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重症系统性红斑狼疮不寻常且独特的初始表现并存:一例儿童病例,表现为与自身免疫性多内分泌腺综合征相关的肾上腺功能不全。

Coexistence of unusual and distinctive initial manifestations of severe systemic lupus erythematosus: A child's case, presenting as adrenal insufficiency associated with autoimmune polyglandular syndrome.

作者信息

Karaoglan M

机构信息

Department of Pediatric Endocrinology, Faculty of Medicine, Gaziantep University, Gaziantep, Turkey.

出版信息

Niger J Clin Pract. 2019 Dec;22(12):1781-1784. doi: 10.4103/njcp.njcp_667_18.

DOI:10.4103/njcp.njcp_667_18
PMID:31793489
Abstract

The involvement of multiple endocrine organs is rarely identified as initial manifestations of systemic lupus erythematosus (SLE) and autoimmune polyglandular syndrome (APS) in infancy. Childhood SLE tends to present with more severe clinical symptoms at an early age and with a set of unexpected findings. In the literature, the case reports of children presenting with SLE and APS components at the same time are extremely rare. Adrenal insufficiency may be overlooked due to mild clinical findings in later periods, except for neonates characterized by marked salt depletion and ambiguous genitalia signs. Moreover, adrenal insufficiency as an initial symptom in childhood is quite unusual as a component of SLE-associated APS. This report describes the overlap of unexpected, unusual, and severe clinical findings in an infant with APS with a comorbidity of SLE, where the involvement of multiple endocrine organs coexists with multiple serious clinical manifestations from the beginning.

摘要

在婴儿期,多内分泌器官受累很少被视为系统性红斑狼疮(SLE)和自身免疫性多内分泌腺综合征(APS)的初始表现。儿童期SLE往往在早期就表现出更严重的临床症状,并伴有一系列意想不到的发现。在文献中,儿童同时出现SLE和APS成分的病例报告极为罕见。除了以明显的盐耗竭和生殖器模糊体征为特征的新生儿外,肾上腺功能不全后期可能因临床表现轻微而被忽视。此外,肾上腺功能不全作为儿童期SLE相关APS的初始症状相当罕见。本报告描述了一名患有APS并合并SLE的婴儿中意外、不寻常和严重临床发现的重叠情况,其中多内分泌器官受累从一开始就与多种严重临床表现并存。

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