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青春期特发性生长激素缺乏症患者的管理。

Management of idiopathic growth hormone deficient patients during puberty.

作者信息

Price D A, Shalet S M, Clayton P E

机构信息

Department of Child Health, University of Manchester, Royal Manchester Children's Hospital, UK.

出版信息

Acta Paediatr Scand Suppl. 1988;347:44-51.

PMID:3151034
Abstract

Boys with idiopathic GH deficiency, treated with hGH and entering spontaneous puberty, have an onset of puberty and peak height velocity (PHV) at a late chronological age but normal bone age. PHV occurs at G3 with testicular volumes of 6-12 ml. The size of PHV and the height gain after G2 are similar to those of a normal delayed adolescent. In contrast, idiopathic GH deficient girls have an onset of puberty and PHV nearer to a normal chronological age and at an early bone age. PHV occurs at B2 and its size and the height gain after B2 are similar to those of normal girls. The length of time of pubertal growth is shorter in both GH deficient boys and girls. Very late induction of puberty in idiopathic GH deficient boys results in psychosocial damage and in bodily disproportion. It is suggested that induction of puberty be considered no later than 14.5 years in boys and 13.5 years in girls with the use of low-dose sex steroids. The decision to induce puberty should be taken to avoid psychosocial problems and be independent of proof of associated gonadotrophin deficiency. In GH deficient girls with early puberty, therapies to delay puberty may be considered. There are theoretical grounds for increasing the GH dose given during puberty, but present dose-response studies fail to include controls for important biological variables and are so far inconclusive. Cost-effectiveness is an important consideration. Increasing the frequency of injections probably improves the growth effect for a given dose of GH.

摘要

患有特发性生长激素缺乏症的男孩,接受生长激素治疗并进入自然青春期后,青春期启动和身高增长峰值速度(PHV)的发生年龄较晚,但骨龄正常。PHV出现在睾丸体积为6 - 12毫升的G3期。G2期后的PHV大小和身高增长与正常青春期延迟的青少年相似。相比之下,特发性生长激素缺乏症女孩的青春期启动和PHV更接近正常实际年龄,且骨龄较早。PHV出现在B2期,其大小和B2期后的身高增长与正常女孩相似。生长激素缺乏的男孩和女孩青春期生长的时间都较短。特发性生长激素缺乏症男孩青春期启动过晚会导致心理社会损害和身体比例失调。建议对于患有特发性生长激素缺乏症的男孩,在14.5岁之前、女孩在13.5岁之前考虑使用低剂量性类固醇诱导青春期。诱导青春期的决定应基于避免心理社会问题,且不依赖于相关促性腺激素缺乏的证据。对于青春期过早的生长激素缺乏症女孩,可考虑采取延迟青春期的治疗方法。从理论上讲,增加青春期期间的生长激素剂量可能有益,但目前的剂量反应研究未纳入对重要生物学变量的对照,因此尚无定论。成本效益是一个重要的考虑因素。增加注射频率可能会提高给定剂量生长激素的生长效果。

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