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)

Pulmonary alveolar proteinosis is characterized by an amorphous lipoproteinaceous material filling the acini of the lung, resulting in hypoxemia. At present, the most effective treatment for alveolar proteinosis is lung lavage. Here, we report the first case in Taiwan treated with this method. A 38-year-old male with a 6-month history of cough and increasing breathlessness was transferred from another hospital. On admission, his arterial oxygen tension was 51 mmHg when breathing room air. Chest X-ray films showed diffuse infiltration of both lung fields. Transbronchoscopic lung biopsy was consistent with alveolar proteinosis, therefore the patient was submitted to pulmonary lavage. Under general anesthesia, the patient was intubated with a left-sided double lumen endotracheal tube, and lavage of the right lung was repeated with 0.9% saline until the washing became clear. At the end of the procedure, the patient was extubated after making strong respiratory efforts. Several days later, the left lung was lavaged using the same technique. The mechanical washing of amorphous material from alveoli by pulmonary lavage is of great benefit in relieving hypoxemia. Judicious use of lung lavage is effective and safe for patient with alveolar proteinosis.
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PMID:[Pulmonary lavage for alveolar proteinosis. A case report]. 272 86

A case of pulmonary alveolar proteinosis associated with tuberculosis of the lung is reported. The patient had fever, cough, and pulmonary cavities. Sputum cultures for Mycobacterium tuberculosis were positive on three occasions. Thirty-three months later, diffuse bilateral lower lobe infiltrates developed. An open lung biopsy revealed filling of the alveoli by a periodic-acid-Schiff-positive amorphous granular material. There is a significant association between this disease and various infectious and fungal agents, but its association with tuberculosis is rare. It has been reported only three times before in the English literature. This appears to be the fourth documented case with this association. A review of the literature with special emphasis on tuberculosis associated with alveolar lipoproteinosis is presented. The electron-microscopic findings are also described.
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PMID:Association of pulmonary alveolar lipoproteinosis with mycobacterial infection. 742 32

The clinical features of 68 Japanese patients (53 men and 15 women; mean age 44 years) with primary pulmonary alveolar proteinosis were reviewed. Pulmonary alveolar proteinosis was diagnosed from histologic findings after open lung biopsy (n = 7) or transbronchial lung biopsy (n = 61). Major symptoms were a dry cough (24.2%) and dyspnea or shortness of breath on exertion (51.5%), but one third of the patients were asymptomatic. Crackles were audible in 30% of the patients, but clubbing (6%) and cyanosis (4%) were rare. Ten patients had been occupationally exposed to dust. Slightly less than half (46%) of the patients first presented with symptoms, and the remainder (54%) first presented with abnormal pulmonary infiltrates seen on chest roentgenograms taken during general health examinations. Many patients had abnormally high levels of LDH and CEA in serum (62% and 63%, respectively). Restrictive pulmonary dysfunction (%VC < 80%) was seen in 31% of the patients, an abnormally low DLco (%DLco < 70%) was seen in 62%,m and hypoxemia (PaO2 < 80 mmHg) was seen in 67%. Arterial blood gas tension was closely correlated with the severity of disease in these patients. Chest roentgenograms usually showed bilateral symmetric alveolar infiltrates, mainly distributed from hilar areas toward the pleura, but on CT scans many of the shadows were mixed with alveolar and interstitial infiltrates of various extent along the pulmonary arteries and bronchi. There was no apparent relation between chest roentgenographic findings and chest CT findings in these patients. Neither the extension nor other characteristics of shadows in the chest roentgenograms and chest CT scans were closely related to symptoms, laboratory data, or pulmonary function in these patients. Symptoms were alleviated and chest roentgenographic findings improved in 82% of the 51 patients who underwent therapeutic bronchoalveolar lavage, and in 94% of the 17 patients who did not undergo that procedure. In patients who underwent therapeutic bronchoalveolar lavage and also in those who recovered spontaneously, both diffusing capacity and blood gas values improved significantly. When compared to the patients who did not undergo therapeutic bronchoalveolar lavage, significantly more of those who did undergo that procedure has initial PaO2 values below 60 mmHg, and fewer of them had values greater than 80 mmHg. Thus, a PaO2 below 60 mmHg may be an indication for therapeutic bronchoalveolar lavage in patients with this disease. During the follow-up period (mean 5 years, range 2 months to 23 years), four patients had pneumothorax and none died of pulmonary alveolar proteinosis.
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PMID:[Primary pulmonary alveolar proteinosis--clinical observation of 68 patients in Japan]. 747 63

