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苏格兰青少年期起病的生长激素缺乏症成年期管理情况审计。

An audit of the management of childhood-onset growth hormone deficiency during young adulthood in Scotland.

作者信息

Ahmid M, Fisher V, Graveling A J, McGeoch S, McNeil E, Roach J, Bevan J S, Bath L, Donaldson M, Leese G, Mason A, Perry C G, Zammitt N N, Ahmed S F, Shaikh M G

机构信息

Developmental Endocrinology Research Group, Royal Hospital for Children, School of Medicine, University of Glasgow, 1345 Govan Road, Glasgow, G51 4TF UK.

JJR Macleod Centre for Diabetes, Endocrinology & Metabolism, Aberdeen Royal Infirmary, Aberdeen, UK.

出版信息

Int J Pediatr Endocrinol. 2016;2016:6. doi: 10.1186/s13633-016-0024-8. Epub 2016 Mar 16.

Abstract

BACKGROUND

Adolescents with childhood onset growth hormone deficiency (CO-GHD) require re-evaluation of their growth hormone (GH) axis on attainment of final height to determine eligibility for adult GH therapy (rhGH).

AIM

Retrospective multicentre review of management of young adults with CO-GHD in four paediatric centres in Scotland during transition.

PATIENTS

Medical records of 130 eligible CO-GHD adolescents (78 males), who attained final height between 2005 and 2013 were reviewed. Median (range) age at initial diagnosis of CO-GHD was 10.7 years (0.1-16.4) with a stimulated GH peak of 2.3 μg/l (0.1-6.5). Median age at initiation of rhGH was 10.8 years (0.4-17.0).

RESULTS

Of the 130 CO-GHD adolescents, 74/130(57 %) had GH axis re-evaluation by stimulation tests /IGF-1 measurements. Of those, 61/74 (82 %) remained GHD with 51/74 (69 %) restarting adult rhGH. Predictors of persistent GHD included an organic hypothalamic-pituitary disorder and multiple pituitary hormone deficiencies (MPHD). Of the remaining 56/130 (43 %) patients who were not re-tested, 34/56 (61 %) were transferred to adult services on rhGH without biochemical retesting and 32/34 of these had MPHD. The proportion of adults who were offered rhGH without biochemical re-testing in the four centres ranged between 10 and 50 % of their total cohort.

CONCLUSIONS

A substantial proportion of adults with CO-GHD remain GHD, particularly those with MPHD and most opt for treatment with rhGH. Despite clinical guidelines, there is significant variation in the management of CO-GHD in young adulthood across Scotland.

摘要

背景

患有儿童期起病的生长激素缺乏症(CO-GHD)的青少年在达到最终身高时需要重新评估其生长激素(GH)轴,以确定是否符合成人生长激素治疗(重组人生长激素,rhGH)的条件。

目的

对苏格兰四个儿科中心在过渡期间患有CO-GHD的年轻成年人的管理进行回顾性多中心研究。

患者

回顾了130例符合条件的CO-GHD青少年(78例男性)的病历,这些青少年在2005年至2013年间达到了最终身高。CO-GHD初始诊断时的中位(范围)年龄为10.7岁(0.1 - 16.4岁),刺激后GH峰值为2.3μg/l(0.1 - 6.5μg/l)。开始使用rhGH的中位年龄为10.8岁(0.4 - 17.0岁)。

结果

在130例CO-GHD青少年中,74/130(57%)通过刺激试验/IGF-1测量对GH轴进行了重新评估。其中,61/74(82%)仍为生长激素缺乏,51/74(69%)重新开始使用成人rhGH。持续性生长激素缺乏的预测因素包括器质性下丘脑 - 垂体疾病和多种垂体激素缺乏(MPHD)。在其余56/130(43%)未重新检测的患者中,34/56(61%)在未进行生化重新检测的情况下被转至成人服务机构接受rhGH治疗,其中32/34患有MPHD。在这四个中心,未进行生化重新检测就接受rhGH治疗的成年人比例在其总队列的10%至50%之间。

结论

相当一部分患有CO-GHD的成年人仍存在生长激素缺乏,尤其是那些患有MPHD的人,并且大多数选择rhGH治疗。尽管有临床指南,但苏格兰各地在年轻成年人CO-GHD的管理方面存在显著差异。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dcd6/4793498/696a61575da6/13633_2016_24_Fig1_HTML.jpg

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