神经分泌功能障碍治疗后的成人身高与特发性 GHD 的比较。

Adult height after treatment of neurosecretory dysfunction and comparison to idiopathic GHD.

机构信息

University-Children's Hospital Tübingen, Pediatric Endocrinology, Tübingen, Germany.

出版信息

Clin Endocrinol (Oxf). 2022 Feb;96(2):184-189. doi: 10.1111/cen.14608. Epub 2021 Oct 13.

DOI:10.1111/cen.14608

PMID:34647318

Abstract

OBJECTIVE

Neurosecretory dysfunction (NSD) causes growth hormone deficiency (GHD). Data on adult height after recombinant human growth hormone (rhGH) treatment are lacking.

DESIGN AND PATIENTS

We collected treatment data of all patients with NSD seen between 1990 and 2017 at our outpatient department (tertiary centre) and measured adult height. For comparison, patients with idiopathic GHD were used. Diagnoses were based on short stature (<-2 standard deviation score [SDS]), continuously low height velocity (<25th percentile), delayed bone age (by >1 SD) and low serum IGF-1 concentration (<-2 SDS). NSD was defined by normal GH challenge results, but subnormal spontaneous GH secretion. Exclusion criteria were no information on adult height, underweight and other short stature disorders.

RESULTS

Out of 67 patients diagnosed with NSD, six were still growing, 31 had test results exceeding validated GH cut-offs and three had other disorders causing short stature. Out of the 25 eligible patients with NSD, 21 could be recruited. These patients reached an adult height of -0.85 SDS (mean); 0.34 SDS below midparental height. Height gain during treatment was 2.01 SDS. This outcome was not different to 32 patients with idiopathic GHD.

CONCLUSIONS

Long-term results suggest the viability of the diagnosis of NSD and the efficacy of rhGH treatment.

摘要

目的

神经分泌功能障碍（NSD）可导致生长激素缺乏（GHD）。缺乏关于 rhGH 治疗后成人身高的数据。

设计和患者

我们收集了 1990 年至 2017 年期间在我们门诊部（三级中心）就诊的所有 NSD 患者的治疗数据，并测量了成人身高。为了进行比较，使用了特发性 GHD 患者。诊断基于身材矮小（<-2 个标准差评分 [SDS]）、持续身高增长缓慢（<25 百分位）、骨龄延迟（>1 SDS）和血清 IGF-1 浓度低（<-2 SDS）。NSD 的定义是 GH 激发试验结果正常，但自发性 GH 分泌不足。排除标准为无成人身高信息、体重不足和其他身材矮小疾病。

结果

在诊断为 NSD 的 67 名患者中，有 6 名仍在生长，31 名患者的测试结果超过了验证的 GH 截止值，3 名患者患有其他导致身材矮小的疾病。在 25 名符合条件的 NSD 患者中，有 21 名能够入组。这些患者的成人身高为-0.85 SDS（平均值）；比中亲身高低 0.34 SDS。治疗期间的身高增长为 2.01 SDS。这一结果与 32 名特发性 GHD 患者没有差异。