Suppr超能文献

青春期前先天性肾上腺皮质增生症患儿的反向昼夜糖皮质激素治疗。

Reverse circadian glucocorticoid treatment in prepubertal children with congenital adrenal hyperplasia.

机构信息

Division for Pediatric Endocrinology, Dr. von Hauner Children's Hospital, University Hospital, LMU Munich, Munich, Germany.

Department of Medicine IV, University Hospital, LMU Munich, Munich, Germany.

出版信息

J Pediatr Endocrinol Metab. 2021 Sep 15;34(12):1543-1548. doi: 10.1515/jpem-2021-0540. Print 2021 Dec 20.

Abstract

OBJECTIVES

Children with salt-wasting congenital adrenal hyperplasia (CAH) have an impaired function of steroid synthesis pathways. They require therapy with glucocorticoid (GC) and mineralocorticoid hormones to avoid salt-wasting crisis and other complications. Most commonly, children receive hydrocortisone thrice daily with the highest dose in the morning, mimicking the regular physiology. However, reverse circadian treatment (RCT) had been suggested previously. In this study, we aimed to determine the efficacy of RCT in prepubertal children with CAH by comparing the salivary 17-hydroxyprogesterone (s17-OHP) levels individually.

METHODS

In this retrospective study, we analyzed the records of children with classical CAH and RCT who were monitored by s17-OHP levels. The study included 23 patients. We identified nine prepubertal children with RCT schemes (three boys and six girls) and compared the s17-OHP levels in the morning, afternoon, and evening. The objective of this study was to demonstrate the non-effectiveness of RCT in terms of lowering the morning s17-OHP concentration. In addition, we compared s17-OHP day profiles in six patients on RCT and non-RCT therapy (intraindividually).

RESULTS

Eight of nine children with RCT showed higher s17-OHP levels in the morning compared to the evening. In addition, none of the children showed a significant deviation of development. Three children were overweight. No adrenal crisis or pubertal development occurred. Comparison of RCT and non-RCT regimens showed no difference in 17-OHP profiles.

CONCLUSIONS

Our data do not support the use of RCT schemes for GC replacement in children with CAH due to lack of benefits and unknown long-term risks.

摘要

目的

患有失盐型先天性肾上腺皮质增生症(CAH)的儿童其类固醇合成途径的功能受损。为避免盐耗竭危象和其他并发症,他们需要接受糖皮质激素(GC)和盐皮质激素的治疗。最常见的治疗方法是每日三次给予氢化可的松,且早上的剂量最高,以模拟正常的生理节律。然而,此前曾提出过反向昼夜节律治疗(RCT)。在这项研究中,我们旨在通过单独比较唾液 17-羟孕酮(s17-OHP)水平,来确定 RCT 在患有 CAH 的青春期前儿童中的疗效。

方法

在这项回顾性研究中,我们分析了接受 s17-OHP 水平监测的患有经典 CAH 和 RCT 的儿童的记录。该研究共纳入了 23 名患者。我们确定了九名采用 RCT 方案的青春期前儿童(3 名男孩和 6 名女孩),并比较了他们早上、下午和晚上的 s17-OHP 水平。本研究的目的是证明 RCT 在降低早上 s17-OHP 浓度方面无效。此外,我们还比较了六名接受 RCT 和非 RCT 治疗(个体内)的患者的 s17-OHP 日谱。

结果

九名采用 RCT 方案的儿童中有八名在早上的 s17-OHP 水平高于晚上。此外,没有儿童出现明显的发育偏差。三名儿童超重。没有发生肾上腺危象或青春期发育。RCT 和非 RCT 方案的比较显示,17-OHP 谱没有差异。

结论

由于缺乏获益且未知长期风险,我们的数据不支持在 CAH 儿童中使用 RCT 方案来替代 GC。

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验