scholarly journals Congenital Pulmonary Alveolar Proteinosis

2013 ◽  
Vol 2013 ◽  
pp. 1-2 ◽  
Author(s):  
Saber Hammami ◽  
Khaled Harrathi ◽  
Khaled Lajmi ◽  
Samir Hadded ◽  
Chebil Ben Meriem ◽  
...  
Keyword(s):  
Pulmonary Surfactant ◽  
Standard Treatment ◽  
Pulmonary Alveolar Proteinosis ◽  
Late Onset ◽  
Spontaneous Remission ◽  
Lung Lavage ◽  
Current Standard ◽  
Whole Lung Lavage ◽  
Current Standard Treatment ◽  
Alveolar Proteinosis

Pulmonary alveolar proteinosis (PAP) is a rare syndrome characterized by pulmonary surfactant accumulation within the alveolar spaces. It occurs with a reported prevalence of 0.1 per 100,000 individuals. Two clinically different pediatric types have been defined as congenital PAP which is fatal and a late-onset PAP which is similar to the adult form and less severe. The clinical course of PAP is variable, ranging from spontaneous remission to respiratory failure. Whole-lung lavage is the current standard treatment for PAP patients. We report a new congenital case of PAP.

scholarly journals Pulmonary alveolar proteinosis: diagnostic and therapeutic challenges

2012 ◽  
Vol 7 ◽  
Author(s):  
Ilaria Campo ◽  
Zamir Kadija ◽  
Francesca Mariani ◽  
Elena Paracchini ◽  
Giuseppe Rodi ◽  
...  
Keyword(s):  
Pulmonary Alveolar Proteinosis ◽  
Gene Mutations ◽  
Spontaneous Remission ◽  
Lung Lavage ◽  
Current Standard ◽  
Gm Csf ◽  
Genes Encoding ◽  
Current Standard Treatment ◽  
Alveolar Proteinosis ◽  
Macrophage Colony

Pulmonary Alveolar Proteinosis (PAP) is a rare syndrome characterized by pulmonary surfactant accumulation within the alveolar spaces. It occurs with a reported prevalence of 0.1 per 100,000 individuals and in distinct clinical forms: autoimmune (previously referred to as the idiopathic form, represents the vast majority of PAP cases, and is associated with Granulocyte-Macrophage Colony Stimulating Factor (GM-CSF) auto-antibodies; GMAbs), secondary (is a consequence of underlying disorders), congenital (caused by mutations in the genes encoding for the GM-CSF receptor), and PAP-like syndromes (disorders associated with surfactant gene mutations). The clinical course of PAP is variable, ranging from spontaneous remission to respiratory failure. Whole lung lavage (WLL) is the current standard treatment for PAP patients and although it is effective in the majority of cases, disease persistence is not an unusual outcome, even if disease is well controlled by WLL. In this paper we review the therapeutic strategies which have been proposed for the treatment of PAP patients and the progress which has been made in the understanding of the disease pathogenesis.

How to do whole lung lavage for treatment of pulmonary alveolar proteinosis

2020 ◽  
Vol 90 (5) ◽  
pp. 877-878
Author(s):  
Tovi Vo ◽  
Justin C. Y. Chan ◽  
Michael Worthington
Keyword(s):  
Pulmonary Alveolar Proteinosis ◽  
Lung Lavage ◽  
Whole Lung Lavage ◽  
Alveolar Proteinosis

scholarly journals Pulmonary alveolar proteinosis treatment by whole-lung lavage

2012 ◽  
Vol 88 (1042) ◽  
pp. 492-493 ◽  
Author(s):  
Chang Cai ◽  
Min Ye ◽  
Honglei Xu ◽  
Yuping Li
Keyword(s):  
Pulmonary Alveolar Proteinosis ◽  
Lung Lavage ◽  
Whole Lung Lavage ◽  
Alveolar Proteinosis

scholarly journals PULMONARY ALVEOLAR PROTEINOSIS SUBMITTED A WHOLE LUNG LAVAGE AFTER COVID-19: A CASE REPORT

CHEST Journal ◽  
2021 ◽  
Vol 160 (4) ◽  
pp. A1171
Author(s):  
Paula D'Ambrosio ◽  
Philippe Colares ◽  
Pedro Ciaralo ◽  
HÉLIO MINAMOTO ◽  
Ronaldo Kairalla
Keyword(s):  
Case Report ◽  
Pulmonary Alveolar Proteinosis ◽  
Lung Lavage ◽  
Whole Lung Lavage ◽  
Alveolar Proteinosis

scholarly journals Bilateral whole lung lavage in pulmonary alveolar proteinosis – A retrospective study

2014 ◽  
Vol 20 (5) ◽  
pp. 254-259 ◽  
Author(s):  
A. Silva ◽  
A. Moreto ◽  
C. Pinho ◽  
A. Magalhães ◽  
A. Morais ◽  
...  
Keyword(s):  
Retrospective Study ◽  
Pulmonary Alveolar Proteinosis ◽  
Lung Lavage ◽  
Whole Lung Lavage ◽  
Alveolar Proteinosis
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