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Bilateral Whole Lung Lavage in Hereditary Pulmonary Alveolar Proteinosis in a 4-year-old Child Using Extracorporeal Membrane Oxygenation

The hereditary form of pulmonary alveolar proteinosis (PAP) is an uncommon entity. We report a case of PAP due to colony-stimulating factor 2 receptor alpha (CSF2RA) gene mutation. The standard of care includes whole lung lavage (WLL). We faced two challenges: Firstly, a severely hypoxemic patient,...

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Detalles Bibliográficos
Autores principales: Prabhudesai, Prahlad, Khosla, Indu, Kulkarni, Satish, Arya, Manish Kumar, Pandey, Abha, Yadav, Namrata
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Jaypee Brothers Medical Publishers 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8664037/
https://www.ncbi.nlm.nih.gov/pubmed/34963730
http://dx.doi.org/10.5005/jp-journals-10071-23968
Descripción
Sumario:The hereditary form of pulmonary alveolar proteinosis (PAP) is an uncommon entity. We report a case of PAP due to colony-stimulating factor 2 receptor alpha (CSF2RA) gene mutation. The standard of care includes whole lung lavage (WLL). We faced two challenges: Firstly, a severely hypoxemic patient, and secondly, the nonavailability of appropriate size of double-lumen endotracheal tube for pediatric patients for a WLL while permitting single-lung ventilation. Hence, we performed WLL using venovenous extracorporeal membrane oxygenation (VV ECMO) with a successful outcome. The patient has been discharged and is off oxygen support since more than a year. There are only a few case reports of children having hereditary PAP treated with WLL using ECMO in Indian and Western literature. HOW TO CITE THIS ARTICLE: Prabhudesai P, Khosla I, Kulkarni S, Arya MK, Pandey A, Yadav N. Bilateral Whole Lung Lavage in Hereditary Pulmonary Alveolar Proteinosis in a 4-year-old Child Using Extra corporeal Membrane Oxygenation. Indian J Crit Care Med 2021;25(9):1069–1072.