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)

A 47-year-old woman with a severe cough and high-grade fever demonstrated proteinuria of 3.2 g/day. Chest radiograph and CT scan revealed scattered small nodules and ground-glass opacities with interlobular septal thickening in both lungs. The serum levels of surfactant A, surfactant D, and KL-6 were increased to 190 ng/ml (normal: 0-43.8), 360 ng/ml (normal: 0-110), and 4850 U/ml (normal: 0-500), respectively. Video-assisted thoracoscopic lung biopsy revealed eosinophilic amorphous material within alveoli and thickened alveolar septa, which is compatible with pulmonary alveolar proteinosis. Kidney biopsy exhibited membranous nephropathy (Stage I-II) accompanied by granular IgG deposition along the glomerular basement membrane. Although the patient refused treatment with granulocyte macrophage colony stimulating factor (GM-CSF) for pulmonary alveolar proteinosis, her proteinuria and the pulmonary lesion gradually diminished and disappeared after one year.
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PMID:Membranous nephropathy and pulmonary alveolar proteinosis. 1782 46

The acquired form of pulmonary alveolar proteinosis was determined in 3 patients, a woman of 31 and 2 men of48 and 38 years, respectively. Their symptoms consisted of progressive dyspnoea, with or without coughing and a tight feeling in the chest. Bronchoscopy with bronchoalveolar lavage yielded milky white, frothy material, and high resolution CT revealed parenchymal densification. All 3 patients were successfully treated with recombinant granulocyte-macrophage colony-stimulating factor (GM-CSF; sargramostim); in addition, the first and last patient underwent total pulmonary lavage. During the pregnancy of the woman, the GM-CSF treatment was suspended; this was resumed after parturition, which took place via caesarean section. Pulmonary alveolar proteinosis is a rare disease characterised by accumulation of surfactant in the alveoli. Until recently, the treatment consisted only of total lung lavage under general anaesthesia. It has recently been discovered that IgG autoantibodies play an important role in the development ofthe disease, namely in the accumulation of surfactant in the alveoli. IgG autoantibodies appear to neutralise the biological activity of natural GM-CSF, which leads to accumulation of used surfactant in the alveoli and a decrease of the pulmonary diffusion capacity. These cases and other publications from the past few years underline the important role of GM-CSF, in addition to a total lung lavage, in the treatment of pulmonary alveolar proteinosis.
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PMID:[Pulmonary alveolar proteinosis: a disease caused by surfactant accumulation, and new treatment with sargramostim]. 1866 60

To describe the clinical, radiological and pathological characteristics of idiopathic pulmonary alveolar proteinosis (I-PAP) and to evaluate the methods of diagnosis and treatment. Three patients were successfully diagnosed and treated in our hospital and the literature on the subject was reviewed. Three patients, two males and one female (mean age 46 years), were diagnosed averagely in 4 months. Two severe patients presented with progressive dyspnea and type I respiratory failure, and one mild patient only with dry cough and hypoxemia. Chest X-ray radiographs all showed perihilar "butterfly" shadow and chest CT scans showed diffused ground-glass opacities (GGO), typically with "map" changes and "crazy paving" patterns. All the patients underwent bronchoscope, branchoalveolar lavage fluid (BALF) had grossly opaque and/or milky appearance and its sediment was periodic acid-Schiff stain positive. Trans-bronchoscopic lung biopsy (TBLB) specimens were obtained and under light microscopy alveoli and some of the small bronchioles were filled with eosinophilic proteinaceous material with needle-like clefts. By electron microscopy numerous cellular debris and extracellular multilamellated bodies were found. Two severe patients were successfully treated with sequential whole-lung lavage and one required repeated lavages. I-PAP is rare and prone to be misdiagnosed. The radiological features may indicate the diagnosis and examinations of TBLB and BALF can make the accurate diagnosis. Whole-lung lavage is the most effective therapy by now and granulocyte-macrophage colony-stimulating factor (GM-CSF) may be beneficial in some patients.
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PMID:[Idiopathic pulmonary alveolar proteinosis: report of three cases and literature review]. 1893 23

