Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:6.2.1.7 (BAL)
 1,977 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

It is well known that silica exposure leads in an experimental model to the development of an acute fibrotic process. In human beings two main observations have already been done: (1) silica exposure is frequently associated with the development of connective tissue disease (CTD), especially progressive systemic sclerosis; (2) 10 to 20% patients with CTD developed pulmonary fibrosis. In this context we report 26 cases of coal miners who presented with clinical, radiological, biological and functional characteristics mimicking idiopathic pulmonary fibrosis (IPF), with or without associated coal worker's pneumoconiosis (CWP). All were men; mean age was 68 +/- 9.2 years. Twenty-three were smokers. Duration of exposure was 28.8 +/- 9.1 years. All the patients had dyspnea (stage III, IV in the NHYA classification) and diffuse crackles. Eleven out of 26 had finger clubbing. Computed tomography showed honeycombing (23 cases), and/or ground glass opacities (6 cases) with bronchiectasis (3 cases) predominant in the lower lobes; 19 had radiological signs of CWP, micronodules (n = 16) and nodules (n = 3) predominant in the upper lobes. BAL exhibited an increased % of neutrophils (11.9 +/- 16.1%). Lung function demonstrated a restrictive pattern (TLC = 73 +/- 15.6% and VC = 80 +/- 18% of predicted values) associated with a decreased DLCO (51.8 +/- 23.6% of predicted values) and hypoxemia (at rest = 66.5 +/- 11.2 mmHg, upon effort = 56 +/- 12 mmHg). Lung biopsies were performed in four cases and demonstrated interstitial fibrosis of intraalveolar septum with an accumulation of immune and inflammatory cells similar to the one described in IPF. The association between IPF and silica exposure with or without associated CWP points out the problem of legal recognition of idiopathic-like pulmonary fibrosis as a complication of the occupational exposure of coal workers.
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PMID:["Primary" diffuse interstitial fibrosis in coal miners: a new entity? Study Group on Interstitial Pathology of the Society of Thoracic Pathology of the North]. 941 11

A 14-year-old girl developed skin rash, fever and dyspnea. The chest roentgenogram showed diffuse reticulonodular infiltration. Pulmonary function tests revealed mild restrictive defect and blood oxygen pressure at 71 mm Hg. BAL showed increased cell counts with lymphocytosis at 15% and neutrophilia at 3%. Outcome was good after carbamazepine withdrawal and without corticosteroid therapy. Relapse was observed after patient-induced rechallenge.
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PMID:[Respiratory manifestations of carbamazepine. Apropos of a case]. 961 32

Aspergillosis is an infrequent but commonly fatal infection among HIV-infected individuals. We review 342 cases of pulmonary Aspergillus infection that have been reported among HIV-infected patients, with a focus on invasive disease. Invasive pulmonary aspergillosis usually occurs among patients with <50 CD4 cells/mm3. Major predisposing conditions include neutropenia and steroid treatment. Fever, cough, and dyspnea are each present in >60% of the cases. BAL is often suggestive, but biopsy specimens are necessary for definite diagnosis. Amphotericin B is the mainstay of treatment and mortality is > 80%. Avoiding neutropenia and judicious use of steroids may be helpful in prevention. Aggressive diagnostic approach, early initiation of treatment, adequate dosing of antifungals, and close follow-up may improve the currently dismal prognosis.
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PMID:Pulmonary aspergillosis and invasive disease in AIDS: review of 342 cases. 967 77

A nonsmoking 54-yr-old man, employed in a peat moss packaging plant, developed dyspnea and recurrent fever. The diagnosis of hypersensitivity pneumonitis (HP) was made. Thirteen of 14 coworkers and 13 nonexposed control subjects were studied. Five workers were nonsmokers, two were minimal smokers, and six were smokers. HP was found in another subject. Monocillium sp. and Penicillium citreonigrum, 4.6 x 10(7) CFU/g, were found in the peat moss. Three nonsmokers, the two minimal smokers (including the subject with HP), and the index case had antibodies to these microorganisms; none of the six heavy smokers had antibodies. Serum TNF-alpha was higher in the workers than in the control subjects (0.930 +/- 0.177 versus 0. 350 +/- 0.076). Three of the four asymptomatic seropositive workers and two seronegative smokers were further evaluated. All three seropositive workers had normal lung functions and CT but they all had a lymphocytic alveolitis (30, 34, and 68% lymphocytes in their bronchoalveolar lavage [BAL]). The smokers had normal lung functions, CT, and percentage of BAL lymphocytes (3 and 13%). This study identified a previously unrecognized work environment that can lead to HP and documented a protective effect of smoking on the response to antigens.
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PMID:Hypersensitivity pneumonitis in peat moss processing plant workers. 970 Jan 14

