clin Endocrinol (Oxf). 2014 Jul;81(1):15-8. doi: 10.1111/cen.12453. Epub 2014 Apr 15.

Abstract

The treatment of congenital adrenal hyperplasia requires administration of glucocorticoid and mineralocorticoid to replace the deficit resulting from the enzymatic block. During puberty, cortisol metabolism changes with an increase in clearance, which may lead to unstable control. This may require an increase in dosing frequency, and if this proves problematic, continuous subcutaneous hydrocortisone delivery using insulin pump technology can be valuable. The potential indications for such therapy along with some practical considerations are detailed in this Clinical Question report.

摘要

先天性肾上腺皮质增生症的治疗需要给予糖皮质激素和盐皮质激素以补充因酶缺乏所致的不足。在青春期,皮质醇代谢随清除率的增加而发生变化,这可能导致控制不稳定。这可能需要增加给药频率,如果这被证明有问题,可以使用胰岛素泵技术进行持续的皮下氢化可的松给药。该临床问题报告详细介绍了这种治疗的潜在适应证和一些实际考虑因素。

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