Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0010200 (cough)
 23,843 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The simultaneous presentation of two noninflammatory pulmonary diseases, pulmonary alveolar proteinosis and Kaposi's sarcoma (Ks), in an HIV-infected patient, is described. A 29-year-old black race patient was admitted to the hospital because of general malaise, weight loss, dyspnea, chest pain, and cough with hemoptoic expectoration. Chest X-rays revealed a patchy bilateral alveolar pattern with a tendency toward the formation of condensations. The serological test revealed HIV positivity (CD4 counts of 393 cells/mm3). Because there was no response to the treatment course, a thoracic CT was performed, showing interlobular thickening with intralobular septal lines and ground glass opacities ("crazy-paving" pattern). An open lung biopsy was performed. Histopathological diagnosis of pulmonary alveolar proteinosis and pulmonary Ks was made.
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PMID:Simultaneous presentation of two noninflammatory lung diseases in an HIV-infected patient. 2846 24

Pulmonary alveolar proteinosis (PAP) is a rare disease in children characterized by intra-alveolar accumulation of surfactant proteins, which severely reduces gaseous exchange. Whole lung lavage (WLL) is the preferred technique for the treatment of severe PAP. Herein, we present a pediatric case of PAP treated with WLL. An 11-year-old boy was admitted with the chief complaint of a dry cough lasting 6 months. He developed symptoms of dyspnea on exertion and had difficulty in climbing stairs. He was ultimately diagnosed with PAP through video-assisted thoracoscopic lung biopsy. As first-line of treatment for PAP, he underwent therapeutic WLL for each of his lungs on separate days. After a brief recovery, his symptoms gradually worsened; therefore, he underwent a second WLL. This is the first pediatric case of PAP relapse despite successful WLL in Korea.
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PMID:A pediatric case of relapsed pulmonary alveolar proteinosis despite successful whole lung lavage. 2886 Nov 15

Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disease characterised by alveolar accumulation of surfactant composed of proteins and lipids. Three main categories of PAP have been defined depending on the aetiology: primer/idiopathic, neonatal/congenital, secondary and exogenous/environmental exposure. Radiologically diffuse ground glass opacities, interlobular and intralobular septal thickening is seen. Although open lung biopsy is accepted as the gold standard in diagnosis, it can be diagnosed by showing bronchoalveolar lavage (BAL) fluid with a milky appearance and periodic acid-schiff (PAS) positive globules in biopsy with clinical and radiological findings. Theraphy for PAP are supportive care (oxygen), total lung lavage, inhale/subcutaneous granulocyte macrophage colony stimulating factor (GM-CSF), rituximab, plasmapheresis, lung transplantation. We report a case of PAP presented with cough and shortness of breath, diagnosed with BAL and transbronchial lung biopsy, treated by total lung lavage by reviewing literature.
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PMID:[Bilateral total lung lavage in a patient with pulmonary alveolar proteinosis]. 2913 4

A 65-year-old Japanese man was referred to our hospital for the further assessment of cough and dyspnea. He had a history of ulcerative colitis for which he was receiving treatment. Chest computed tomography showed a crazy-paving pattern. His bronchoalveolar lavage fluid had a milky appearance, and a transbronchial lung biopsy specimen revealed acellular periodic acid-Schiff stain-positive bodies. The serum anti-granulocyte macrophage-colony stimulating factor (GM-CSF) antibody titer was elevated. The diagnosis was autoimmune pulmonary alveolar proteinosis (PAP). There are few reports of autoimmune PAP in patients with ulcerative colitis. Some reports suggest that PAP and inflammatory bowel disease might have a common pathogenesis involving the anti-GM-CSF antibody.
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PMID:Pulmonary Alveolar Proteinosis with Ulcerative Colitis. 2970 38

Pulmonary alveolar proteinosis (PAP) is a rare disease diagnosed pathologically by the build up of surfactant in the alveolar spaces. Establishing a diagnosis usually requires invasive procedures such as bronchoalveolar lavage and forceps biopsy to obtain tissue specimens. Infrequently, surgery is required when histopathological results from other modalities are equivocal. Cryobiopsy has emerged as a novel technique for obtaining lung tissues in pulmonary diseases. Recently, cryobiopsy has been used to diagnose diffuse parenchymal lung disease, but it has rarely been used for the diagnosis of PAP. Here, we describe a 54-year-old male businessman presenting with intermittent coughing with yellowish sputum and dyspnoea upon exertion for half a year. Tissues from forceps biopsy fail to yield a specific diagnosis, whereas those from cryobiopsy confirm the diagnosis of PAP. Cryobiopsy offers several diagnostic advantages compared to conventional techniques and appears to be a potential diagnostic tool for diagnosing PAP.
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PMID:Novel application of cryobiopsy in the diagnosis of pulmonary alveolar proteinosis. 2993 12

