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马凡综合征、洛伊茨-迪茨综合征和胶原病中的心肌和瓣膜受累。

The Heart Muscle and Valve Involvement in Marfan Syndrome, Loeys-Dietz Syndromes, and Collagenopathies.

机构信息

Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Naples 80131, Italy.

Grupo de Enfermedades Cardiovasculares, Vall d'Hebron Institut de Recerca (VHIR), Barcelona, Spain.

出版信息

Heart Fail Clin. 2022 Jan;18(1):165-175. doi: 10.1016/j.hfc.2021.07.007. Epub 2021 Oct 25.

Abstract

The inherited connective tissue disorders (Marfan syndrome, Loeys-Dietz syndrome [LDS], and Ehlers-Danlos syndrome [EDS]) involve connective tissue of various organ systems. These pathologies share many common features, nonetheless compared to Marfan syndrome, LDS' cardiovascular manifestations tend to be more severe. In contrast, no association is reported between LDS and the presence of ectopia lentis. The EDS are currently classified into thirteen subtypes. There is substantial symptoms overlap between the EDS subtypes, and they are associated with an increased incidence of cardiovascular abnormalities, such as mitral valve prolapse and aortic dissection.

摘要

遗传性结缔组织疾病(马凡综合征、Loeys-Dietz 综合征[LDS]和埃勒斯-当洛斯综合征[EDS])涉及各种器官系统的结缔组织。这些病理具有许多共同特征,但与马凡综合征相比,LDS 的心血管表现往往更为严重。相比之下,LDS 与晶状体异位之间并无关联。EDS 目前分为十三种亚型。EDS 亚型之间存在大量症状重叠,并且与心血管异常的发生率增加有关,如二尖瓣脱垂和主动脉夹层。

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