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Management of primary pulmonary alveolar proteinosis: A multicentric experience

BACKGROUND: Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by alveolar accumulation of surfactant material with reduced lung function and resulting hypoxemia. It is characterized by a variable clinical course, and whole lung lavage (WLL) is the standard treatment. Herein, we re...

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Autores principales: Marwah, Vikas, Katoch, CDS, Singh, Sarvinder, Handa, Ajay, Vardhan, Vasu, Rajput, AK, Barthwal, MS, Bhattacharyya, D, Rai, SP
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer - Medknow 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7507921/
https://www.ncbi.nlm.nih.gov/pubmed/32643638
http://dx.doi.org/10.4103/lungindia.lungindia_401_19
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author Marwah, Vikas
Katoch, CDS
Singh, Sarvinder
Handa, Ajay
Vardhan, Vasu
Rajput, AK
Barthwal, MS
Bhattacharyya, D
Rai, SP
author_facet Marwah, Vikas
Katoch, CDS
Singh, Sarvinder
Handa, Ajay
Vardhan, Vasu
Rajput, AK
Barthwal, MS
Bhattacharyya, D
Rai, SP
author_sort Marwah, Vikas
collection PubMed
description BACKGROUND: Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by alveolar accumulation of surfactant material with reduced lung function and resulting hypoxemia. It is characterized by a variable clinical course, and whole lung lavage (WLL) is the standard treatment. Herein, we report our multicentric experience of management of primary PAP. MATERIALS AND METHODS: This retrospective study included patients with PAP managed at various armed forces respiratory centers from 2009 to 2019. The diagnosis of primary PAP was based on histopathologic confirmation on transbronchial lung biopsy or open lung biopsy and absence of causes of secondary PAP. We analyzed the response to WLL in these patients as well as the safety of the procedure. RESULTS: During the above-specified period, ten patients with a diagnosis of PAP were admitted to various armed forces respiratory centers. The median age of the patients was 34.5 years (range 23–59); there were nine males (90%). The mean duration (± standard deviation) of symptoms was 10.8 (±2.70) months. For management, WLL was done for eight patients with a median volume of 23.5 L (range 18–45) per patient. All the patients showed significant symptomatic response as well as improvement in physiological parameters with no major complications. The median follow-up of all patients was 18 (range 5–44) months. CONCLUSIONS: WLL is a safe, effective therapy in an experienced setting in patients with PAP and provides long-lasting benefits.
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spelling pubmed-75079212020-10-02 Management of primary pulmonary alveolar proteinosis: A multicentric experience Marwah, Vikas Katoch, CDS Singh, Sarvinder Handa, Ajay Vardhan, Vasu Rajput, AK Barthwal, MS Bhattacharyya, D Rai, SP Lung India Original Article BACKGROUND: Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by alveolar accumulation of surfactant material with reduced lung function and resulting hypoxemia. It is characterized by a variable clinical course, and whole lung lavage (WLL) is the standard treatment. Herein, we report our multicentric experience of management of primary PAP. MATERIALS AND METHODS: This retrospective study included patients with PAP managed at various armed forces respiratory centers from 2009 to 2019. The diagnosis of primary PAP was based on histopathologic confirmation on transbronchial lung biopsy or open lung biopsy and absence of causes of secondary PAP. We analyzed the response to WLL in these patients as well as the safety of the procedure. RESULTS: During the above-specified period, ten patients with a diagnosis of PAP were admitted to various armed forces respiratory centers. The median age of the patients was 34.5 years (range 23–59); there were nine males (90%). The mean duration (± standard deviation) of symptoms was 10.8 (±2.70) months. For management, WLL was done for eight patients with a median volume of 23.5 L (range 18–45) per patient. All the patients showed significant symptomatic response as well as improvement in physiological parameters with no major complications. The median follow-up of all patients was 18 (range 5–44) months. CONCLUSIONS: WLL is a safe, effective therapy in an experienced setting in patients with PAP and provides long-lasting benefits. Wolters Kluwer - Medknow 2020 2020-07-01 /pmc/articles/PMC7507921/ /pubmed/32643638 http://dx.doi.org/10.4103/lungindia.lungindia_401_19 Text en Copyright: © 2020 Indian Chest Society http://creativecommons.org/licenses/by-nc-sa/4.0 This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
spellingShingle Original Article
Marwah, Vikas
Katoch, CDS
Singh, Sarvinder
Handa, Ajay
Vardhan, Vasu
Rajput, AK
Barthwal, MS
Bhattacharyya, D
Rai, SP
Management of primary pulmonary alveolar proteinosis: A multicentric experience
title Management of primary pulmonary alveolar proteinosis: A multicentric experience
title_full Management of primary pulmonary alveolar proteinosis: A multicentric experience
title_fullStr Management of primary pulmonary alveolar proteinosis: A multicentric experience
title_full_unstemmed Management of primary pulmonary alveolar proteinosis: A multicentric experience
title_short Management of primary pulmonary alveolar proteinosis: A multicentric experience
title_sort management of primary pulmonary alveolar proteinosis: a multicentric experience
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7507921/
https://www.ncbi.nlm.nih.gov/pubmed/32643638
http://dx.doi.org/10.4103/lungindia.lungindia_401_19
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