The clinical syndrome "Bronchiolitis Obliterans Organising Pneumonia" (BOOP) has to be considered in patients with a flu-like illness since some weeks, fine crackles, and on chest X-ray bilateral patchy infiltrates. There is no response to antibiotics. BOOP is essentially idiopathic, but associations to other conditions exist. Lung function is often restrictive; biochemistry is not pathognomonic. BAL shows a mixed cellular pattern. The gold standard for pathologic diagnosis is open or thoracoscopic lung biopsy. However, a BOOP pattern or reaction is often seen on histologic specimens without the clinical-radiologic features of the BOOP-entity. Therapy consists of corticosteroids, which have to be prescribed for a long time at a rather high dose. Recurrence is frequent, but prognosis is good. Evolution to respiratory insufficiency and death is rare and may occur in rapidly progressive BOOP. This study reports on 11 cases (6 males/5 females) of clinical-pathological BOOP-syndrome (mean age 58 yrs, range 17-73 yrs), with an unexpectedly high mortality rate of 36% (4 cases). The disease was idiopathic in 7, and was associated with intake of amiodarone (in 1), with past Mycoplasma pneumonia (in 1) and with connective tissue disease (in 2). There was a history of a flu-like syndrome, cough and dyspnea of a mean duration of 4 months (range 1 week to 8 months). Lung function was mostly restrictive or/and obstructive with a diffusing capacity ranging between 47 and 95% predicted; there was hypoxia in about half of the patients. Chest X-ray and computed tomography (CT) scan showed a patchy consolidation with linear opacities (unilateral in 4 patients, bilateral in 5) and/or a ground glass pattern (in 4 patients), and a focal pseudo-tumoral lesion (in 1). Bronchoalveolar lavage showed a variable pattern of mixed, or eosinophilic or neutrophilic alveolitis. Histologic diagnosis was based on open lung biopsy (in 3), on thoracoscopic biopsy (in 2), on transbronchial biopsy (in 2), on wedge resection of the nodular lesion (in 1) and on postmortem lung biopsy (in 3). One patient recovered spontaneously, 1 remained cured after resection of the focal lesion, 7 were treated with 16-125 mg methylprednisolone (of whom 3 had a temporary flare-up during tapering the corticosteroids and 2 died after 1 and 3 months due to infectious complications), 2 died due to rapidly progressive BOOP.
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PMID:Bronchiolitis obliterans organising pneumonia. A report of 11 cases and a review of the literature. 986 59

The purpose of this study was to describe the clinical features at onset and outcome and the diagnostic approach in subjects with bronchiolitis obliterans-organizing pneumonia (BOOP). Over a 7-year period we observed 78 cases of biopsy-proven bronchiolitis obliterans-organizing pneumonia, in which well documented clinical and radiographic data were available. The final diagnosis of BOOP was validated when patients presented: (i) negative microbiological analysis on BAL fluid; (ii) a well documented improvement either spontaneous, or after steroid treatment or (iii) cases with progressive respiratory failure and increasing radiographic shadows, an open lung biopsy or autopsy that excluded other entities. There were 42 males and 36 females; the mean age was 61+/-12 years (range 12-85 years). Forty-two (54%) patients were current smokers, 25 (32%) had never smoked and 11 (14%) were ex-smokers. The clinical pattern at presentation of BOOP was more frequently similar to classical acute or sub-acute infectious pneumonia. Fever (63%), dyspnoea (58%) and dry cough (53%) were the typical symptoms on admission. A flu-like syndrome preceeding BOOP was observed in 21 cases (27%). Inspiratory crackles (78%) were the most typical finding at physical examination. However, 13% of the patients were asymptomatic and an abnormal chest X-ray film was the reason for seeking medical attention. Radiographically the most frequent pattern of BOOP was a unilateral consolidation (44%) with lower field predominance. A migratory behaviour was present in 22% of the cases. High-resolution computed tomographic (HR-CT) scan when performed, was more sensitive in detecting ground glass infiltrates, sub-pleural or peri-bronchovascular distribution or the presence of nodules or cavitation. Most patients (68%) were classified as having idiopathic BOOP. However, the same clinical-roentgenological pattern was observed in patients after radiotherapy for ductal breast carcinoma (6%), in collagen-vascular diseases (6%), related to drugs (9%), to infections serologically documented (4%), and to graft vs. host disease (4%). Four patients (all of whom had idiopathic BOOP) presented a rapid progressive respiratory failure needing mechanical ventilation. In another two cases respiratory failure appeared after a long period during which patients experienced exertional dyspnoea and low grade fever. BAL profile was characterized by lymphocytosis with a reduction of the CD4/CD8 ratio, associated with a slight increase of neutrophils and eosinophils and scattered mast cells. However in two cases we had an increased CD4/CD8 ratio and in one case the presence of a significant 12% of polyclonal B cells. In a few cases atypical (cytokeratin-positive cells) epithelial cells were detected: these cells were constantly present in the BAL fluid of patients with rapidly progressive respiratory failure. From the diagnostic point of view this series documents that transbronchial lung biopsy (coupled with BAL) can be the first diagnostic step. However, therapy can be started on the basis of BAL data (when a characteristic morphological and phenotypical profile is evident) in cases in which the clinical presentation is suggestive and a biopsy cannot be made. Most patients showed a rapid and good response to steroid therapy. However, three patients died (4%) in spite of steroid therapy (two cases) and steroid and cyclophosphamide therapy (one case). In conclusion, although clinical findings, chest X-ray film and CT Scan findings usually suggest the diagnosis a definite confirmation requires transbronchial lung biopsy and BAL and, less frequently, open lung biopsy.
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PMID:Bronchiolitis obliterans-organizing pneumonia: an Italian experience. 1092 43

