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Pulmonary alveolar proteinosis
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Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by progressive accumulation of the alveolar surfactant. Whole lung lavage (WLL) using a high volume of warmed saline remains the standard therapy.
7p
vitiki
30-01-2024
3
2
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Pulmonary alveolar proteinosis is a rare disorder that is characterized by a large accumulation of lipoproteinaceous material within the alveoli. This causes respiratory failure due to a restriction of gas exchange and changes in the ventilation/perfusion ratio. Clinical symptoms are variable and depend on the severity of damage of the lung parenchyma.
5p
viisaacnewton
25-04-2022
13
2
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Whole Lung Lavage (WLL) has been an important part in the management of Pulmonary Alveolar Pro‑ teinosis (PAP) since it improves radiologic and clinical parameters. Bilateral WLL is usually performed in two sessions on different days. Few case reports have described one-session bilateral sequential lung lavage (OSBSWLL), and none have described ambulatory management (same-day discharge).
7p
vimackenziebezos
29-11-2021
4
1
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The majority of cases with severe pulmonary alveolar proteinosis (PAP) are caused by auto-antibodies against GM-CSF. A multitude of genetic and exogenous causes are responsible for few other cases. Goal of this study was to determine the prevalence of GATA2 deficiency in children and adults with PAP and hematologic disorders.
8p
vimontana2711
05-04-2021
11
2
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Although no report has demonstrated the efficacy of corticosteroid therapy for autoimmune pulmonary alveolar proteinosis (aPAP), we sometimes encounter patients who have received this therapy for various reasons.
9p
vimontana2711
05-04-2021
10
2
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Secondary pulmonary alveolar proteinosis (sPAP) is a very rare lung disorder comprising approximately 10% of cases of acquired PAP. Hematological disorders are the most common underlying conditions of sPAP, of which 74% of cases demonstrate myelodysplastic syndrome (MDS). However, the impact of sPAP on the prognosis of underlying MDS remains unknown.
10p
vimontana2711
05-04-2021
9
2
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Whole-lung lavage (WLL) is classically the first-line treatment for symptomatic pulmonary alveolar proteinosis (PAP). However, some patients require multiple WLLs because of refractory nature of their PAP. In this instance, these patients may benefit from new treatment regimens, and new therapies should be tried for these patients.
5p
vimontana2711
05-04-2021
10
1
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Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare lung disease caused by the autoantibody against granulocyte-macrophage colony stimulating factor (GM-CSF). The clinical course of aPAP is variable; in severe cases, patients develop lethal respiratory failure due to pulmonary fibrosis.
4p
vimontana2711
05-04-2021
10
1
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Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by accumulation of phospholipoproteinaceous material in the alveoli. The presentation is nonspecific but typically includes dyspnea; the spectrum of disease includes rapidly progressive hypoxic respiratory failure.
6p
vimaine2711
26-03-2021
10
2
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Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by surfactant accumulation, and is caused by disruption of granulocyte/macrophage colony-stimulating factor (GM-CSF) signaling. Abnormalities in CSF2 receptor alpha (CSF2RA) were reported to cause pediatric hereditary PAP.
7p
vimaine2711
26-03-2021
12
2
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Pulmonary alveolar proteinosis (PAP) is characterized by the accumulation of surfactant proteins within the alveolar spaces. Autoimmune PAP (APAP) caused by elevated levels of GM-CSF autoantibodies (GM-Ab) is very rarely associated with systemic autoimmune disease. Here we report a case of APAP manifested during immunosuppressive treatment for polymyositis with interstitial lung disease.
6p
vimaine2711
26-03-2021
12
2
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Pulmonary alveolar proteinosis (PAP) is a very rare lung disease and its prevalence and incidence remain unclear. The prevalence and incidence of PAP were investigated by using nationwide claims data from the Korean Health Insurance Review and Assessment service.
8p
vimaine2711
26-03-2021
14
2
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This study aimed to investigate clinical characteristics of Korean PAP patients and to examine the potential risk factors of PAP. Methods: We retrospectively reviewed medical records of 78 Korean PAP patients diagnosed between 1993 and 2014. Patients were classified into two groups according to the presence/absence of treatment (lavage).
9p
vimaine2711
26-03-2021
10
1
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Secondary pulmonary alveolar proteinosis (sPAP) is an extremely rare disease. The clinical features of sPAP patients remain to be summarizeds. MDS and TB infection were the most frequent underlying causes of sPAP in this single-center research in China, with cases secondary to MDS having a poor survival rate.
7p
vimaine2711
26-03-2021
8
3
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Dr. Sharon Rounds, the editor for this series who invited us to write a book on rare lung diseases, developed the idea after attending the 2004 Lymphangioleiomyomatosis (LAM) Foundation annual research meeting. She was a keynote speaker at that event (during her tenure as the president of the American Thoracic Society) and was witness to the power of patient advocacy and the mission-based scientific effort that had brought this rare disease of women from obscurity to clinical trials with targeted molecular therapies in under a decade.
443p
crius75
04-01-2013
55
10
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Tuyển tập báo cáo các nghiên cứu khoa học quốc tế ngành y học dành cho các bạn tham khảo đề tài: Anaesthesia for serial whole-lung lavage in a patient with severe pulmonary alveolar proteinosis: a case report
3p
thulanh30
20-12-2011
40
2
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Tuyển tập báo cáo các nghiên cứu khoa học quốc tế ngành y học dành cho các bạn tham khảo đề tài: Unsuspected pulmonary alveolar proteinosis in a patient with acquired immunodeficiency syndrome: a case report
5p
thulanh26
08-12-2011
46
3
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Tuyển tập các báo cáo nghiên cứu về y học được đăng trên tạp chí y học 'Respiratory Research cung cấp cho các bạn kiến thức về ngành y đề tài: " Iron homeostasis and oxidative stress in idiopathic pulmonary alveolar proteinosis: a case-control study
8p
toshiba12
21-10-2011
58
2
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Tuyển tập các báo cáo nghiên cứu về y học được đăng trên tạp chí y học 'Respiratory Research cung cấp cho các bạn kiến thức về ngành y đề tài: " Elemental analysis of lung tissue particles and intracellular iron content of alveolar macrophages in pulmonary alveolar proteinosis...
7p
toshiba11
20-10-2011
64
2
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