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[性早熟。关于诊断条件和病因学方面的评论]

[Precocious puberty. Comment on the diagnostic conditions and etiological aspects].

作者信息

Brauner R, Thibaud E, Pomarede R, Cachin O, Hausser C, Czernichow P, Rappaport R

出版信息

Ann Endocrinol (Paris). 1982 Dec;43(6):497-508.

PMID:7183238
Abstract

The present review is based on the retrospective study of 124 children with precocious puberty, 92 girls and 32 boys. In girls, the analysis of the clinical initial presentation has shown that premature pubarche (n = 18), premature adrenarche (n = 2) or isolated menstruations (n = 3) must be ruled out, as these symptoms can remain isolated for more than a year. However, in most cases the presence of growth acceleration and vaginal estrogenisation was of major diagnostic value. Bone maturation, although generally accelerated, can be normal in recently developed puberty. Precocious puberty may proceed by steps, with complete disappearance of physical signs in the intervals. Organic causes were found in 31% of the girls, and 44% of the boys with some characteristic features as rapid progression, and elevated LH response to LRF stimulation. Main causes were glioma of the optic chiasma (n = 11), 3rd ventricule invasive tumors, hamartoma (n = 8). The latter should be looked for by a non invasive procedure as the CT scan. In girls, precocious puberty with very high circulating estrogen levels was observed as part of a McCune-Albright syndrome. As the effect of precocious puberty on the final adult height is variable, the evaluation of therapeutic results remains uncertain. Medroxyprogesterone as well cyproterone acetate have not been fully efficient in controlling bone maturation. More recently, and still controversial, the treatment with long acting LRF analogues might provide a more satisfactory statural prognosis.

摘要

本综述基于对124例性早熟儿童的回顾性研究,其中女孩92例,男孩32例。对于女孩,临床初始表现分析表明,必须排除阴毛早现(n = 18)、肾上腺功能早现(n = 2)或孤立性月经(n = 3),因为这些症状可能会孤立存在一年以上。然而,在大多数情况下,生长加速和阴道雌激素化的出现具有重要的诊断价值。骨成熟虽然通常加速,但在近期开始发育的青春期可能正常。性早熟可能呈阶段性进展,期间体征会完全消失。在31%的女孩和44%的男孩中发现了器质性病因,其具有一些特征,如进展迅速,对促黄体生成素释放因子(LRF)刺激的促黄体生成素(LH)反应升高。主要病因是视交叉神经胶质瘤(n = 11)、第三脑室侵袭性肿瘤、错构瘤(n = 8)。后者应通过如CT扫描等非侵入性检查来寻找。在女孩中,观察到作为McCune - Albright综合征一部分的性早熟,其循环雌激素水平非常高。由于性早熟对最终成人身高的影响各不相同,治疗效果的评估仍然不确定。甲羟孕酮以及醋酸环丙孕酮在控制骨成熟方面尚未完全有效。最近,长效LRF类似物的治疗虽然仍有争议,但可能会提供更令人满意的身高预后。

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