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致密性骨发育不全患儿生长激素分泌缺陷及生长激素治疗后线性生长改善

Defective growth hormone secretion in children with pycnodysostosis and improved linear growth after growth hormone treatment.

作者信息

Soliman A T, Rajab A, AlSalmi I, Darwish A, Asfour M

机构信息

Department of Paediatrics, Royal Hospital, Muscat, Oman.

出版信息

Arch Dis Child. 1996 Sep;75(3):242-4. doi: 10.1136/adc.75.3.242.

DOI:10.1136/adc.75.3.242
PMID:8976667
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1511688/
Abstract

Short stature is a characteristic feature of pycnodysostosis. We report defective growth hormone secretion in response to provocation and low insulin-like growth factor-I (IGF-I) concentration in five out of six patients with pycnodysostosis. Physiological replacement with growth hormone increased IGF-I concentration and improved linear growth in these children.

摘要

身材矮小是致密性成骨不全症的一个特征性表现。我们报告了6例致密性成骨不全症患者中有5例在激发试验中生长激素分泌存在缺陷且胰岛素样生长因子-I（IGF-I）浓度较低。对这些儿童进行生长激素的生理性替代治疗可提高IGF-I浓度并改善线性生长。

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