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Thoracic involvement in generalised lymphatic anomaly (or lymphangiomatosis)

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-or...

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Detalles Bibliográficos
Autores principales: Luisi, Francesca, Torre, Olga, Harari, Sergio
Formato: Online Artículo Texto
Lenguaje:English
Publicado: European Respiratory Society 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9487238/
https://www.ncbi.nlm.nih.gov/pubmed/27246594
http://dx.doi.org/10.1183/16000617.0018-2016
Descripción
Sumario: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.