Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0034069 (pulmonary fibrosis)
 7,050 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Respiratory symptoms and spirometric pulmonary function data [i.e., first-second forced expiratory volume (FEV1.0) and forced vital capacity (FVC)] for 128 (30%) males who were exposed to alkyl benzene sulphonate in a detergent factory and for 56 (76%) unexposed workers in the same factory are reported herein. Exposed subjects had been employed for 1 month to 15 yr, and they generally complained of cough and mucus secretions, nasal catarrh, chest pain, and breathlessness. Unexposed workers had been employed for 1 month to 13 yr and had a significantly lower (P less than .001) frequency of symptoms, as well as significantly higher (.01 greater than P greater than .001) FEV1.0 and FVC than the exposed workers. The reduction in pulmonary function of exposed subjects from the predicted was significantly higher (.01 greater than P greater than .001) than that experienced by the unexposed subjects. There was a significant 8-hr workshift depression in lung function. There was radiological evidence of pulmonary fibrosis, but lack of pre-employment chest radiographs renders this inconclusive. Respiratory symptoms in exposed subjects decreased with duration of employment, which probably indicates the exodus from the work force of those who could not tolerate the nonsoapy detergent.
 ...
PMID:Pulmonary function of exposed and control workers in a Nigerian nonsoapy detergent factory. 672 84

Six patients with breast cancer developed pulmonary toxicity following mitomycin therapy. The presenting symptoms were shortness of breath and a dry cough. The radiological pictures varied from a normal chest x-ray to extensive bilateral pneumonitis. The histological findings consisted of diffuse alveolar damage progressing to interstitial pulmonary fibrosis. Corticosteroid therapy resulted in complete resolution of pneumonia in one patient. Respiratory symptoms improved in 3 patients following discontinuation of the drug. Two patients with extensive bilateral pneumonitis, who were not treated with steroids, died of respiratory failure. Mitomycin-induced lung toxicity appears to be reversible with the discontinuation of drug and the administration of corticosteroids. Lung biopsy is necessary in order to rule out other diagnoses.
 ...
PMID:Pulmonary toxicity of mitomycin. 735 Oct 10

A variety of common and uncommon conditions affect the trachea. Respiratory symptoms rarely occur until there is a 50% narrowing of the trachea. Chondromalacia, sometimes seen in conjunction with congenital tracheoesophageal fistula, can be identified with fluoroscopy. Patients with tracheal narrowing who undergo general anesthesia are at great risk for life-threatening acute airway obstruction after removal of the tube. Tracheal narrowing is associated with calcified mediastinal and hilar masses in fibrosing mediastinitis. Tracheal widening has been reported in 30% of patients with pulmonary fibrosis. Primary tracheal neoplasms are rare. In adults 90% of such lesions are malignant, but in children 90% are benign; these benign lesions include squamous cell papilloma and hemangioma. Malignant involvement of the trachea is usually secondary to invasion from adjacent lung, larynx, esophagus or thyroid tissue. Because misplaced tubes are associated with several complications, the radiologist must confirm the location of all tubes. Displacement of the endotracheal tube from full extension to flexion averages 2 cm. Therefore, the tube's tip should be at least this far above the carina to avoid insertion of the tube into the mainstem bronchus. Malpositioning of feeding tubes may cause pneumothorax. Tracheal trauma may be missed unless the radiologist is highly suspicious, as would occur for patients with unresponsive pneumothorax. Observation of the hyoid bone above the third cervical vertebra suggests transection of the cervical trachea.
 ...
PMID:The trachea: pathologic conditions and trauma. 850 26

