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[皮质醇-肾上腺-生殖器综合征。诊断、治疗、随访]

[Cortico-adrenal-genital syndrome. Diagnosis, therapy, follow-up].

作者信息

Gargantini L, Trifirò G, Bianchi C, Chiumello G

出版信息

Pediatr Med Chir. 1985 Jul-Aug;7(4):487-90.

PMID:3016673
Abstract

Recently new advances are achieved in the diagnosis of CAH, by discovering clinical forms different from the classical one: the late-onset and the cryptic-CAH. While the diagnosis of classical form mainly depends on dosing 17-OH-progesterone levels in all newborns, the detection of non-classical form requires measurement of adrenal steroids in basal conditions and after ACTH stimulation. The Authors recommend a careful follow-up of treated patients by frequent clinical and hormonal evaluations in order to prevent slightly unsuitable therapy that could compromise the achievement of adequate adult height. It is necessary to evaluate not only the daily total amount, but also the pattern of administration and which adrenal preparation is preferable.

摘要

最近,先天性肾上腺皮质增生症(CAH)的诊断取得了新进展,发现了与经典类型不同的临床形式:迟发型和隐匿型CAH。虽然经典型的诊断主要依赖于检测所有新生儿的17-羟孕酮水平,但非经典型的检测需要在基础状态和促肾上腺皮质激素(ACTH)刺激后测量肾上腺类固醇。作者建议通过频繁的临床和激素评估对接受治疗的患者进行仔细随访,以防止可能影响达到正常成人身高的轻微不适当治疗。不仅要评估每日总量,还要评估给药方式以及哪种肾上腺制剂更可取。

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