A 49-year-old woman with acute lymphocytic leukemia (ALL) who had been intermittently treated with chemotherapy during a 3-year period developed cough and expectoration of increasing severity. Chest X-ray film revealed miliary shadows and bilateral hilar enlargement. Milky fluid was obtained by bronchoalveolar lavage. The intensity of these shadows showed temporary decrease, but thereafter progressively increased until the time of the patient's death. Autopsy findings were consistent with pulmonary alveolar proteinosis and systemic aspergillosis. Secondary alveolar proteinosis is rare, and in most reported cases is associated with hematological malignancy. To our knowledge, only 45 cases in which it was associated with hematological disorders have been reported. The examination of a patient with any hematological disorder, however, should include differential diagnosis of chest X-ray abnormalities to exclude secondary alveolar proteinosis, which can be lethal if left untreated.
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PMID:[An autopsy case of acute lymphocytic leukemia associated with secondary pulmonary alveolar proteinosis and systemic aspergillosis]. 831 8

Pulmonary alveolar proteinosis (PAP) is an uncommon disease in which alveoli are progressively filled with surfactant-related material. Although a definitive diagnosis is usually made by an open lung biopsy, bronchoalveolar lavage (BAL) cytology may play a decisive role in the clinical work-up of these patients, and, in some cases, may spare a patient a more invasive diagnostic procedure. The authors present three patients in whom BAL cytology specimens contained the characteristic (although not specific) globules of amorphous proteinaceous PAS-positive material accompanied by only rare background macrophages and inflammatory cells. The patients include a 40-year-old man with an 8-year history of fever of unknown origin, a 30-year-old man with a chronic nonproductive cough, and a 6-year-old boy diagnosed at 5 months of age with osteopetrosis and hypogammaglobulinemia who subsequently developed a disseminated Mycobacterium avium-intracellulare infection. All specimens stained with Gomori methenamine silver (3) and Ziehl-Neelsen (2) were negative for microbial organisms, Ultrastructural examination of two specimens revealed the characteristic lamellar structures of surfactant, increasing diagnostic specificity. Lung biopsies and/or autopsy subsequently confirmed the diagnosis in all three cases. The characteristic cytologic and ultrastructural features of PAP in BAL specimens are presented along with the morphologic differential features of other entities which potentially could be confused with PAP.
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PMID:Bronchoalveolar lavage cytology in pulmonary alveolar proteinosis. 885 40

Partial liquid ventilation (PLV) has been applied in various pulmonary diseases. We describe the use of partial liquid ventilation as a lavage method following normal saline (NS) lavage in an infant with pulmonary alveolar proteinosis (PAP) and severe hypoxemia. A 6 weeks old 3.4 kg former 36 weeks gestation boy on supplemental oxygen was transferred to our NICU with persistent tachypnea, dry cough, and increasing oxygen requirements. A lingular open lung biopsy revealed PAP. He developed progressive respiratory failure requiring ventilatory support, necessitating conventional NS lavage, followed by lung lavage with perflubron (LiquiVent; Alliance Pharmaceutical Corp. and Hoechst Marion Roussel) while on venovenous extracorporeal life support (ECLS). Lung lavage with NS and perflubron yielded minimal cloudy effluent. Gas exchange and pulmonary function deteriorated following NS lavage and attempts to discontinue ECLS were poorly tolerated. In contrast, tidal volume, PaO2, and pulmonary compliance increased after PLV, while the (A-a) D(O2) decreased to a point where ECLS was no longer required. Once perflubron was added repeatedly to the ventilator circuit to correct for evaporation over the 4 days of PLV. Cardiovascular status remained stable for several days; however, eventually he required reinitiation of ECLS and more mechanical ventilatory support with each trial off ECLS. He was maintained on high pressures and FiO2 without any possibility to wean him from mechanical ventilation. Life support was withdrawn 1 month after admission. The survival from PAP in infants remains dismal, even with total lung NS lavage. While both NS and perflubron lavage in this patient were not effective in removing the proteinaceous alveolar debris, PLV following NS lavage was associated with an improvement in gas exchange and lung compliance.
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PMID:Liquid ventilation in an infant with pulmonary alveolar proteinosis. 981 Oct 79

Pulmonary alveolar proteinosis (PAP) is a rare disease characterised by the accumulation of proteinaceous material within alveoli. In order to evaluate the clinical features and the course of PAP we reviewed 7 cases (2F/5M) diagnosed during a 11-year period (1989-1999). The mean age of patients was 40.7 +/- 11.2 years. Diagnosis was obtained by open lung biopsy in all cases. Clinical findings included dyspnea (43%), cough (28%) and crackles (28%). Lung function tests were normal in 5 cases and showed a moderate restrictive pattern in 1 and mild airflow obstruction in 1. Three patients had reduced Dlco (mean was 63% of predicted). Four patients had hypoxemia at rest. Chest X-ray revealed bilateral alveolar opacities (71%), involving perihilar areas and lower lobes. HRCT scans demonstrated diffuse ground glass opacities (83%) with interlobular septa thickening (50%). Three patients were treated with repeated segmental BAL (2 improved). The spontaneous partial remission occurred in 4.
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PMID:[Pulmonary alveolar proteinosis]. 1127 75

