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先天性生长激素缺乏症与髋关节发育不良和 Legg-Calve-Perthes 病有关。

Congenital growth hormone deficiency associated with hip dysplasia and Legg-Calve-Perthes disease.

机构信息

Great Ormond Street Hospital for Children, London, UK.

UCL GOS Institute of Child Health, University College London, London, UK.

出版信息

Clin Endocrinol (Oxf). 2021 Apr;94(4):590-597. doi: 10.1111/cen.14365. Epub 2020 Nov 20.

DOI:10.1111/cen.14365
PMID:33147364
Abstract

OBJECTIVE

Growth hormone deficiency (GHD) is usually treated with recombinant human GH (rhGH), and this has been rarely associated with hip disorders. We analysed the clinical data of patients with congenital GHD receiving rhGH who had associated hip dysplasia or Legg-Calve-Perthes disease (LCPD), with a view to determining whether the hip dysplasia was associated with the underlying disease or with rhGH treatment.

DESIGN

We performed a retrospective analysis of paediatric and adolescent patients seen between 1992-2018 with congenital GHD and hip disorders. Data were collected through a review of the patients' medical records and included demographics, clinical and imaging data, and the time frame between the onset of the symptoms related to the hip disorders and the onset of GH treatment.

RESULTS

Of the 13 patients with hip disorders, hip dysplasia was present in ten patients and LCPD in three. Hip dysplasia was diagnosed before rhGH was initiated in 50% of cases. These patients had bilateral hip dysplasia and isolated GHD. LCPD was diagnosed in one patient before rhGH was commenced and did not progress. In two patients, LCPD was diagnosed after rhGH was started and did temporarily progress in one of them, but rhGH was not discontinued because LCPD did not seem to be related to rhGH treatment.

CONCLUSIONS

This study suggests that hip dysplasia could be a manifestation of an underlying GHD. Additionally, rhGH treatment may not necessarily be causative of LCPD.

摘要

目的

生长激素缺乏症(GHD)通常采用重组人生长激素(rhGH)治疗,但这种治疗很少与髋关节疾病有关。我们分析了接受 rhGH 治疗的先天性 GHD 患者伴发髋关节发育不良或 Legg-Calve-Perthes 病(LCPD)的临床资料,以确定髋关节发育不良是与基础疾病有关,还是与 rhGH 治疗有关。

设计

我们对 1992 年至 2018 年间就诊的患有先天性 GHD 和髋关节疾病的儿科和青少年患者进行了回顾性分析。通过查阅患者病历收集数据,包括人口统计学、临床和影像学数据,以及与髋关节疾病相关症状发作与 GH 治疗开始之间的时间框架。

结果

在 13 例髋关节疾病患者中,10 例存在髋关节发育不良,3 例存在 LCPD。50%的髋关节发育不良病例在开始 rhGH 治疗之前被诊断出来。这些患者存在双侧髋关节发育不良和单纯性 GHD。1 例患者在开始 rhGH 治疗之前被诊断为 LCPD,但未进展。在 2 例患者中,LCPD 在开始 rhGH 治疗后被诊断出来,其中 1 例暂时进展,但并未停止 rhGH 治疗,因为 LCPD 似乎与 rhGH 治疗无关。

结论

本研究表明,髋关节发育不良可能是 GHD 的一种表现。此外,rhGH 治疗不一定会导致 LCPD。

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