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广泛性淋巴管异常(或淋巴管瘤病)的胸部受累情况。

Thoracic involvement in generalised lymphatic anomaly (or lymphangiomatosis).

作者信息

Luisi Francesca, Torre Olga, Harari Sergio

机构信息

Unità Operativa di Pneumologia e Terapia Semi-Intensiva Respiratoria, Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe, Multimedica IRCCS, Milan, Italy.

Unità Operativa di Pneumologia e Terapia Semi-Intensiva Respiratoria, Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe, Multimedica IRCCS, Milan, Italy

出版信息

Eur Respir Rev. 2016 Jun;25(140):170-7. doi: 10.1183/16000617.0018-2016.

Abstract

Generalised lymphatic anomaly (GLA), also known as lymphangiomatosis, is a rare disease caused by congenital abnormalities of lymphatic development. It usually presents in childhood but can also be diagnosed in adults. GLA encompasses a wide spectrum of clinical manifestations ranging from single-organ involvement to generalised disease. Given the rarity of the disease, most of the information regarding it comes from case reports. To date, no clinical trials concerning treatment are available. This review focuses on thoracic GLA and summarises possible diagnostic and therapeutic approaches.

摘要

广泛性淋巴管异常(GLA),也称为淋巴管瘤病,是一种由淋巴管发育先天性异常引起的罕见疾病。它通常在儿童期出现,但也可在成人中诊断出来。GLA涵盖了从单器官受累到全身性疾病的广泛临床表现。鉴于该疾病的罕见性,关于它的大多数信息来自病例报告。迄今为止,尚无关于治疗的临床试验。本综述聚焦于胸部GLA,并总结了可能的诊断和治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f917/9487238/588b0484f4f6/ERR-0018-2016.01.jpg

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