Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0206061 (interstitial pneumonia)
 6,105 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The case of a 10.5-year-old girl, who was diagnosed with a case of thalassemia major at the age of 8 months and had been on regular blood transfusions since then, is related. Donor screening for HIV was started in mid-1988, thus she had received unscreened blood for a number of years. In February 1991, she presented with a dry persistent cough, moderate grade continuous fever, and breathlessness on exertion for over 2 weeks. Chest X-ray showed bilateral infiltrations. She was put on penicillin and chloramphenicol with a provisional diagnosis of bronchopneumonia. In March 1991, she had to be hospitalized for impending respiratory failure. After treatment with intravenous fluids and parenteral antimicrobials, her condition stabilized and she was discharged. In April 1991, she was readmitted because of complaints of difficulty in swallowing and weight loss. Her chest signs had persisted and she had developed oropharyngeal candidiasis with ulcerations. She also had alopecia, a generalized lymphadenopathy, digital clubbing, and bilateral parotid enlargement. Candidiasis responded to vigorous therapy with clotrimazole. Fine needle aspiration of lymph node showed a reactive hyperplasia. HIV antibodies were detected in the serum with ELISA and confirmed by Western blot. Immunologic tests showed evidence of severe immunodeficiency. The Multitest CMI, which simultaneously tests delayed skin hypersensitivity to seven common recall antigens, was totally nonreactive. She was classified as having AIDS according to World Health Organization criteria for children under 13 years of age. The diagnosis of lymphocytic interstitial pneumonitis (LIP) was also made based on the symptoms. Oral prednisolone was given 2 mg/kg/day in 3 divided doses for a month. The cough and dyspnea showed great improvement and the parotid swellings disappeared; lymphadenopathy, clubbing, and alopecia, however, persisted. The child was kept on maintenance therapy of prednisolone and on alternate day co-trimoxazole for prophylaxis against Pneumocystis carinii infection.
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PMID:Acquired immunodeficiency syndrome (AIDS) with lymphocytic interstitial pneumonitis (LIP) in a multi transfused child with thalassemia major. 129 97

Three cases of cholesterol interstitial pneumonia in patients 3, 9 and 10 years of age respectively are reported. All three were born in Island of Reunion. Two were sisters. All had failure to thrive, dyspnea on rest and clubbing. Respiratory symptoms had appeared early in infancy. Open pulmonary biopsy was diagnostic. Prognosis was poor the boy dying at 4 years of age and severe respiratory insufficiency at 9 or 10 years in the two girls. Current etiological investigations were non contributory. However a profile of chronic infection with Epstein Barr virus (EBV) was found in each case while serological profiles ruled out infection with a virus of the herpes group virus (cytomegalovirus, herpes simplex). The possible role of EBV as an etiological agent of cholesterol pneumonia is discussed and genetic or environmental factors as well.
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PMID:[Cholesterol pneumopathy in children. Apropos of 3 cases. Possible role of chronic Epstein-Barr virus infection]. 300 Mar 13

Two major pulmonary diseases were defined on the basis of lung biopsies in 15 children with acquired immune deficiency syndrome (AIDS) or AIDS-related complex. Pneumocystis carinii pneumonia was observed in eight children, and pulmonary lymphoid hyperplasia in six. One child had nonspecific interstitial pneumonitis. Children with P. carinii pneumonia had more severe hypoxemia, with higher alveolar-arterial oxygen gradients, and higher isomorphic elevations of serum lactate dehydrogenase. Clinically, children with pulmonary lymphoid hyperplasia were older, and had digital clubbing, parotid gland enlargement, and elevated serum IgG levels. Results of serologic assays and lung tissue analysis were suggestive of persistent Epstein-Barr virus infection exclusively in patients with pulmonary lymphoid hyperplasia. Recognition of the clinical and laboratory findings characteristic of each entity may assist in the differential diagnosis without the need of surgical biopsy.
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PMID:Pulmonary disease in children with acquired immune deficiency syndrome and AIDS-related complex. 300 14

