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拉脱维亚儿科垂体功能减退症患者的特征:单中心 25 年回顾性研究。

Characteristics of paediatric hypopituitarism patients in Latvia: a single-centre 25-year retrospective study.

机构信息

Faculty of Medicine, Riga Stradins University, Latvia.

Department of Cariology, Academic Centre for Dentistry Amsterdam (ACTA), Vrije Universiteit Amsterdam and University of Amsterdam, Amsterdam, Netherlands.

出版信息

Pediatr Endocrinol Diabetes Metab. 2022;28(4):287-293. doi: 10.5114/pedm.2022.118320.

Abstract

INTRODUCTION

Hypopituitarism is a chronic disease characterized by partial or complete absence of secretion of one or more pituitary hormones. Delayed diagnosis can have serious consequences during the neonatal period and adulthood.

MATERIAL AND METHODS

A retrospective study was conducted on patients who had started treatment in the Children's Clinical University Hospital from 1 January 1995 to 31 December 2020. In total 243 patients were included; they were divided into 3 subgroups: isolated growth hormone deficiency, multiple pituitary hormone deficiency, and panhypopituitarism.

RESULTS

The prevalence of hypopituitarism in Latvia is about 45 cases per 100,000 live births. The average detection age of abnormal growth in Latvia is 8 years and 3 months. Most cases had isolated growth hormone deficiency, at 67.1% (n = 163), followed by cases of multiple pituitary hormone deficiency, at 26.3% (n = 64), and cases of panhypopituitarism, at 6.6% (n = 16). Abnormalities in MRI were found in 44.7% (n = 101) of patients. The most best therapeutic effect was achieved in the first year of treatment: with growth of an average of 9.3 cm (+0.1 SD) for isolated growth hormone deficiency, 9.0 cm (+0.6 SD) for multiple pituitary hormone deficiency, and 11.7 cm (+1SD) for patients with panhypopituitarism.

CONCLUSIONS

It is important to increase awareness and promote early diagnosis for hypopituitarism patients in Latvia and in Europe. More attention should be paid to education about growth restriction problems to parents, caregivers, and other specialists. The treatment should be adjusted individually with the focus not only on physical and mental health but also on safety and treatment costs.

摘要

引言

垂体功能减退症是一种慢性疾病,其特征是部分或完全缺乏一种或多种垂体激素的分泌。在新生儿期和成年期延迟诊断可能会产生严重后果。

材料和方法

对 1995 年 1 月 1 日至 2020 年 12 月 31 日期间在儿童临床大学医院开始治疗的患者进行了回顾性研究。共纳入 243 例患者;他们分为 3 个亚组:单纯生长激素缺乏症、多种垂体激素缺乏症和全垂体功能减退症。

结果

在拉脱维亚,垂体功能减退症的患病率约为每 10 万活产儿 45 例。拉脱维亚异常生长的平均检测年龄为 8 岁零 3 个月。大多数病例为单纯生长激素缺乏症,占 67.1%(n = 163),其次是多种垂体激素缺乏症,占 26.3%(n = 64),全垂体功能减退症占 6.6%(n = 16)。44.7%(n = 101)的患者 MRI 异常。在治疗的第一年取得了最佳的治疗效果:单纯生长激素缺乏症的平均生长为 9.3 厘米(+0.1 SD),多种垂体激素缺乏症为 9.0 厘米(+0.6 SD),全垂体功能减退症为 11.7 厘米(+1SD)。

结论

提高拉脱维亚和欧洲对垂体功能减退症患者的认识并促进早期诊断非常重要。应更加关注向家长、照顾者和其他专家宣传生长受限问题。治疗应根据个体进行调整,不仅要关注身心健康,还要关注安全性和治疗成本。

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