Indian Journal of Critical Care Medicine

Register      Login

SEARCH WITHIN CONTENT

FIND ARTICLE

Volume / Issue

Online First

Archive
Related articles

VOLUME 25 , ISSUE 9 ( September, 2021 ) > List of Articles

CASE REPORT

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

Prahlad Prabhudesai, Indu Khosla, Satish Kulkarni, Manish Kumar Arya, Abha Pandey, Namrata Yadav

Keywords : Extracorporeal membrane oxygenation, Pulmonary alveolar proteinosis, Whole lung lavage

Citation Information : Prabhudesai P, Khosla I, Kulkarni S, Arya M K, Pandey A, Yadav N. Bilateral Whole Lung Lavage in Hereditary Pulmonary Alveolar Proteinosis in a 4-year-old Child Using Extracorporeal Membrane Oxygenation. Indian J Crit Care Med 2021; 25 (9):1069-1072.

DOI: 10.5005/jp-journals-10071-23968

License: CC BY-NC 4.0

Published Online: 08-09-2021

Copyright Statement:  Copyright © 2021; Jaypee Brothers Medical Publishers (P) Ltd.


Abstract

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.


PDF Share
  1. Trapnell BC, Whitsett JA, Nakata K. Pulmonary alveolar proteinosis. New Engl J Med 2003;349(26):2527–2539. DOI: 10.1056/NEJMra023226.
  2. Bush A, Pabary R. Pulmonary alveolar proteinosis in children. Breathe 2020;16(2). DOI: 10.1183/20734735.0001-2020.
  3. Borie R, Danel C, Debray MP, Taille C, Dombret MC, Aubier M, et al. Pulmonary alveolar proteinosis. Eur Respir Rev 2011;20(120):98–107. DOI: 10.1183/09059180.00001311.
  4. Martinez-Moczygemba M, Doan ML, Elidemir O, Fan LL, Cheung SW, Lei JT, et al. Pulmonary alveolar proteinosis caused by deletion of the GM-CSFRα gene in the X chromosome pseudoautosomal region 1. J Exp Med 2008;205(12):2711–2716. DOI: 10.1084/jem.20080759.
  5. Suzuki T, Sakagami T, Rubin BK, Nogee LM, Wood RE, Zimmerman SL, et al. Familial pulmonary alveolar proteinosis caused by mutations in CSF2RA. J Exp Med 2008;205(12):2703–2710. DOI: 10.1084/jem.20080990.
  6. Hildebrandt J, Yalcin E, Bresser HG, Cinel G, Gappa M, Haghighi A, et al. Characterization of CSF2RA mutation related juvenile pulmonary alveolar proteinosis. Orphanet J Rare Dis 2014;9(1):171. DOI: 10.1186/s13023-014-0171-z.
  7. Suzuki T, Maranda B, Sakagami T, Catellier P, Couture CY, Carey BC, et al. Hereditary pulmonary alveolar proteinosis caused by recessive CSF2RB mutations. Eur Respir J 2011;37(1):201–204. DOI: 10.1183/09031936.00090610.
  8. Tanaka T, Motoi N, Tsuchihashi Y, Tazawa R, Kaneko C, Nei T, et al. Adult-onset hereditary pulmonary alveolar proteinosis caused by a single-base deletion in CSF2RB. J Med Genet 2011;48(3):205–209. DOI: 10.1136/jmg.2010.082586.
  9. Hamvas A, Deterding RR, Wert SE, White FV, Dishop MK, Alfano DN, et al. Heterogeneous pulmonary phenotypes associated with mutations in the thyroid transcription factor gene NKX2-1. Chest 2013;144(3):794–804. DOI: 10.1378/chest.12-2502.
  10. Ramirez JR. Bronchopulmonary lavage: new techniques and observations. Dis Chest 1966;50(6):581–588. DOI: 10.1378/chest.50.6.581.
  11. Sihoe AD, Ng VM, Liu RW, Cheng LC. Pulmonary alveolar proteinosis in extremis: the case for aggressive whole lung lavage with extracorporeal membrane oxygenation support. Heart Lung Circ 2008;17(1):69–72. DOI: 10.1016/j.hlc.2006.11.007.
  12. Hiratzka LF, Swan DM, Rose EF, Ahrens RC. Bilateral simultaneous lung lavage utilizing membrane oxygenator for pulmonary alveolar proteinosis in an 8-month-old infant. Ann Thorac Surg 1983;35(3): 313–317. DOI: 10.1016/S0003-4975(10)61565-2.
PDF Share
PDF Share

© Jaypee Brothers Medical Publishers (P) LTD.