Idiopathic pulmonary alveolar proteinosis (PAP) is a rare disorder in which lipoproteinaceous material accumulates within alveoli. There were few reports on Asian populations with idiopathic PAP. We retrospectively reviewed 38 patients with idiopathic PAP in Korea. We assessed clinical features, therapeutic efficacy and outcomes of whole lung lavage in patients with idiopathic PAP. The mean age at diagnosis was 52 yr. Eighty six percent of patients were symptomatic at diagnosis. Dyspnea and cough were the most common symptoms. Crackles were the most common physical examination finding. On pulmonary function test, a mild restrictive ventilatory defect was common, with a predicted mean forced vital capacity (FVC) of 77% and forced expiratory volume in one second (FEV(1)) of 84.6%. Diffusing capacity was disproportionately reduced at 67.7%. Arterial blood gas analysis revealed hypoxemia with a decreased PaO2 of 69.0 mmHg and an increased D(A-a)O2 of 34.2 mmHg. After whole lung lavage, PaO2, D(A-a)O2 and DL(CO) were significantly improved, but FVC and total lung capacity (TLC) were not different. This is the first multicenter study to analyze 38 Korean patients with idiopathic PAP. The clinical features and pulmonary parameters of Korean patients with idiopathic PAP are consistent with reports in other published studies. Whole lung lavage appears to be the most effective form of treatment.
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PMID:Clinical features and outcomes of idiopathic pulmonary alveolar proteinosis in Korean population. 2019 Oct 38

Pulmonary alveolar proteinosis (PAP) is a rare diffuse lung disease characterized by abnormal accumulation of surfactant-associated phospholipoproteinaceous material in the pulmonary alveoli. The clinical findings of slow-onset dyspnea or dyspnea on exertion and persistent dry cough are nonspecific; radiographic findings of "bat-wing configuration" and "crazy paving" appearance in high-resolution computed tomography are suggestive, but not diagnostic of PAP. The current gold standard of PAP diagnosis involves histopathological examination of alveolar specimens obtained from bronchoalveolar lavage and transbronchial lung biopsy. The characteristic histopathological features are intraalveolar periodic acid Schiff (PAS)-positive eosinophilic homogeneous material with well-preserved architecture ofalveolar septa. The current standard medical treatment of PAP involves the physical removal of the surfactant-associated phospholipoproteinaceous alveolar deposit by whole lung lavage, which causes clinical and radiological improvement in a majority of patients. Some patients have been successfully treated with recombinant granulocyte-macrophage colony-stimulating factor (GM-CSF).
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PMID:Pulmonary alveolar proteinosis: an overview for internists and hospital physicians. 2046 23

Pulmonary alveolar proteinosis (PAP) has been associated with the immunosuppressant sirolimus in transplant patients. PAP is a progressive lung disease characterized by the accumulation of surfactant-like material in the lungs leading to decreased pulmonary function with shortness of breath and cough as common symptoms. We report a rare case of sirolimus-associated PAP in a kidney transplant recipient with a history of end-stage renal disease secondary to haemolytic uraemic syndrome (HUS) and review of the literature. Discontinuation of sirolimus and initiation of tacrolimus led to resolution of PAP without recurrence of HUS.
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PMID:Pulmonary alveolar proteinosis in a kidney transplant: a rare complication of sirolimus. 2048 3

Secondary pulmonary alveolar proteinosis (PAP) is rare but may occur in association with malignancy, certain infections, and exposure to inorganic or organic dust and some toxic fumes. This case report describes the second recorded case of PAP due to exposure to cotton dust. A 24-year-old man developed PAP after working as a spinner for eight years without respiratory protection. He was admitted as an emergency patient with very severe dyspnea for four months and cough for several years. Chest X-ray showed bilateral diffuse alveolar consolidation. He died 16 days later, and a diagnosis of acute pulmonary alveolar proteinosis was made at autopsy. The histopathology demonstrated alveoli and respiratory bronchioles filled with characteristic periodic acid Schiff-positive material, which also revealed birefringent bodies of cotton dust under polarized light. Secondary PAP can be fatal and present with acute respiratory failure. The occupational history and characteristic pathology can alert clinicians to the diagnosis.
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PMID:Acute pulmonary alveolar proteinosis due to exposure to cotton dust. 2053 3

