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特纳综合征女孩在接受人生长激素促生长治疗期间的肾上腺功能初现和阴毛发育

Adrenarche and pubarche in girls with turner syndrome during growth-promoting therapy with human growth hormone.

作者信息

Dörr Helmuth G, Penger Theresa, Marx Michaela, Rauh Manfred, Oppelt Patricia G, Völkl Thomas K M

机构信息

Paediatric Endocrinology, University Hospital of Erlangen, Loschgestr. 15, 91054, Erlangen, Germany.

Department of Gynaecology and Obstetrics, University Hospital of Erlangen, Loschgestr. 15, 91054, Erlangen, Germany.

出版信息

BMC Endocr Disord. 2019 Jan 18;19(1):9. doi: 10.1186/s12902-019-0333-z.

Abstract

BACKGROUND

Data on adrenarche and pubarche in girls with Turner syndrome (TS) are inconsistent in the literature.

METHODS

The cohort consisted of 94 girls and young women with TS born between 1971 and 2001 (age range: 3.1-23.2 yrs.), who were treated with human growth hormone and regularly presented at our outpatient clinic every 4 to 6 months.The longitudinal data of all patients were ascertained retrospectively from patient charts. The data collection ended in January 2016. Adrenarche was assessed by serum DHEAS levels and pubertal status by Tanner stages. Pubarche was defined as the appearance of pubic hair (PH2), whereas spontaneous puberty was defined as Tanner stage B2. The patients were retrospectively subdivided in two groups with regard to pubertal development: group 1 (n = 21) with spontaneous puberty and group 2 (n = 70) with induced puberty. Since blood samples were not taken at every visit, we generated seven groups according to the age of the children at which the blood samples were taken: 3-5, 5-7, 7-9, 9-11, 11-13, 13-15, and 15-17 yrs. Serum DHEAS and follicle-stimulating hormone (FSH) levels were measured by chemiluminescence immunoassay and compared with those of a control group of healthy girls.

RESULTS

Adrenarche started in TS girls between 5 and 7 years. TS girls had higher DHEAS levels than the control group, with statistically significant differences in the age groups 7 to 17 years. No differences were determined between the TS girls with spontaneous puberty and those with POI. TS girls in group 2 reached the Tanner stages PH2 (p < 0.04), PH3 (p < 0.01), PH4 and PH5 (p < 0.001) markedly later than TS girls in group 1.

CONCLUSIONS

The onset of adrenarche in girls with TS undergoing GH therapy does not differ from that in healthy girls. However, adrenarche is more pronounced in girls with TS. There is no difference in DHEAS levels between the TS girls with spontaneous puberty and the TS girls with primary ovarian insufficiency (POI), while the tempo of pubarche is markedly slower in the girls with POI.

摘要

背景

关于特纳综合征(TS)女孩肾上腺初现和阴毛初现的数据在文献中并不一致。

方法

该队列包括94名1971年至2001年出生的患有TS的女孩和年轻女性(年龄范围:3.1 - 23.2岁),她们接受了人生长激素治疗,并每4至6个月定期到我们的门诊就诊。所有患者的纵向数据均从病历中回顾性确定。数据收集于2016年1月结束。通过血清硫酸脱氢表雄酮(DHEAS)水平评估肾上腺初现,通过坦纳分期评估青春期状态。阴毛初现定义为阴毛出现(PH2),而自然青春期定义为坦纳B2期。根据青春期发育情况,将患者回顾性分为两组:第1组(n = 21)为自然青春期组,第2组(n = 70)为诱导青春期组。由于并非每次就诊都采集血样,我们根据采血时儿童的年龄将其分为七组:3 - 5岁、5 - 7岁、7 - 9岁、9 - 11岁、11 - 13岁、13 - 15岁和15 - 17岁。通过化学发光免疫分析法测量血清DHEAS和促卵泡生成素(FSH)水平,并与健康女孩对照组进行比较。

结果

TS女孩的肾上腺初现始于5至7岁。TS女孩的DHEAS水平高于对照组,在7至17岁年龄组中差异具有统计学意义。自然青春期的TS女孩与原发性卵巢功能不全(POI)的TS女孩之间未发现差异。第2组的TS女孩达到坦纳分期PH2(p < 0.04)、PH3(p < 0.01)、PH4和PH5(p < 0.001)的时间明显晚于第1组的TS女孩。

结论

接受生长激素治疗的TS女孩的肾上腺初现起始与健康女孩无异。然而,TS女孩的肾上腺初现更为明显。自然青春期的TS女孩与原发性卵巢功能不全(POI)的TS女孩的DHEAS水平没有差异,而POI女孩的阴毛初现节奏明显较慢。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/000a/6339350/6eb73c759175/12902_2019_333_Fig1_HTML.jpg

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