Data di Pubblicazione:
2017
Citazione:
New insights in lymphangioleiomyomatosis and pulmonary langerhans cell histiocytosis / O. Torre, D. Elia, A. Caminati, S. Harari. - In: EUROPEAN RESPIRATORY REVIEW. - ISSN 0905-9180. - 26:145(2017), pp. 170042.1-170042.13. [10.1183/16000617.0042-2017]
Abstract:
Lymphangioleiomyomatosis (LAM) and pulmonary Langerhans cell histiocytosis (PLCH) are rare diseases that lead to progressive cystic destruction of the lungs. Despite their distinctive characteristics, these diseases share several features. Patients affected by LAM or PLCH have similar radiological cystic patterns, a similar age of onset, and the possibility of extrapulmonary involvement. In this review, the recent advances in the understanding of the molecular pathogenesis, as well as the current and most promising biomarkers and therapeutic approaches, are described.
Tipologia IRIS:
01 - Articolo su periodico
Keywords:
Animals; Diagnosis, Differential; Genetic Markers; Genetic Predisposition to Disease; Humans; Molecular Targeted Therapy; Phenotype; Predictive Value of Tests; Protein Kinase Inhibitors; Proto-Oncogene Proteins B-raf; Respiratory Function Tests; Risk Factors; Signal Transduction; TOR Serine-Threonine Kinases; Tomography, X-Ray Computed; Histiocytosis, Langerhans-Cell; Lung; Lymphangioleiomyomatosis
Elenco autori:
O. Torre, D. Elia, A. Caminati, S. Harari
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