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一项关于幼年系统性红斑狼疮生长和青春期的纵向 PRINTO 研究。

A longitudinal PRINTO study on growth and puberty in juvenile systemic lupus erythematosus.

机构信息

Faculty of medicine, Department of Laboratory Medicine, Children’s and Women’s Health, Norwegian University of Science and Technology, Trondheim, Norway.

出版信息

Ann Rheum Dis. 2012 Apr;71(4):511-7. doi: 10.1136/annrheumdis-2011-200106. Epub 2011 Oct 13.

Abstract

OBJECTIVE

To obtain longitudinal data on growth/puberty in a large-scale, multi-national prospective cohort of juvenile systemic lupus erythematosus (SLE).

METHODS

Data from 331/557 (59.4%) patients ≤18 years old with juvenile SLE in active phase, with anthropometric data available at four follow-up visits, were studied.

RESULTS

There was a significant reduction in parent-adjusted height z score with time in females and males (p<0.0001), with a significant gender difference (p<0.0001) and with male height being most affected. Median body mass index z score peaked at 6 months and was still significantly above baseline after 26 months (p<0.01), with no gender difference. Standardised height reduction was inversely related to age at onset. Females with onset age <12 years had a median parent-adjusted height z score of -0.87 with no catch-up growth. At the end of the study, growth failure was seen in 14.7% of the females and 24.5% of the males. Height deflection (less than -0.25/year) was found in 20.7% of the females and 45.5% of the males. Delayed pubertal onset was seen in 15.3% and 24% of the females and males, respectively, and delayed/absent menarche was seen in 21.9%, while 36.1% of the females and 44% of the males had some degree of delayed pubertal development. Growth failure baseline determinants were previous growth failure (OR: 56.6), age at first visit ≤13.4 years (OR: 4.2) and cumulative steroid dose >426 mg/kg (OR: 3.6).

CONCLUSIONS

The children at risk of having a negative effect on height and pubertal development are prepubertal and peripubertal children treated with >400 mg/kg cumulative dose of corticosteroids.

摘要

目的

获取大规模、多国家幼年特发性系统性红斑狼疮(SLE)前瞻性队列中生长/青春期的纵向数据。

方法

研究了 331/557(59.4%)名处于活动期的≤18 岁幼年 SLE 患者的数据,这些患者有 4 次随访时的人体测量数据。

结果

女性和男性的家长调整身高 z 评分随时间显著降低(p<0.0001),存在显著的性别差异(p<0.0001),且男性身高受影响最大。中位体质指数 z 评分在 6 个月时达到峰值,26 个月后仍显著高于基线(p<0.01),无性别差异。标准化身高降低与发病年龄呈反比。发病年龄<12 岁的女性中位家长调整身高 z 评分为-0.87,没有追赶生长。研究结束时,14.7%的女性和 24.5%的男性出现生长不良。女性中身高偏离(<0.25/年)为 20.7%,男性为 45.5%。分别有 15.3%和 24%的女性和男性出现青春期延迟,分别有 21.9%和 44%的女性和男性出现初潮延迟/缺失,而 36.1%的女性和 44%的男性存在不同程度的青春期发育延迟。生长不良的基线决定因素是既往生长不良(OR:56.6)、首次就诊时年龄≤13.4 岁(OR:4.2)和累积类固醇剂量>426mg/kg(OR:3.6)。

结论

有身高和青春期发育受负面影响风险的儿童是接受>400mg/kg 累积剂量皮质类固醇治疗的青春期前和青春期儿童。

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