Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0231807 (exertional dyspnea)
 3,402 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Patients with common variable hypogammaglobulinemia (CVH) are susceptible to recurrent respiratory infections. We studied the prevalence and severity of respiratory dysfunction in 32 patients with CVH by measuring routine pulmonary function tests (PFT) and interpreting chest radiographs using a quantitative score system. Pulmonary symptoms were determined by patient interview and standard questionnaire. The mean age of 29 living patients was 28 +/- 2.2 yr; there were 22 males and 10 females. The mean age at diagnosis was 17.3 +/- 2.4 yr. Sinopulmonary symptoms were present in all 32 patients and included chronic sinusitis, otitis, bronchitis, recurrent pneumonia, and bronchiectasis. Complete PFT in 25 patients revealed restrictive, obstructive, and normal patterns in 10, 6, and 9 patients, respectively. Nine patients had dyspnea on exertion. Recent chest radiographs in 32 patients showed evidence of recurrent pulmonary infection, including air trapping, linear markings, and pleural abnormalities. Panlobular emphysema, documented in one case at autopsy, was suggested in 5 other patients by the presence of lower lobe bullous lesions and marked lower lobe hyperlucency on chest radiographs. The forced vital capacity (FVC) and forced expiratory volume in 1 second (FEV1) had remained stable in 19 adults (18 receiving adequate immunoglobulin replacement therapy) for 6.9 +/- 0.7 yr. The expected increase of the FEV1 and FVC with growth was normal in 6 of 8 children (all 8 receiving adequate therapy). Chest radiograph scores deteriorated only slightly from 20.3 +/- 0.6 to 18.4 +/- 0.8 (25 = normal) during the 6.9 +/- 0.7 yr in 30 patients (p less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Respiratory dysfunction in patients with common variable hypogammaglobulinemia. 349 Feb

Lymphoid interstitial pneumonitis is an uncommon process, and an association with hypogammaglobulinemia in children is rare. Three patients, a 10-yr-old boy (Patient 1), a 17-yr-old girl (Patient 2), and a 13-yr-old boy (Patient 3) were evaluated for progressive interstitial pneumonitis and hypogammaglobulinemia. At presentation, symptoms or signs of lung disease had been present for 9, 6, and 1.5 yr and consisted of severe exertional dyspnea asymptomatic pneumonitis on roentgenogram, and mild exertional dyspnea, respectively. Serum immunoglobulin deficiencies were variable with IgG and IgA being depressed in all patients and IgM being within normal limits in Patient 2. In vitro neutrophil function and T lymphocyte number and mitogen responses were normal. Measurement of in vitro immunoglobulin production suggested a primary B lymphocyte dysfunction. Pulmonary function testing revealed restrictive lung disease in Patient 1, arterial hypoxemia in Patients 1 and 2, and increased alveolar-arterial oxygen differences in all patients. Microscopic examination of lung specimens from each patient revealed diffuse lymphoid parenchymal infiltration with formation of follicles with occasional germinal centers, varying degrees of fibrosis, and negative examinations for viruses, bacteria, and fungi. The factors responsible for the cessation of antibody production and for the striking lympho-proliferative process seen in these patients remain to be identified.
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PMID:Lymphoid interstitial pneumonitis and hypogammaglobulinemia in children. 697 50

A 57-year-old male presented with palpitations and dyspnea on exertion. Examination of the peripheral blood and bone marrow showed pancytopenia with marked red cell aplasia. Hypogammaglobulinemia was also recognized. Chest X-ray and CT showed a mass in the anterior mediastinum. A biopsy showed thymoma. Two months after admission, the patient died of sepsis secondary to worsening pancytopenia and hypogammaglobulinemia. Autopsy showed non-invasive spindle cell type thymoma and a marked decrease of hematogenous cells. Review of the literature indicates that pancytopenia associated with thymoma is resistant to all forms of treatment and its prognosis is poor.
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PMID:[Autopsy case of non-invasive thymoma associated with pancytopenia and hypogammaglobulinemia]. 851 9