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努南综合征的生长激素治疗:生长反应及特征

Growth hormone therapy in Noonan syndrome: growth response and characteristics.

作者信息

Westphal Otto

机构信息

Queen Silvia Childrens Hospital, The Sahlgrenska Academy, University of Gothenburg, Goteborg, Sweden.

出版信息

Horm Res. 2009 Dec;72 Suppl 2:41-5. doi: 10.1159/000243778. Epub 2009 Dec 22.

Abstract

Growth hormone treatment in Noonan syndrome increases growth velocity significantly during the first 2 years of treatment and, to some extent, until puberty. This increase is more pronounced if treatment is started at an early age. Treatment before the age of 5 years is not recommended due to an increased risk of malignancies. In contrast to other growth hormone-treated patients, a slight but significant further increase in height gain can be expected during pubertal growth (at least in boys). Final height improvement varies between 1 and 2 SDS in different studies. Cardiac function does not seem to be impaired during treatment. No significant adverse events have been reported.

摘要

努南综合征患者接受生长激素治疗,在治疗的头两年生长速度显著加快,在一定程度上这种加快持续至青春期。如果在幼年开始治疗,生长速度加快更为明显。由于患恶性肿瘤风险增加,不建议5岁前开始治疗。与其他接受生长激素治疗的患者不同,青春期生长期间(至少在男孩中)预计身高会有轻微但显著的进一步增加。不同研究中最终身高改善幅度在1至2个标准差之间。治疗期间心脏功能似乎未受损害。未报告重大不良事件。

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