Data di Pubblicazione:
2015
Citazione:
The changing face of a rare disease: Lymphangioleiomyomatosis / S. Harari, O. Torre, R. Cassandro, J. Moss. - In: EUROPEAN RESPIRATORY JOURNAL. - ISSN 0903-1936. - 46:5(2015), pp. 1471-1485. [10.1183/13993003.00412-2015]
Abstract:
Lymphangioleiomyomatosis is a rare disease characterised by cystic destruction of the lung, lymphatic abnormalities and abdominal tumours. It affects almost exclusively females and can occur sporadically or in patients with tuberous sclerosis complex. In the past decade remarkable progress has been made in understanding of the pathogenesis of this disease leading to a new therapeutic approach. This review summarises recent advances regarding pathogenic mechanisms and clinical manifestations, and highlights the current and the most promising future therapeutic strategies.
Tipologia IRIS:
01 - Articolo su periodico
Elenco autori:
S. Harari, O. Torre, R. Cassandro, J. Moss
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