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

A 64-year-old man was hospitalized for productive cough and dyspnea. Both chest radiographs and CT scans showed areas of ground-glass opacity in the middle and lower lung fields on both sides. The BAL and TBLB findings were compatible with hypersensitivity pneumonitis. The serum was negative for antibodies against Trichosporon species, and the result of a lymphocyte stimulating test for administered drugs including a Chinese medicine was also negative. A humidifier was suspected as the cause because it had been used for more than 10 years in the factory where the patient had been working. An inhalation test using the humidifier fluid successfully provoked dyspnea, fever and fine crackle, and laboratory tests demonstrated hypoxemia, reduction in vital capacity and the elevation of CRP. Agar gel diffusion using the patient's serum showed a precipitating line against Cephalosporium acremonium, but this line did not fuse with any precipitating line formed between the humidifier fluid and the serum, indicating that no Cephalosporium was Present in the humidifier fluid. Since a high level of beta-D glucan was detected in the humidifier fluid, an unidentified fungus was suspected to be the antigen.
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PMID:[Hypersensitivity pneumonitis caused by a factory humidifier. A case report]. 1143 13

Langerhans cell histiocytosis with only pulmonary involvement is a rare, probably reactive, proliferating disease of young and smoking persons. During last 7 years 7 cases of Langerhans cell histiocytosis were diagnosed in our Department. There were 6 men and one woman. Mean age was about 32 years. All of them were smokers. Intensity and duration of smoking varied widely from 5 to 40 cigarettes/day and 5 to 30 years respectively. The most frequent symptoms were dry cough, dyspnea. Two patients had no complaints. Pneumothorax preceded diagnosis in 4 patients. Duration of symptoms varied from 3 months to 10 years--in a patient who was previously diagnosed as allergic alveolitis. All patients had typical radiological and HRCT appearance of disseminated nodular lesions with small cysts formation with sparing of costo-phrenic angles. Three of patients had very advanced disease with cystic lung destruction. Restriction was noticed in those three patients. Diminished values of compliance and diffusing capacity for CO was observed in four patients. Bronchoscopy examination of bronchial tree revealed only slight inflammation in all patients. BAL was assessed for presence of CD1a cells in 6 cases, but in no one patient elevated number of those cells was noticed (2-4.5%). In all patients open lung biopsy was performed, and typical histologic appearance was observed. In six patients also CD1a positive cells were revealed in lung specimens.
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PMID:[Pulmonary Langerhans cell histiocytosis--clinicopathologic analysis of 7 cases]. 1173 89

A 73-year-old woman suffering from dyspnea on effort and chronic cough was admitted to our hospital. Chest computed tomography disclosed ground-glass opacities, irregular linear opacities and honeycombing distributed predominantly in the subpleural area. The serum levels of SP-D and KL-6 rose to 889 ng/ml, 1,755 U/ml, respectively. These findings indicated idiopathic pulmonary fibrosis. However, the number of lymphocytes and the CD4/CD8 ratio in the BAL fluid were elevated. Transbronchial lung biopsy specimens demonstrated alveolitis with granuloma formation. The evidence that she had lived in a house with a heavy fungal growth and that tests of precipitation in response to Penicillium corylophilum were positive confirmations of a diagnosis of chronic hypersensitivity pneumonia.
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PMID:[A case of chronic hypersensitivity pneumonia with elevation of serum SP-D and KL-6]. 1192 22


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