Ten-year experience of whole lung lavage in pediatric Pulmonary Alveolar Proteinosis.

ABSTRACT:

Background

Pulmonary Alveolar Proteinosis (PAP) is extremely rare and can be caused by hereditary dysfunction of the granulocyte macrophage colony-stimulating factor receptor (GM-CSF) receptor, autoantibodies against GM-CSF, or other diseases leading to alveolar macrophage (AM) dysfunction. This leads to protein accumulation in the lung and severe dyspnea and hypoxemia. Whole lung lavage (WLL) is the first line treatment strategy.

Methods

Here, we present data from more than ten years of WLL practice in pediatric PAP. WLL performed by the use of a single lumen or double lumen tube (SLT vs. DLT) were compared for technical features, procedure time, and adverse events.

Results

A total of n=57 procedures in six PAP patients between 3.5 and 14.3 years of age were performe

SUBMITTER: Nickel K 

PROVIDER: S-EPMC10883753 | biostudies-literature | 2024 Feb

REPOSITORIES: biostudies-literature