肌肉收缩性埃尔斯-丹洛斯综合征（mcEDS）患者大量皮下出血引起的低血容量性休克。

IF 2.1 4区医学 Q2 MEDICINE, GENERAL & INTERNAL

Wiener Klinische Wochenschrift Pub Date : 2025-08-04 DOI:10.1007/s00508-025-02577-9

R Laggner, S Payr, L Adam, D Baron, L Zak

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引用次数: 0

摘要

肌肉收缩性埃尔斯-丹洛斯综合征（mcEDS）是一种罕见的常染色体隐性结缔组织疾病，其特征是皮肤、脉管系统和肌肉骨骼结构的脆弱。我们报告一例年轻男性CHST14（碳水化合物硫转移酶14）相关的mcEDS，他在轻微创伤后出现大量皮下血肿，需要手术撤离。该病例强调了mcEDS可能出现危及生命的出血并发症，并强调了早期识别和多学科管理的重要性。

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Hypovolemic shock due to massive subcutaneous hemorrhage in a patient with musculocontractural Ehlers-Danlos syndrome (mcEDS).

Musculocontractural Ehlers-Danlos syndrome (mcEDS) is a rare autosomal recessive connective tissue disorder characterized by fragility of skin, vasculature and musculoskeletal structures. We report a case of a young male with CHST14(Carbohydrate sulfotransferase 14)-related mcEDS who developed a massive subcutaneous hematoma following minor trauma, necessitating surgical evacuation. This case highlights the potential for life-threatening bleeding complications in mcEDS and underscores the importance of early recognition and multidisciplinary management.

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来源期刊

Wiener Klinische Wochenschrift 医学-医学：内科

CiteScore

4.70

自引率

3.80%

发文量

110

审稿时长

4-8 weeks

期刊介绍： The Wiener klinische Wochenschrift - The Central European Journal of Medicine - is an international scientific medical journal covering the entire spectrum of clinical medicine and related areas such as ethics in medicine, public health and the history of medicine. In addition to original articles, the Journal features editorials and leading articles on newly emerging topics, review articles, case reports and a broad range of special articles. Experimental material will be considered for publication if it is directly relevant to clinical medicine. The number of international contributions has been steadily increasing. Consequently, the international reputation of the journal has grown in the past several years. Founded in 1888, the Wiener klinische Wochenschrift - The Central European Journal of Medicine - is certainly one of the most prestigious medical journals in the world and takes pride in having been the first publisher of landmarks in medicine.