Description:
<jats:title>Abstract</jats:title><jats:p>
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.</jats:p><jats:p>
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.</jats:p><jats:p>
Results A total of n=57 procedures in six PAP patients between 3.5
and 14.3 years of age were performed. SLT based WLL in smaller children was
associated with comparable rates of adverse events but with longer intervention
times and postprocedural intensive care treatment when compared to DLT based
procedures.</jats:p><jats:p>
Discussion Our data shows that WLL is feasible even in small children. DLT
based WLL seems to be more effective, and our data supports the notion that it
should be considered as early as possible in pediatric PAP.</jats:p><jats:p>
Conclusion WLL lavage is possible in small PAP patients but should
performed in close interdisciplinary cooperation and with age appropriate
protocols.</jats:p>