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
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.
Int J Pediatr Endocrinol. 2016;2016:6. doi: 10.1186/s13633-016-0024-8. Epub 2016 Mar 16.
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).
Retrospective multicentre review of management of young adults with CO-GHD in four paediatric centres in Scotland during transition.
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).
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.
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.
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。
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。
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)。
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。
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。