Bird fancier's lung (BFL) is one of the most common types of hypersensitivity pneumonitis. Nevertheless, the criteria for diagnosing this condition are not standardized. The current study is an in-depth investigation into the clinical characteristics of BFL in the largest series examined for this purpose by a single group, to our knowledge, taking into account the acute, subacute, or chronic clinical presentation. From 1977 to 2003, BFL was diagnosed in 86 patients using a homogeneous protocol. Data from the clinical history and physical examination were analyzed, as well as the results from the following complementary examinations: laboratory analyses, specific serum IgG antibodies determination, chest X-ray, chest computed tomography (CT), pulmonary function testing, immediate hypersensitivity skin testing, delayed cutaneous hypersensitivity testing, bronchofibroscopy with bronchoalveolar lavage (BAL) and/or transbronchial biopsy, bronchial challenge testing, and surgical lung biopsy. In addition, clinical and epidemiologic characteristics were determined in a control group of 60 pigeon breeders who did not meet the diagnostic criteria of BFL. Eighty-six patients (21 men and 65 women) with a mean age of 47 years were studied. Seven (8%) patients were younger than 15 years of age at the time of the diagnosis. In 3 cases, the disease was caused by exposure to feather-filled bedding. Nearly 1 in 5 patients was diagnosed in the chronic phase of the disease. The mean diagnostic delay was 1.6 years overall, and 3.2 years in patients diagnosed in the chronic phase of the disease. Among the 17% of patients with chronic disease, the mean interval from initiation of exposure to diagnosis was 16 years, a higher value than in the acute or subacute presentation forms. Dyspnea and cough were the most common clinical symptoms (98% and 82%, respectively), and nearly 25% had grade III or IV dyspnea at diagnosis. Only 18% of patients experienced chest tightness, a symptom classically considered to be frequent in this condition. Erythrocyte sedimentation rate was elevated (>30 mm/h) in 44% of patients. Urinary calcium was elevated in 20% of patients. Angiotensin-converting enzyme was not elevated in any of the patients in which it was measured. Lactate dehydrogenase increases were found in 51% of patients. Specific IgG antibodies to avian antigens were documented in 92% of BFL patients, but also in 87% of pigeon breeder controls. The most frequent radiologic finding was an interstitial pattern in 79% of patients. Common chest CT features were ground glass areas (68%) and a mosaic pattern (61%); areas of emphysema were found in 7/41 (17%) patients, 5 of whom had never smoked. Two patients had a CT pattern of pulmonary fibrosis indistinguishable from idiopathic pulmonary fibrosis. Immediate hypersensitivity skin testing with bird sera and pigeon bloom was positive in 78% and 100% of BFL patients, respectively, and in 64% and 88% of control pigeon breeders, respectively. Almost one-third of the patients (29%) presented an anergic response on delayed cutaneous hypersensitivity testing. Restrictive ventilatory impairment was the most frequent functional pattern (77%), although 9% and 4% showed a pure obstructive and mixed pattern, respectively. The carbon monoxide diffusing capacity was decreased (<80% of the predicted value) in 85% of cases. Forty-one percent of patients had PaO2 <60 mm Hg at diagnosis when blood gas analysis was performed. Lymphocytosis (>20% lymphocytes) was documented in 83% of patients who underwent BAL, with a similar frequency in the 3 presentation forms: 70% acute, 89% subacute, and 85% chronic. In addition, inversion of the CD4/CD8 ratio (<1) was observed in 62% of the patients, but 38% of cases showed a CD4 predominance. The characteristic triad of histopathologic findings in hypersensitivity pneumonitis was found in only 9% of patients undergoing transbronchial biopsy, but at least 1 of these findings was seen in 69%. Surgical lung biopsy was undertaken in 14/86 (16%) patients; the complete triad was observed in 50% and at least 1 finding in 100%. In 54/86 (63%) patients, the diagnosis was confirmed by bronchial challenge testing, a test with a sensitivity of 92% and specificity of 100%. BFL is a potentially severe disease that can progress to respiratory failure secondary to pulmonary fibrosis or chronic obstructive pulmonary disease, as a form of chronic occupational respiratory disease. Respiratory symptoms in exposed patients, including children and adults who have only 1 pet bird at home, should raise the suspicion of BFL. Diagnosis in the chronic phase is frequent, and the delay to diagnosis was greatest in these cases. Elevated urinary calcium, lactate dehydrogenase, and erythrocyte sedimentation rate in a bird fancier may constitute a combined marker for suspected BFL. Chest CT frequently discloses emphysema and a pattern of idiopathic pulmonary fibrosis in some patients. An anergic response on delayed cutaneous hypersensitivity testing is not infrequent. The presentation with respiratory failure and the predominance of CD4 T lymphocytes in some patients' BAL are both remarkable. Lymphocytosis on BAL also persists in the chronic phase of the disease. Bronchial challenge testing has a high diagnostic yield, and surgical lung biopsy is not needed to reach the final diagnosis in the vast majority of cases.
 ...
PMID:Bird fancier's lung: a series of 86 patients. 1834 8

