scholarly journals PULMONARY ALVEOLAR PROTEINOSIS - A CASE REPORT AND REVIEW OF LITERATURE

2020 ◽  
pp. 1-3
Author(s):  
Abhilash Narvenkar ◽  
Uday C. Kakodkar* ◽  
Chetan Kerkar ◽  
John Muchahary

Pulmonary alveolar proteinosis (PAP) is a rare disorder characterised by accumulation of lipoproteinaceous material in the alveolar air spaces. Diagnosis depends on histopathological and radiological features. Treatment includes whole lung lavage (WLL) and GM-CSF therapy. We present a case report of primary idiopathic PAP treated with bilateral whole lung lavage. A 50 year old female presented with history of progressive breathlessness and recurrent lower respiratory tract infection. There were bilateral basal ne crepitations on auscultation and she was maintaining saturation of 70% at room air. Serial chest radiographs showed persistent bilateral alveolar opacities. HRCT thorax showed crazy paving pattern involving both lungs. BAL uid showed lipoproteinaceous material which was PAS stain positive. Patient was subsequently treated with bilateral WLLfollowing which there was radiological and clinical improvement

Author(s):  
Renee Horner ◽  
Douglas Gardenhire ◽  
Ralph Zimmerman

ABSTRACT Background: Pulmonary Alveolar Proteinosis (PAP) is a rare pulmonary disorder caused by a congregation of excessive lipoproteinaceous material in the alveolar spaces due to impaired surfactant metabolism. The congregation of the protein in the alveolar space leads to difficulty in breathing, impaired pulmonary immunity, and susceptibility to both opportunistic and acquired pulmonary infections. Although Pulmonary Alveolar Proteinosis is rare, there are potential treatments. Whole-lung lavage is the most widely accepted therapy and course of treatment. An additional form of therapy, GM-CSF stimulating therapy, uses recombinant deoxyribonucleic acid (DNA) technology to increase white cell production. Purpose: The purpose of this case report is to follow a patient through whole-lung lavage therapy to determine outcome and clinical improvement. Case Description: This case report follows a 55-year-old female patient diagnosed with secondary, idiopathic Pulmonary Alveolar Proteinosis through eight normal saline whole-lung lavages. The patient’s treatment was led by a pulmonologist with previous Pulmonary Alveolar Proteinosis experience. Outcome: By the end of the first four lavages, the patient showed clinical improvement, but during a two-month break from therapy, symptoms returned. Following the break, the patient underwent four additional lung lavage sessions and experienced similar relief as in previous courses of treatment. Discussion: The patient did complete an additional eight lavages to wash out excess lipoproteinaceous material to provide a longer period of symptom relief. The additional form of therapy, GM-CSF, is not a therapy option for this patient as the disease was idiopathic in nature. The last available treatment option for this patient is a lung transplant.


CHEST Journal ◽  
2021 ◽  
Vol 160 (4) ◽  
pp. A1171
Author(s):  
Paula D'Ambrosio ◽  
Philippe Colares ◽  
Pedro Ciaralo ◽  
HÉLIO MINAMOTO ◽  
Ronaldo Kairalla

2008 ◽  
Vol 43 (8) ◽  
pp. 828-830 ◽  
Author(s):  
Hajime Yamamoto ◽  
Etsuro Yamaguchi ◽  
Hiroatsu Agata ◽  
Nobuhisa Kandatsu ◽  
Toru Komatsu ◽  
...  

Thorax ◽  
2001 ◽  
Vol 56 (8) ◽  
pp. 664-665
Author(s):  
R M Barraclough ◽  
A J Gillies

Pulmonary alveolar proteinosis is a rare condition traditionally requiring treatment with whole lung lavage. The case is presented of a young man who obtained complete remission following treatment with granulocyte-macrophage colony stimulating factor, a new treatment option.


2017 ◽  
Vol 11 (1) ◽  
Author(s):  
Janith Galhenage ◽  
Buddhika Weerasinghe ◽  
Wadasinghe Dilesha ◽  
Roshana Constantine ◽  
Bandu Gunasena

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