A 26-year-old male presented with complaints of dry cough of six months and progressive breathlessness of three months duration. He was coughing out milky white sputum for two months and had lost 12 kg weight in two months. He had an evening rise in temperature of one month duration. Clinically, the patient was in respiratory distress and the respiratory system examination revealed bilateral velcro crackles. High resolution computed tomography chest showed bilateral diffuse reticulonodular opacities and "Crazy Paving" pattern suggestive of alveolar proteinosis. Broncho alveolar lavage showed eosinophilic granular material, which was periodic acid-Schiff positive. Open lung biopsy was done to confirm the diagnosis and the histopathologic examination revealed eosinophilic secretions with granular appearance suggestive of pulmonary alveolar proteinosis. Subsequently, patient underwent bilateral sequential whole lung lavage under general anesthesia. Patient showed marked clinical and radiological improvement after sequential whole lung lavage.
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PMID:Whole lung lavage for pulmonary alveolar proteinosis. 2053 70

A 37-year-old man was admitted with complaints of continuous cough and sputum production for 1 month. Computed tomography (CT) of the chest revealed a solitary mass with a cavity in the apex of the left lung and bilateral ground-glass opacities (GGO). Thereafter, the patient complained of fever, and an increase in the mass shadow size and expansion of the ground-glass opacities were observed on serial CT. The patient was given diagnoses of pulmonary nocardiosis and pulmonary alveolar proteinosis by bronchoscopic examination. Serum anti-GM-CSF antibody tests were positive. On the basis of these findings, we diagnosed autoimmune pulmonary alveolar proteinosis. After beginning antituberculosis drugs and antibiotics, the tumor shadow and GGO reduced. The pulmonary alveolar proteinosis rapidly worsened on exacerbation of the pulmonary nocardiosis, but prompt overall improvement was obtained after treating the latter. We believe this to be a valuable case for examining the time progression of autoimmune pulmonary alveolar protein syndrome, because of the clinical course of the exacerbation, and the improvement in the pulmonary alveolar proteinosis after treatment of pulmonary nocardiosis.
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PMID:[A case of pulmonary alveolar proteinosis which initially deteriorated rapidly with exacerbation of pulmonary nocardiosis, responded promptly to treatment of the pulmonary nocardiosis]. 2080 74

Pulmonary alveolar proteinosis (PAP) is a rare disease that affects young population usually in the age group of 20-40 years, characterized by the deposition of lipoproteinacious material in the alveoli secondary to abnormal processing of surfactant by macrophages. We report a case of a 15-year-old female who had history of cough with sputum for 3 days along with fever. She was seen in another hospital and was treated as a case of pneumonia where she received antibiotic but with no improvement. Computerized tomography (CT) chest showed diffuse interlobular septal thickening in the background of ground glass opacity giving a picture of crazy paving pattern which was consistent with the diagnosis of PAP. The patient was scheduled to undergo, first right-sided whole lung lavage (WLL) under general anesthesia. Endobronchial intubation using left sided 37 Fr double lumen tube. Continuous positive airway pressure (CPAP) as described in our previously published report was connected to the right lumen of the endobronchial tube. CPAP ventilation was used during the suctioning of lavage fluid phase in order to improve oxygenation. WLL was done using 5 L of warm heparinized saline (500 i.u/litre). The same procedure was repeated on the left side using 6 L of heparinized normal saline solution. In conclusion, anesthesia in alveolar proteinosis for patients undergoing WLL is challenging to the anesthesiologist. It requires meticulous preoperative preparation with antibiotics, mucolytics and chest physiotherapy. Also it requires careful intraoperative monitoring and proper oxygenation especially during the suctioning phase of the lavaged fluid. With this second case report of successful anesthetic management using the modified CPAP system we recommend with confidence the application of CPAP ventilation to improve oxygenation during WLL.
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PMID:Continuous positive airway pressure ventilation during whole lung lavage for treatment of alveolar proteinosis -A case report and review of literature. 2165 22


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