An audit of the management of childhood-onset growth hormone deficiency during young adulthood in Scotland.
An audit of the management of childhood-onset growth hormone deficiency during young adulthood in Scotland.
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DOI：
10.1186/s13633-016-0024-8
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发表时间：
2016
期刊：
影响因子：
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通讯作者：
Shaikh MG
中科院分区：
文献类型：
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作者：
Ahmid M;Fisher V;Graveling AJ;McGeoch S;McNeil E;Roach J;Bevan JS;Bath L;Donaldson M;Leese G;Mason A;Perry CG;Zammitt NN;Ahmed SF;Shaikh MG
患有儿童期发作的生长激素缺乏症（CO-GHD）的青少年需要在达到最终身高时重新评估其生长激素（GH）轴，以确定是否有资格接受成人GH治疗（rhGH）。回顾性多中心审查管理的年轻人与CO-GHD在苏格兰的四个儿科中心在过渡期间。对2005年至2013年期间达到最终身高的130名符合条件的CO-GHD青少年（78名男性）的病历进行了审查。首次诊断CO-GHD时的中位（范围）年龄为10.7岁（0.1-16.4），刺激GH峰值为2.3 μg/l（0.1-6.5）。开始rhGH治疗时的中位年龄为10.8岁（0.4-17.0岁）。在130例CO-GHD青少年中，74/130例（57%）通过刺激试验/IGF-1测量进行了GH轴重新评估。其中，61/74（82%）仍为GHD，51/74（69%）重新开始成人rhGH。持续性GHD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症（MPHD）。在其余56/130例（43%）未进行复检的患者中，34/56例（61%）在未进行生化复检的情况下转入成人服务，其中32/34例患有MPHD。在四个中心中，未进行生化复检而提供rhGH的成人比例在其总队列的10%至50%之间。相当一部分患有CO-GHD的成年人仍然是GHD，特别是那些患有MPHD的人，大多数人选择用rhGH治疗。尽管有临床指南，但在苏格兰的年轻人中，CO-GHD的管理存在显着差异。
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.