Pulmonary alveolar proteinosis (PAP) is a rare, diffuse infiltrative lung disease of unknown etiology characterized by accumulation of abnormal lung surfactant in the alveoli and distal airways. Although it was first described in 1958, few large scale studies have been published. The diagnosis is conventionally based on lung biopsy and the value of less invasive procedures such as bronchoalveolar lavage (BAL) remains controversial. A multi-center retrospective study was conducted in an attempt to update epidemiological, clinical and long term outcome and to assess the usefulness of chest CT scan and BAL in the management of a large homogeneous series of 41 patients. Inclusion criteria required a clinical picture compatible with PAP and either histological confirmation, or a BAL with a milky macroscopic appearance coupled with positive staining to PAS. Mean age was 48.8 years with a 4: 1 male predominance. The prevalence of smokers (80%) and occupational exposure to various inhaled dusts (39%) was high. The clinical, roentgenographic and functional features were in keeping with previous series. Worsening dyspnea was the most common symptom. Other common manifestations were dry cough, recurrent lung infections, weight loss, finger clubbing, crackles and cyanosis. LDH serum levels were increased in 51% of cases. Lung function tests usually showed a mild restrictive ventilatory defect with hypoxemia and reduced DLCO. Chest X ray revealed bilateral ground glass opacities with consolidation or air bronchogram involving peri-hilar or lower lobes. Chest CT scan, performed in 38 patients, revealed a similar pattern in most of them with diffuse intra-alveolar ground glass opacities (33/38) and interlobular thickening (35/38), resulting in a crazy paving appearance in the typical geographic pattern (32/38). This latter aspect was highly suggestive of the diagnosis. Segmental BAL led to a definitive diagnosis in 62% and lung biopsy was only required in 38% of cases. These data suggest that careful BAL analysis can be sufficient to provide a diagnosis without the need for a lung biopsy. During follow-up, spontaneous remission or improvement was reported in 26%. Segmental (n=3) or whole lung lavage (n=22) was required in 62.5% with a favorable course (complete remission 37%, improvement 34%, stable course 21%) in 92% of the cases. In this series the overall prognosis for PAP remained good, although unpredictable.
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PMID:[Pulmonary alveolar proteinosis]. 1252 3

The current mainstay of treatment for pulmonary alveolar proteinosis (PAP) is whole-lung lavage. Therapy with granulocyte-macrophage colony-stimulating factor is a possibility, although its long-term safety has not been determined. An alternative procedure is selected lobar lavage by fiberoptic bronchoscopy (FOB). We report here our experiences with lobar lavage by FOB in treating three patients with PAP. PAP was diagnosed in three patients (two men, one woman) who had dyspnea and hypoxemia after undergoing open-lung biopsy. The patients underwent lobar lavage by FOB under local anesthesia. The bronchoscope was wedged into a lobar bronchus. Approximately 2,000 mL warm normal saline solution was instilled via syringe in 50-mL aliquots through a fiberoptic bronchoscope. After undergoing multiple lobar lavages, two patients showed clinical, physiologic, and radiologic improvement. The third patient, who had more advanced disease, showed improvement only in oxygenation. The major complications were severe cough and hypoxemia during lavage. Our experience suggests that bronchoscopic lobar lavage is simple and safe, and may find application in patients in whom a whole-lung lavage with generalized anesthesia may be hazardous, and in patients with less advanced disease whose proteinaceous substances can be removed with a small volume of lavage fluid.
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PMID:Pulmonary alveolar proteinosis: treatment by bronchofiberscopic lobar lavage. 1237 31

A 53-year-old woman with refractory acute myeloid leukemia had a cough and chest pain. Chest X-ray and computed tomography demonstrated a cavity for which antibiotics, antituberculosis and antifungal agents were not effective. A diagnosis of pulmonary aspergillosis and pulmonary alveolar proteinosis (PAP) was made on the basis of the detection of aspergillus using transbronchial lung biopsy and PAS-positive materials in the sputum. Even though some cases with PAP in hematological malignancy have been reported, the diagnosis of PAP was obtained in most of them at autopsy. In our experience three of seven cases of hematological malignancy had concomitant occurrence of aspergillosis and PAP. We should therefore pay particular attention to the possibility of PAP in patients with hematological neoplasia exhibiting pulmonary fungal infection, especially aspergillosis.
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PMID:[Invasive aspergillosis and pulmonary alveolar proteinosis in acute myeloid leukemia]. 1241 96


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