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儿科急诊中肾上腺功能不全的管理及肾上腺危象发生的危险因素。

Adrenal insufficiency management in the pediatric emergency setting and risk factors for adrenal crisis development.

机构信息

Department of Pediatric Endocrinology, Regina Margherita Children's Hospital, Piazza Polonia 94, Turin, 10126, Italy.

Postgraduate School of Pediatrics, University of Turin, Turin, Italy.

出版信息

Ital J Pediatr. 2023 Jun 6;49(1):63. doi: 10.1186/s13052-023-01475-y.

DOI:10.1186/s13052-023-01475-y
PMID:37280667
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10242818/
Abstract

BACKGROUND

In patients with adrenal insufficiency (AI), adrenal crisis (AC) represents a clinical emergency. Early recognition and prompt management of AC or AC-risk conditions in the Emergency Department (ED) can reduce critical episodes and AC-related outcomes. The aim of the study is to report the clinical and biochemical characteristics of AC presentation to improve their timely recognition and proper management in a ED setting.

METHODS

Single-centre, retrospective, observational study on pediatric patients followed at the Department of Pediatric Endocrinology of Regina Margherita Children's Hospital of Turin for primary AI (PAI) and central AI (CAI).

RESULTS

Among the 89 children followed for AI (44 PAI, 45 CAI), 35 patients (21 PAI, 14 CAI) referred to the PED, for a total of 77 accesses (44 in patients with PAI and 33 with CAI). The main causes of admission to the PED were gastroenteritis (59.7%), fever, hyporexia or asthenia (45.5%), neurological signs and respiratory disorders (33.8%). The mean sodium value at PED admission was 137.2 ± 1.23 mmol/l and 133.3 ± 1.46 mmol/l in PAI and CAI, respectively (p = 0.05). Steroids administration in PED was faster in patients with CAI than in those with PAI (2.75 ± 0.61 and 3.09 ± 1.47 h from PED access, p = 0.83). Significant factors related to the development of AC were signs of dehydration at admission (p = 0.027) and lack of intake or increase of usual steroid therapy at home (p = 0.059). Endocrinological consulting was requested in 69.2% of patients with AC and 48.4% of subjects without AC (p = 0.032).

CONCLUSION

children with AI may refer to the PED with an acute life-threatening condition that needs prompt recognition and management. These preliminary data indicate how critical the education of children and families with AI is to improve the management at home, and how fundamental the collaboration of the pediatric endocrinologist with all PED personnel is in raising awareness of early symptoms and signs of AC to anticipate the proper treatment and prevent or reduce the correlated serious events.

摘要

背景

在肾上腺功能不全(AI)患者中,肾上腺危象(AC)代表一种临床急症。在急诊科(ED)中早期识别和及时处理 AC 或 AC 风险情况可减少危急发作和 AC 相关结局。本研究旨在报告 AC 表现的临床和生化特征,以提高 ED 环境中对其的及时识别和适当管理。

方法

这是一项针对在都灵 Regina Margherita 儿童医院小儿内分泌科就诊的原发性 AI(PAI)和中枢性 AI(CAI)儿童患者的单中心、回顾性、观察性研究。

结果

在 89 名 AI 患儿(44 名 PAI,45 名 CAI)中,35 名患儿(21 名 PAI,14 名 CAI)因总计 77 次就诊至 PED(44 名 PAI 患儿,33 名 CAI 患儿)。患儿就诊 PED 的主要原因是胃肠炎(59.7%)、发热、食欲不振或乏力(45.5%)、神经症状和呼吸障碍(33.8%)。在 PED 就诊时,PAI 和 CAI 患儿的平均血清钠值分别为 137.2±1.23mmol/L 和 133.3±1.46mmol/L(p=0.05)。与 PAI 患儿相比,CAI 患儿在 PED 中接受类固醇治疗的速度更快(就诊至开始类固醇治疗的时间分别为 2.75±0.61 和 3.09±1.47h,p=0.83)。发生 AC 的相关显著因素为就诊时存在脱水体征(p=0.027)和在家中未摄入或增加常规类固醇治疗(p=0.059)。69.2%的 AC 患儿和 48.4%的非 AC 患儿请求内分泌科会诊(p=0.032)。

结论

AI 患儿可能因危及生命的急性疾病就诊于 PED,需要快速识别和处理。这些初步数据表明,对 AI 患儿及其家庭进行教育以改善家庭管理至关重要,儿科内分泌医生与所有 PED 人员的合作对于提高对 AC 早期症状和体征的认识、及时进行适当治疗以及预防或减少相关严重事件也非常重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/80af/10242818/007a404a069f/13052_2023_1475_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/80af/10242818/007a404a069f/13052_2023_1475_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/80af/10242818/007a404a069f/13052_2023_1475_Fig1_HTML.jpg

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Real-World Estimates of Adrenal Insufficiency-Related Adverse Events in Children With Congenital Adrenal Hyperplasia.现实世界中先天性肾上腺皮质增生症患儿肾上腺皮质功能不全相关不良事件的估计。
J Clin Endocrinol Metab. 2021 Jan 1;106(1):e192-e203. doi: 10.1210/clinem/dgaa694.
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Etiology of primary adrenal insufficiency in children: a 29-year single-center experience.儿童原发性肾上腺皮质功能减退症的病因:29年单中心经验
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