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Efficacy of Whole-Lung Lavage in Pulmonary Alveolar Proteinosis: A Multicenter International Study of GELF
- Source :
- Respiration, Respiration, Karger, 2017, 93 (3), pp.198-206. ⟨10.1159/000455179⟩, Respiration, 2017, 93 (3), pp.198-206. ⟨10.1159/000455179⟩
- Publication Year :
- 2017
- Publisher :
- S. Karger AG, 2017.
-
Abstract
- Background: New therapies have emerged in the treatment of pulmonary alveolar proteinosis (PAP) and, therefore, there is a real need to evaluate the efficacy of whole-lung lavage (WLL) in this rare disease. Objectives: The aim of this study was to assess the efficacy of WLL in patients with PAP. Methods: We included 33 patients from 12 centers, which are members of the French-Speaking Thoracic Endoscopy Group, for analysis. Data collection concerned patients and disease characteristics, pulmonary function tests (PFTs) and technical information on the procedure. Results: The median age of the patients was 44 years (range 13-77). There were 23 (71.9%) patients with respiratory insufficiency at presentation. All patients underwent WLL by general anesthesia and selective lung ventilation, except 1 who underwent awake flexible bronchoscopy. We noted differences in the technique, as 12 (36.36%) patients had percussion during the procedure and only 4 (12.1%) patients underwent 2-lung lavage during 1 anesthesia. A median of 12 L was used to perform WLL (1.0-40 L). Complications occurred in 11 (33.3%) patients, and 18 (56.25%) of them relapsed in a median period of 16.9 months. No significant changes were found in any PFT parameters studied, except for PaO2, which was significantly improved by 6.375 mm Hg (p = 0.0213) after the procedure compared to before. Conclusions: Although the application of the WLL technique was variable, overall, it significantly improved patients' short-term respiratory condition by improving PaO2. However, a long-term effect needs to be confirmed, as many of our patients relapsed.
- Subjects :
- Adult
Male
Pulmonary and Respiratory Medicine
medicine.medical_specialty
Adolescent
Pulmonary Alveolar Proteinosis
030204 cardiovascular system & hematology
Bronchoalveolar Lavage
[SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract
Pulmonary function testing
Young Adult
03 medical and health sciences
0302 clinical medicine
Bronchoscopy
Humans
Medicine
In patient
Respiratory system
Flexible bronchoscopy
Aged
medicine.diagnostic_test
business.industry
Whole lung lavage
Middle Aged
medicine.disease
Respiratory Function Tests
3. Good health
Endoscopy
Surgery
Treatment Outcome
030228 respiratory system
[SDV.MHEP.PSR] Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract
Female
Respiratory Insufficiency
business
Pulmonary alveolar proteinosis
Rare disease
Subjects
Details
- ISSN :
- 14230356 and 00257931
- Volume :
- 93
- Database :
- OpenAIRE
- Journal :
- Respiration
- Accession number :
- edsair.doi.dedup.....02ca345376997f1aee43c6fe3839a744