Idiopathic pulmonary fibrosis (IPF), also called interstitial pneumonia or fibrosing alveolitis, is a progressive interstitial lung disease of unknown origin. Two distinct forms are known which differ in course, morphologic features and cytological findings in bronchoalveolar lavage: a cellular "desquamative interstitial pneumonia" and a hypocellular and more fibrotic "usual interstitial pneumonia". The two types appear to represent different stages of the same disease. The clinical findings in IPF are dyspnoea, unproductive cough, clubbing and rales. Pulmonary function tests reveal restriction, reduced diffusion capacity and hypoxaemia during exercise. Chest X-rays show localized linear or nodular densities usually accentuated at the lung bases; in 10% of patients with IPF, chest radiography is normal. To assess the inflammatory process bronchoalveolar lavage and transbronchial or open lung biopsy have proven necessary. Before beginning treatment the activity of the inflammatory process and degree of lung function impairment should be established. Therapy of IPF is unspecific and its aim is to suppress alveolitis and the fibrotic process. Corticosteroids are the initial therapy. If there is no improvement after two months, immunosuppressive agents are added. Regular clinical and functional follow-up is required. Course and prognosis vary in different forms of IPF. Without treatment, median survival in the cellular form is over 10 years and in the hypocellular form between 3.2 and 5.6 years. Only 12-30% of patients respond to antiinflammatory treatment.
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PMID:[Idiopathic lung fibrosis]. 304 52

To assess the role of chest radiography in the differential diagnosis of bronchiolitis obliterans organizing pneumonia (BOOP) and usual interstitial pneumonia (UIP), records of 34 patients with biopsy-proved BOOP (16 patients) or UIP (18 patients) were reviewed. Chest radiographs taken before biopsy were available in 26 patients, clinical information in 33, and pulmonary function data in 22. These data were reviewed independently, without knowledge of the pathologic diagnosis. The clinical symptoms of BOOP were similar to those of UIP, although the duration of symptoms was longer in UIP (P less than .05), and the prevalence of systemic symptoms was higher in BOOP (P less than .025). The physical findings were similar except that finger clubbing was more common in patients with UIP than in those with BOOP (P less than .01). There was no significant difference in lung volumes, flows, or diffusing capacity between BOOP and UIP. The chest radiograph showed distinguishing features between UIP and BOOP in 23 of 26 patients. The most characteristic radiologic finding in BOOP was the presence of patchy areas of air-space consolidation (eight of 11 patients).
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PMID:Differential diagnosis of bronchiolitis obliterans with organizing pneumonia and usual interstitial pneumonia: clinical, functional, and radiologic findings. 378 54

This report is based on 43 cases where a diagnosis of either bronchiolitis obliterans with organizing pneumonia (BOOP), usual interstitial pneumonia (UIP), or small airways disease (SAD) was established by lung biopsy. The severity of histologic abnormalities in the peripheral airways and interstitial spaces were measured on these biopsies using semiquantitative techniques and compared with the clinical data available in 42 of 43 cases, preoperative chest radiographs in 31 of 43, and preoperative pulmonary function tests in 29 of 43. The data show that when a diagnosis of BOOP was made, there was a higher total pathologic score for membraneous bronchiolitis (MB) and respiratory bronchiolitis (RB) than for UIP and SAD (p less than 0.005). This was due to peribronchiolar inflammation and the presence of loose connective tissue in the RB lumen. The pathologic changes in the interstitial space were less severe in SAD than in BOOP or UIP (p less than 0.005). Clubbing was more frequent in UIP (p less than 0.01), and symptoms were of shorter duration in BOOP (p less than 0.05). The radiographic assessment showed that the characteristic finding in BOOP was patchy air-space consolidation, a finding that was not present in UIP or SAD.
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PMID:A comparison of bronchiolitis obliterans with organizing pneumonia, usual interstitial pneumonia, and small airways disease. 382 96

Interstitial pneumonitis in children is a rare and poorly understood disease. Controversy exists as to whether the varoius histologic changes encountered represent different disease or a spectrum of the same disease. Fourteen biopsy-confirmed cases of desquamative interstitial pneumonitis in children were seen at the Mayo Clinic between 1953 and 1975. A search of the literature revealed 14 additional cases but no series of exclusively desquamative interstitial pneumonitis. The most frequent symptoms were retardation of growth and dyspnea, often accompanied by cough. Tachypnea was the most common finding on examination; rales, cyanosis, and clubbing were variably present. The chest roentgenogram was distinctly abnormal in all cases; it usually revealed a combined interstitial and alveolar pattern extending bilaterally from the hilus to the base. Results of laboratory studies were nonspecific for desquamative interstitial pneumonitis. All 28 patients in this review were treated with corticosteroids; 17 (61 percent) survived. Desquamative interstitial pneumonitis was found in association with a variety of other major illnesses. The cause remains unknown.
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PMID:Desquamative interstitial pneumonitis in children. 735 10

