Lymphangioléiomyomatose pulmonaire [Lymphangioleiomyomatosis]

Détails

ID Serval
serval:BIB_871D3B4F3E8C
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Synthèse (review): revue aussi complète que possible des connaissances sur un sujet, rédigée à partir de l'analyse exhaustive des travaux publiés.
Collection
Publications
Titre
Lymphangioléiomyomatose pulmonaire [Lymphangioleiomyomatosis]
Périodique
Presse Médicale
Auteur(s)
Cottin Vincent, Archer Fabienne, Khouatra Chahera, Lazor Romain, Cordier Jean-Francois
ISSN
0755-4982
Statut éditorial
Publié
Date de publication
2010
Peer-reviewed
Oui
Volume
39
Numéro
1
Pages
116-125
Langue
français
Notes
Publication types: English Abstract ; Journal Article Publication Status: ppublish
Résumé
Lymphangioleiomyomatosis is a rare pulmonary disease encountered almost exclusively in women of reproductive age. Pulmonary involvement is characterized by multiple thin-walled cysts in the lungs, recurrent pneumothorax, obstructive lung disorders, and progression to chronic respiratory failure over a mean period of 10 years. Certainty of diagnosis requires a lung biopsy, but international criteria have been proposed for a diagnosis without such a biopsy. International recommendations were recently issued for the diagnosis and treatment of lymphangioleiomyomatosis. Treatment is principally symptomatic and relies on the management of bronchial obstruction by bronchodilators; of hypoxemia by oxygen therapy; of pleural complications by pleurodesis, most often surgical; and of renal angiomyolipomas by percutaneous embolization in cases of hemorrhagic risk. Hormone treatment is not recommended. Hopes are high for mTor inhibitors (sirolimus and everolimus) and treatment trials are currently underway. Lung transplantation must be considered when chronic respiratory failure occurs in patients younger than 60 years.
Mots-clé
Tuberous Sclerosis Complex, Pulmonary Lymphangioleiomyomatosis, Lung Transplantation, Air-Travel, Gene TSC2, Disease, Cells, Women, CT, Pneumothorax
Pubmed
Web of science
Création de la notice
06/02/2010 19:40
Dernière modification de la notice
03/03/2018 19:01
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