Genetic diffuse cystic lung disease in adults
Rev Mal Respir. 2023 Nov 9:S0761-8425(23)00264-4. doi: 10.1016/j.rmr.2023.08.005. Online ahead of print.ABSTRACTMultiple cystic lung diseases comprise a wide range of various diseases, some of them of genetic origin. Lymphangioleiomyomatosis (LAM) is a disease occurring almost exclusively in women, sporadically or in association with tuberous sclerosis complex (TSC). Patients with LAM present with lymphatic complications, renal angiomyolipomas and cystic lung disease responsible for spontaneous pneumothoraces and progressive respiratory insufficiency. TSC and LAM have been ascribed to mutations in TSC1 or TSC2 genes. Patie...
Source: Revue des Maladies Respiratoires - November 11, 2023 Category: Respiratory Medicine Authors: R Diesler K Ahmad L Chalabreysse J-C Gl érant I Harzallah R Touraine S Si-Mohamed V Cottin Source Type: research

Bronchial diseases and heroin use. A systematic review
CONCLUSION: Bronchial diseases in heroin users can be identified by means of respiratory function exploration and chest CT scans. These tests should be performed frequently in view of optimizing their care, which includes their weaning themselves from addictive substances.PMID:37925326 | DOI:10.1016/j.rmr.2023.09.006 (Source: Revue des Maladies Respiratoires)
Source: Revue des Maladies Respiratoires - November 4, 2023 Category: Respiratory Medicine Authors: M Underner J Perriot G Peiffer G Brousse N Jaafari Source Type: research

Genetic counselling and testing in pulmonary arterial hypertension - A consensus statement on behalf of the International Consortium for Genetic Studies in PAH - French version
Rev Mal Respir. 2023 Nov 1:S0761-8425(23)00263-2. doi: 10.1016/j.rmr.2023.10.004. Online ahead of print.ABSTRACTPulmonary arterial hypertension (PAH) is a rare disease that can be caused by (likely) pathogenic germline genomic variants. In addition to the most prevalent disease gene, BMPR2 (bone morphogenetic protein receptor 2), several genes, some belonging to distinct functional classes, are also now known to predispose to the development of PAH. As a consequence, specialist and non-specialist clinicians and healthcare professionals are increasingly faced with a range of questions regarding the need for, approaches to a...
Source: Revue des Maladies Respiratoires - November 3, 2023 Category: Respiratory Medicine Authors: D Montani C A Eichstaedt C Belge W K Chung S Gr äf E Gr ünig M Humbert R Quarck J A Tenorio-Castano F Soubrier R C Trembath N W Morrell PAH-ICON associated with the PVRI Source Type: research

Anti-IL-5 in severe asthma associated with eosinophilic granulomatosis with polyangiitis. Real-life study
CONCLUSIONS: Mepolizumab at a "severe asthma" dose (100mg/4weeks) is effective in treatment of GC-dependent severe asthma in most patients with EGPA.PMID:37923651 | DOI:10.1016/j.rmr.2023.10.003 (Source: Revue des Maladies Respiratoires)
Source: Revue des Maladies Respiratoires - November 3, 2023 Category: Respiratory Medicine Authors: F Akdime S Habib L Regard B Terrier P Cohen L Mouthon L Guillevin P R Burgel I Honore X Pu échal N Roche Source Type: research

Evaluation of the risk of sleep apnea hypopnea syndrome in patients awaiting general anesthesia in Burkina Faso
CONCLUSION: The risk of OSAHS in this population is comparable to that of the general population. Confirmation of OSAHS by ventilatory polygraphy or polysomnography would help to achieve further precision.PMID:37866979 | DOI:10.1016/j.rmr.2023.10.002 (Source: Revue des Maladies Respiratoires)
Source: Revue des Maladies Respiratoires - October 22, 2023 Category: Respiratory Medicine Authors: G Bougma A R Ouedraogo K Boncoungou P Bonkoungou S Adambounou A J F Tiendrebeogo S Damoue J Ouedraogo G Ouedraogo G Badoum F Soyez M Ouedraogo Source Type: research