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先天性肾上腺皮质增生症的管理。尿类固醇测定——其价值综述。

Management of congenital adrenal hyperplasia. Urinary steroid estimations--review of their value.

作者信息

Bailey C C, Komrower G M, Palmer M

出版信息

Arch Dis Child. 1978 Feb;53(2):132-5. doi: 10.1136/adc.53.2.132.

Abstract

A retrospective study was made of 16 children with 21-hydroxylase-deficient congenital adrenal hyperplasia of the salt-losing variety, who were treated with fludrocortisone and prednisone and were in good health during the period under review. The height velocity of the children was subnormal, height achievement was poor, and their bone ages retarded. Urinary 17-oxosteroid and pregnanetriol excretion were used to monitor the therapy of the children and these data have been related to growth velocities. In spite of urinary steroid figures in excess of those published as desirable for monitoring therapy, the children failed to grow properly, probably as a result of glucocorticoid overdosage. Published urinary steroid criteria are considered too strict and in order to achieve them one would need to give unnecessarily high doses of steroid. Regular measurement of height velocity and skeletal maturation rate are better indicators of therapeutic control and should lead to more satisfactory growth and ultimate height.

摘要

对16名患有失盐型21-羟化酶缺乏先天性肾上腺增生症的儿童进行了一项回顾性研究,这些儿童接受了氟氢可的松和泼尼松治疗,且在研究期间健康状况良好。这些儿童的身高增长速度低于正常水平,身高发育不佳,骨龄延迟。尿17-氧类固醇和孕三醇排泄量被用于监测儿童的治疗情况,这些数据与生长速度相关。尽管尿类固醇数值超过了公布的监测治疗所需的理想数值,但这些儿童生长仍不正常,可能是由于糖皮质激素过量。已公布的尿类固醇标准被认为过于严格,为了达到这些标准,需要给予不必要的高剂量类固醇。定期测量身高增长速度和骨骼成熟率是治疗控制的更好指标,应该能带来更令人满意的生长和最终身高。

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本文引用的文献

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Growth in congenital adrenal hyperplasia.
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J Pediatr. 1968 Nov;73(5):767-73. doi: 10.1016/s0022-3476(68)80188-x.
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Am J Dis Child. 1971 Nov;122(5):408-13. doi: 10.1001/archpedi.1971.02110050078008.
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