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 singleorgan 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.

Thoracic involvement in generalised lymphatic anomaly (or lymphangiomatosis) / F. Luisi, O. Torre, S. Harari. - In: EUROPEAN RESPIRATORY REVIEW. - ISSN 0905-9180. - 25:140(2016), pp. 170-177. [10.1183/16000617.0018-2016]

Thoracic involvement in generalised lymphatic anomaly (or lymphangiomatosis)

S. Harari
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 singleorgan 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.
Biopsy; Diagnosis, Differential; Disease Progression; Humans; Lung Diseases; Lymphangiectasis; Palliative Care; Predictive Value of Tests; Prognosis; Risk Factors; Tomography, X-Ray Computed; Rare Diseases
Settore MED/10 - Malattie dell'Apparato Respiratorio
2016
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/749199
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