AUTHOR=Petrarulo Simone , Lucchin Manuel , Oldani Stefano , Dubini Alessandra , Piciucchi Sara , Gori Alberto , Aiello Luca , Maitan Stefano , Spagnolo Paolo , Ravaglia Claudia , Poletti Venerino 

TITLE=A complicated case of whole-lung lavage: a case report

JOURNAL=Frontiers in Medicine

VOLUME=Volume 10 - 2023

YEAR=2023

URL=https://www.frontiersin.org/journals/medicine/articles/10.3389/fmed.2023.1225167

DOI=10.3389/fmed.2023.1225167

ISSN=2296-858X

ABSTRACT=Introduction. We report a life threatening case of severe respiratory failure due to a pulmonary alveolar proteinosis (PAP) secondary to lysinuric protein intolerance (LPI), complicated by a pre-existing right pneumothorax, which we treated using a rescue whole lung lavage (WLL).To date, in literature there are no cases of WLL performed in this condition.Patient was referred to our center because of rapidly worsening dyspnea and deterioration of gas exchange, caused by a secondary form of PAP which required an immediate therapeutic option such as the one offered by WLL. On physical examination bilateral crackles were present and peripheral blood oxygen saturation was 78% on oxygen with a FiO2 of 40%.Interventions. After stabilizing the clinical conditions with oxygen therapy erogated through high flow nasal cannula, shortly after admission we performed a rescue WLL among two procedures. The procedure was very effective and the patient was later discharged without oxygen therapy and in good clinical conditions.Our case report represents a chance to help fill the gap of knowledge relative to secondary forms of PAP. The patient we presented suffers from a very rare genetic condition (LPI) that only has a few reported cases in literature and has a very low prevalence which makes it difficult to produce the affected people: newborns ratio. We believe that through presenting difficult and rare cases like this one, we can improve our understanding of the disease and, most importantly, of how much the only therapeutic option we had, a rescue WLL, is effective to improve gas exchange and radiological features, despite it was performed in this severe respiratory conditions.