Dilatation and oesophageal body aperistalsis in achalasia can lead to stasis which in turn can induce repeated microaspiration. It is therefore conceivable that patients with achalasia may also have abnormalities in lungs secondary to repeated episodes of microaspiration. There is a lack of systematic study on involvement of lungs in patients with achalasia. Thirty patients with achalasia underwent pulmonary function tests (spirometry, and carbon mono-oxide diffusion capacity) and high resolution computerized tomography (HRCT) of the chest. The mean age of patients and mean duration of disease were 33.5 +/- 10.9 years and 28.1 +/- 27.3 months respectively. Regurgitation was present in 22 (73.3%) of them. Respiratory symptoms in them were dry cough in 17 (56.6%), and chest pain in 18 (60%). The oesophagus was dilated in 26 (86.6%) and 13 (43.3%) had residue in oesophagus. Sixteen (53.3%) patients had either anatomical changes as seen on HRCT or functional changes as observed on pulmonary function tests. Of those with functional abnormalities, five (16.6%) and one (3.3%) had restrictive and obstructive airways disease respectively. While evidence of tracheo-bronchial compression by dilated oesophagus was present in eight (26.6%), 10 (33.3%) patients had parenchymal lung disease [nodular opacities in five (16.6%), ground glass appearance six (20%), patchy pulmonary fibrosis five (16.6%), air trapping two (6.6%), consolidation and bronchiectasis one (3.3%) each]. There was a significant association between presence of regurgitation and dilatation of oesophagus (P = 0.032). More than half (53.3%) of patients with achalasia have structural and/or functional abnormalities in lungs.
 ...
PMID:Structural and functional abnormalities in lungs in patients with achalasia. 1922 59

Pulmonary manifestations of SSc are leading causes of disease-related morbidity and mortality. Key clinical issues relate to the early detection of fibrotic lung disease, the interpretation of its significance, and decisions on when to start therapy. Respiratory symptoms are common in patients with SSc, but physical examination often fails to establish if the underlying cause is interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), impaired locomotion due to systemic disease or loss of fitness. Impaired lung function is usually evident on pulmonary function testing, with the pattern of functional impairment often discriminating usefully between ILD and PAH. The presence of PAH can be indicated by echocardiography and must be confirmed by right heart catheterization. Whereas chest radiographs detect established ILD, high-resolution CT can identify earlier or very mild inflammatory changes. Treatment options for ILD are limited to immunosuppressive agents, notably cyclophosphamide, often given in combination with low-dose prednisolone. Recent studies have shown that cyclophosphamide is effective in stabilizing pulmonary function--especially in patients with severe fibrotic disease--and in improving health-related quality of life. Progression of pulmonary manifestations and responses to treatment are best monitored using pulmonary function testing. For patients with severe end-stage pulmonary fibrosis, lung transplantation may offer a viable alternative therapeutic option.
 ...
PMID:Pulmonary complications: one of the most challenging complications of systemic sclerosis. 1948 23