Our objective was to assess the capacity of clinical and laboratory information to predict findings in the lung biopsy in interstitial lung diseases (ILD). We studied 121 patients with ILD as a cohort recruited in our institute from 1983 to 1987 with the diagnosis of hypersensitivity pneumonitis (HP) and usual interstitial pneumonia (UIP). Histologic diagnosis (HP vs UIP) and degree of fibrosis (< 50% of the biopsy surface vs > or = 50%) were used as the gold standard to compare a series of clinical and laboratory variables in the initial assessment. We used a stepwise logistic regression model to predict the biopsy results. The model was calculated in half of the patients selected by random sampling, and the calculated model was tested in the other half of the patients. Variables found to predict degree of fibrosis were (with relative risk RR and 95% confidence interval): a radiographic pattern of honeycombing (RR 5.0 from 0.9-29), digital clubbing (RR 8 from 1.4-48) and gender (RR 2.9 from 0.4-20). This model classified correctly 72% of the biopsies, with a sensitivity of 0.38, a specificity of 0.85 and a kappa of 0.25 +/- 0.19 (p = 0.17 NS). For histologic diagnosis (NIU vs NH), the model included gender (RR 6.6, 1.3-33), honeycombing (RR 1.6, from 0.4-6.0), digital clubbing (RR 4.6, from 1.2-18), and vital capacity expressed as percent of predicted (RR 0.96, from 0.92-1.00).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Capability of clinical and laboratory findings to predict the grade of fibrosis and the diagnosis in diffuse interstitial lung diseases]. 761 Feb 89

Diffuse interstitial pneumonia of unknown etiology has been subclassified into two groups on the patho-histological basis by the study group under the sponsorship of the Japanese Ministry of Health and Welfare. One is Group A, typical idiopathic subtype, fundamentally corresponding to UIP (usual interstitial pneumonia, Liebow) and the other is Group B, closely similar to Group A but possibly modified by respiratory infection. As the clinical features of Group B have not been clarified so far, we investigated them in 8 cases of Group B (M:F = 7:1, 56.7 years at first visit) and 5 cases of Group A (M:F = 4:1, 54.4 years), all of which were pathologically diagnosed by open lung biopsy and/or autopsy. Only in Group B, there were 3 cases with occupations related to dust exposure, and 7 cases with intermittent but persistent mucus expectoration. The duration of symptoms before the first presentation to hospital was longer in Group B than in Group A (1.6 years vs. 0.6 years). Clubbed fingers were more frequently observed in Group B (n = 6 vs. n = 1), but induration in the tuberculin test was less frequent in Group B (n = 1 vs. n = 5). In the chest X-ray findings, pleural thickening was frequent in Group B (n = 6 vs. n = 0), but elevation of the diaphragm was unusual in Group B (n = 2 vs. n = 5).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Clinical features and morbidity of group B diffuse interstitial pneumonia, atypical subtype, compared with group A, typical idiopathic subtype]. 796 34

We report the experience with and evaluation of treatment strategies in fibrosing alveolitis and desquamative interstitial pneumonitis (FA/DIP) over the last 16 years by a review of all cases referred to a tertiary referral center. There were 25 cases, 16 boys and 9 girls (mean age at onset, 2.3 years; range, 7 days to 11.6 years). In each case the diagnosis was confirmed by open lung biopsy at a mean age of 3.3 years (range, 7 weeks to 15.1 years). Presently features were tachypnea (19), cyanosis (15), cough (12), exertional dyspnea (7), recurrent chest infections +/- wheezing (9), and clubbing (8). Four patients recovered without antiinflammatory medication. The others received specific treatment. Of 11 patients given only prednisolone, six improved, two did not, and three died despite treatment. Of five patients receiving only chloroquine, four responded. Five patients received both prednisolone and chloroquine; one died, two responded well. There was poor progress in the remaining two. Of the 10 patients receiving chloroquine six (60%) showed a good response. A younger presentation carried a worse prognosis, but chest radiology at presentation and outcome were not interrelated. Those with mild histological changes all survived, but severe desquamation or fibrosis at biopsy was not related to outcome. In four cases there was a family history (16%). Patients with FA/DIP probably represent a disease spectrum of multiple etiology with a variable prognosis and response to treatment.
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PMID:Fibrosing alveolitis and desquamative interstitial pneumonitis. 809 Jun 6


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