生长激素治疗软骨毛发发育不全：对生长和免疫系统的影响。

Growth hormone treatment in cartilage-hair hypoplasia: effects on growth and the immune system.

作者信息

Bocca G, Weemaes C M, van der Burgt I, Otten B J

机构信息

Department of Pediatrics, University Medical Center St Radboud, Nijmegen, The Netherlands.

出版信息

J Pediatr Endocrinol Metab. 2004 Jan;17(1):47-54. doi: 10.1515/jpem.2004.17.1.47.

DOI:10.1515/jpem.2004.17.1.47

PMID:14960021

Abstract

Cartilage-hair hypoplasia (CHH) is a rare autosomal recessive disorder characterized by metaphyseal chondrodysplasia with severe growth retardation and impaired immunity. We studied the effects of growth hormone treatment on growth parameters and the immune system in four children with CHH. The effects of growth hormone on growth parameters are the most prominent in patients with the mildest growth retardation. However, the effects are temporary and last only for 1 year. There is no gain in final height. Serum immunoglobulins did not change during growth hormone treatment. We conclude that growth hormone treatment is not beneficial in children with CHH.

摘要

软骨毛发发育不全（CHH）是一种罕见的常染色体隐性疾病，其特征为干骺端软骨发育异常，伴有严重生长迟缓及免疫功能受损。我们研究了生长激素治疗对4例CHH患儿生长参数及免疫系统的影响。生长激素对生长参数的影响在生长迟缓程度最轻的患者中最为显著。然而，这些影响是暂时的，仅持续1年。最终身高并无增加。生长激素治疗期间血清免疫球蛋白未发生变化。我们得出结论，生长激素治疗对CHH患儿并无益处。