Pulmonary alveolar proteinosis (PAP) - an unusual diffuse lung disease characterized by alveolar accumulation of phospholipoprotein material, with a peak incidence in third to fourth decade and male predominance is also described in children. Recorded prevalence is 0.1/100,000 individuals. Major clinicopathogenetic subtypes include autoimmune (idiopathic) associated with granulocyte-macrophage colony-stimulating factor autoantibodies, secondary form, and the congenital form (associated with surfactant gene mutations). Common presenting features include dyspnea, cough, low-grade fever, inspiratory crackles, and digital clubbing. Pulmonary function shows a restrictive ventilatory defect. X-rays show bilateral patchy to extensive consolidations, and bronchial lavage yields a milky fluid. Characteristic microscopic findings on lung biopsy include filling of terminal bronchioles and alveolar spaces by deep pink granular PAS-positive material. Whole lung lavage is the safest and most effective form of treatment. We present brief profiles of two young children identified as having PAP, along with follow-up data on one of them.
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PMID:Pulmonary alveolar proteinosis in children: An unusual presentation with significant clinical impact. 3000 70

We herein report a case of autoimmune pulmonary alveolar proteinosis (PAP) diagnosed after one-time exposure to silica powder. Owing to the misuse of a silica-containing fire extinguisher and the inhalation of large amounts of its powder, the patient experienced prolonged cough and visited our hospital. The findings of chest computed tomography and surgical lung biopsy specimens led to the diagnosis of PAP. Interestingly, the presence of anti-GM-CSF antibody was detected; therefore, both autoimmune characteristics and exposure to large amounts of silica may have caused the development of PAP in this patient. This case provides important insight into the mechanisms leading to the onset of PAP.
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PMID:Autoimmune Pulmonary Alveolar Proteinosis Diagnosed after Exposure to a Fire Extinguisher Containing Silica Powder. 3099 57

In this report, we describe an unusual manifestation of pulmonary alveolar proteinosis (PAP). The patient is a 43-year-old non-smoking female without underlying hematologic or auto-immune disorder. Her initial presentation included non-specific respiratory symptoms (exertional dyspnoea and cough), an unremarkable physical examination, a mild elevation in her serum level of lactate dehydrogenase, a mild impairment in the diffusion capacity for carbon monoxide but a normal spirometry, and multiple ground-glass opacities with a "crazy-paving" pattern predominantly in upper lung zones on her chest radiographic images. PAP was diagnosed histologically. PAP commonly occurs in males with smoking history, and tends to affect the lung parenchyma diffusely or, as in auto-immune PAP, lower lobes predominantly. Upper-lobe predominant PAP, particularly in a non-smoking female, is rare. This report would add PAP to the list of differential diagnosis for upper-lung ground-glass opacities. A review on the relevant literature is also included in the discussion.
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PMID:Pulmonary alveolar proteinosis with upper-lobe predominance in a non-smoking female. 3116 Oct 40

Myelodysplastic syndrome (MDS) is frequently complicated by pulmonary disease. Here, we describe secondary pulmonary alveolar proteinosis (sPAP) that developed during corticosteroid therapy for organising pneumonia (OP) associated with MDS. A 75-year-old woman with MDS complained of cough for 2 weeks. Chest CT showed bilateral patchy consolidations with reversed halo sign. Bronchoalveolar lavage (BAL) examination showed remarkably increased cell density with an increased lymphocyte proportion. Abnormal radiological findings improved rapidly on administration of systemic corticosteroid under the diagnosis of OP; however, they relapsed a few weeks later. Transbronchial lung biopsy showed periodic acid-Schiff stain-positive amorphous materials. Autoantibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF) in serum and BAL fluid (BALF) were both negative, while GM-CSF level in BALF was elevated. The patient was diagnosed with sPAP. When chest radiological findings show exacerbation during corticosteroid therapy for OP in a patient with MDS, physicians should consider sPAP complication as a differential diagnosis.
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PMID:Secondary pulmonary alveolar proteinosis during corticosteroid therapy for organising pneumonia associated with myelodysplastic syndrome. 3153 95

Pulmonary alveolar proteinosis (PAP) is an uncommon lung disease characterized by excessive accumulation of pulmonary surfactant that usually requires treatment with whole-lung lavage. A 47-year-old female presented with history of dry cough and breathlessness for past 6months. Chest radiograph demonstrated bilateral alveolar shadows and high resolution computerized tomography thorax showed crazy paving pattern. Broncho-alveolar lavage (BAL) and transbronchial lung biopsy confirmed a diagnosis of PAP. Due to worsening hypoxemia and respiratory failure, wholelung lavage was planned and performed. Anaesthetic management involved integrated use of pre-oxygenation, complete lung isolation, one-lung ventilation with optimal positive end-expiratory pressure, vigilant use of positional manoeuvres, and use of recruitment manoeuvres for the lavaged lung. We have discussed valuable strategies for the anaesthetic management of patients undergoing this multifaceted procedure in a case of severe PAP.
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PMID:Anaesthetic considerations for whole lung lavage for pulmonary alveolar proteinosis. 3174 97


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