scholarly journals Pulmonary Alveolar Proteinosis (PAP) in pregnancy: An important spot diagnosis

2021 ◽  
Vol 5 (2) ◽  
pp. 16-18
Author(s):  
Choon Seong Ng ◽  
Boon Hau Ng

Pulmonary alveolar proteinosis is a relatively rare syndrome of pulmonary surfactant clearance dysfunction that could present like asthma. A middle-aged pregnant lady presented with asthma-like symptoms which was negative for autoimmune screening, whom thoracic imaging consistent with ground-glass opacity superimposed with septal thickening. Whole lung bronchopulmonary lavage fluid analysis showed predominantly eosiniphilic material within alveolar space. Subsequent lung biopsy revealed positive PAS stain for eosinophilic material. Its presentation in pregnancy could pose challenge to delivery. The associated maternal infection risk could compromise fetal survival.

2021 ◽  
Vol 11 (3) ◽  
pp. 191-195
Author(s):  
Fanny Fachrucha ◽  
Sita Andarini ◽  
Mia Elhidsi ◽  
Rizky Yudha Irawan ◽  
Romi Beginta ◽  
...  

Pulmonary alveolar proteinosis (PAP) is a rare disease with mostly due to autoimmune toward granulocyte-macrophage colony-stimulating factor. In some conditions, PAP followed with secondary infection. A 34-year-old woman came with progressive shortness of breath, chronic dry cough, and mild fever. The chest High-Resolution Computed Tomography showed ground-glass opacity with septal reticulation or known as the crazy-paving pattern, and a cavity on the upper lobe of the left lung. The patient underwent bronchoscopy for diagnostic and therapeutic measures and found milky appearance bronchoalveolar lavage fluid (BALF). The serum galactomannan came out positive. Fungal infection detected from the BALF culture, Aspergillus fumigatus, hence fulfilling the diagnosis of PAP with probable invasive pulmonary aspergillosis. The patient showed clinical improvement after undergoing whole lung lavage and given anti-fungal medications.


2016 ◽  
Vol 2016 ◽  
pp. 1-5 ◽  
Author(s):  
Qiongya Mo ◽  
Bingbin Wang ◽  
Nian Dong ◽  
Lianmin Bao ◽  
Xiaoqiong Su ◽  
...  

Pulmonary alveolar proteinosis (PAP) is a rare interstitial lung disease characterized by the abnormal alveolar accumulation of surfactant components. The diagnosis of PAP can be easily missed since it is rare and lacks specific clinical symptoms. It is of great importance to have a better understanding of the crucial clue to clinically diagnose PAP and take PAP into consideration in the differential diagnosis of interstitial pulmonary diseases or other diseases with similar manifestations. Here, we analyze the clinical characteristics of 11 cases of PAP patients in local hospital and review the relevant literature in order to provide more information in diagnosis and management of PAP. In our observation, cyfra21-1 and neuron-specific enolase (NSE) known as tumor markers probably can be useful serum markers for diagnosis of PAP. As for the method of pathologic diagnosis, open-lung biopsy was the gold standard but now it is less required because findings on examination of bronchoalveolar lavage fluid (BALF) can help to make the diagnosis. We also have deep experience about when and how to carry out lung lavage.


2014 ◽  
Vol 32 (11) ◽  
pp. 657-660 ◽  
Author(s):  
Katsuhide Kojima ◽  
Katsuya Kato ◽  
Takuya Fukazawa ◽  
Ichiro Morita ◽  
Nagio Takigawa ◽  
...  

2013 ◽  
Vol 2013 ◽  
pp. 1-2 ◽  
Author(s):  
Saber Hammami ◽  
Khaled Harrathi ◽  
Khaled Lajmi ◽  
Samir Hadded ◽  
Chebil Ben Meriem ◽  
...  

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.


2016 ◽  
Vol 2016 ◽  
pp. 1-8 ◽  
Author(s):  
JiuWu Bai ◽  
JinFu Xu ◽  
WenLan Yang ◽  
Beilan Gao ◽  
Weijun Cao ◽  
...  

Background. Pulmonary Alveolar Proteinosis (PAP) is a syndrome characterized by pulmonary surfactant accumulation. Small proportion of PAP patients experienced spontaneous remission.Objective. The aim of this study was to assess the severity and prognosis of PAP using various indexes.Methods. Characteristics, PaO2, lung function parameters, and HRCT score of 101 patients with PAP were retrospectively analyzed. Many indexes were explored and integrated into a scale.Results. PaO2was lower among smokers than among never-smokers. PaO2differed between each pair of patient groups stratified according to HRCT score or DLCO, % predicted, which differed between any two groups stratified according to PaO2. The PAP patients who died presented with more symptoms, a higher HRCT score, and lower DLCO, % predicted, than survivors. Smoking status, symptoms, PaO2, HRCT score, and DLCO, % predicted, were integrated into a scale (severity and prognosis score of PAP (SPSP)). SPSP correlated positively with PaO2, FVC, % predicted, FEV1, % predicted, and DLCO, % predicted, and negatively with HRCT score. The patients who died displayed a higher SPSP than survivors.Conclusion. Smoking status, symptoms, PaO2, HRCT score, and DLCO, % predicted, were integrated into a scale (SPSP) that can be used to assess the severity and prognosis of PAP to some degree.


2006 ◽  
Vol 13 (4) ◽  
pp. 204-206 ◽  
Author(s):  
Matthew D. Jankowich ◽  
Momen M. Wahidi ◽  
David Feller-Kopman ◽  
Armin Ernst

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