Biomedical subjects
Publications and source records attributed to E Tempany.
Two patients with isolated atresia of the pulmonary veins are described. One patient presented with haemoptysis and the other with recurrent unilateral chest infections. The diagnosis was confirmed in both instances by cardiac catheterisation and pulmonary angiography. One patient died one year after the diagnosis was made and the second child has been stable during a follow up period of three years.
An 8-year-old boy with cystic fibrosis presented with a chronic polyarthritis. Histological examination of the synovium demonstrated multiple non-caseating granulomata. A systematic search for sarcoidosis revealed characteristic ophthalmic abnormalities and an elevated serum level of angiotensin converting enzyme. An association between cystic fibrosis and sarcoidosis is likely.
Allergic bronchopulmonary aspergillosis has been recognized in association with cystic fibrosis in children since 1965. Since then, however, there have been a paucity of reports of pediatric cystic fibrosis complicated by allergic bronchopulmonary aspergillosis, and, in most cases, these have been diagnosed retrospectively. A cluster of five acute cases seen during a 4-month period in a single cystic fibrosis center with a systemic illness and deterioration in respiratory status are described. In all five patients, reversible bronchoconstriction and infiltrative changes on x-ray films suggested the diagnosis. This was confirmed by the presence of (1) peripheral blood eosinophilia, (2) elevated total IgE and Aspergillus fumigatus-specific IgE, and (3) circulating serum precipitins against A fumigatus in all cases. All children tested had positive type 1 immediate hypersensitivity to skin tests for A fumigatus, in sputum eosinophilia, and Aspergillus cultured from sputum. Only three of five children were previously noted to be atopic and none had severe advanced suppurative lung disease. All children had previously received bronchodilator therapy and appropriate antibiotics. Following treatment with corticosteroids, acute symptoms and radiologic changes resolved for 1 to 5 months. To date, no children have had recurrence of their allergic bronchopulmonary aspergillosis while receiving alternate-day steroid treatment for 6 months.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Over a 12-months period respiratory Pseudomonas aeruginosa isolated from CF patients were typed by serology and pyocin production to determine whether cross-infection was occurring. Results of typing were interpreted in relation to the degree of contact patients had with each other. One strain appeared in 4 unrelated patients. However, since none of these patients had been in contact with each other the strains considered to have been acquired from the environment. Each of six pairs of siblings shared the same strain, but the pairs of strains were distinct from each other. These results suggest that the environment is the most important source of Pseudomonas strains for CF patients and that for cross-infection to occur prolonged intimate contact is required.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
We have investigated the prevalence of atopy within families of cystic fibrosis (CF) patients and compared its frequence in CF patients, their parents and their non-CF siblings. By studying families with two CF patients it was also possible to evaluate the factors influencing the development of atopy in one CF patient relative to the other patient within the same family. A significant correlation with age (P less than 0.001) for skin test positivity to common allergens within CF sibling pairs was observed. In 14/18 families studied, only the older CF patient was atopic. The presence of atopy in CF patients was independent of the atopic status of their parents.
We studied 25 adolescent and adult patients with cystic fibrosis (CF) and 25 control subjects to determine if the prevalence of atopy and bronchial hyperreactivity was increased in this disease. Results showed that atopic symptoms, as defined by history, were more frequently present in the CF patients. Prick testing of the skin produced positive reactions in 88% of the CF group and 36% of the control subjects (p less than 0.001), and the mean number of reactions per subject was significantly higher in the former group (p less than 0.001); reactions to fungal antigens were strikingly positive in the CF group. The CF patients had a significantly higher mean serum IgG4 (p less than 0.001), IgE (p less than 0.01), and higher mean eosinophil count (p less than 0.05). Clear-cut bronchial hyperreactivity was demonstrated in the CF group compared with control subjects. Bronchial provocation with 400 micrograms of histamine led to a greater than 15% fall in the preinhalation FEV1 in 35% of the CF subjects compared with 4% of the control group, with a mean percentage fall of 15% and 3% respectively (p less than 0.001). In the CF group a greater than 15% rise in PEFR occurred in 32% after inhalation of the parasympatholytic, ipratropium bromide (54 micrograms), and in 27% after inhalation of the sympathomimetic, fenoterol (400 micrograms). No correlation was found between bronchial reactivity and atopic status, HLA phenotype pattern, or disease severity. The cause of the increased prevalence of atopy and bronchial reactivity in CF patients remains unknown. However, it is clear that a trial of bronchodilator therapy is warranted in adolescents and young adults with CF.
Explore the source record for details and available documents.
A new method which uses a differential inhibitor to measure pancreatic and salivary type alpha-amylases (EC 3.2.1.1) was applied to serum samples from 46 cystic fibrosis (CF) patients (age range 4-14 years) and 50 controls of the same age group. The levels of pancreatic type amylase were lower in the CF patients (median 26.5 I.U./1) than the controls (median 81.5) (P less than 0.001). The results for salivary-type enzyme, however, did not support the previously reported finding of higher than usual levels in CF patients. This discrepancy is probably due to differences in analytical methods. It is felt that this procedure will be of value in the investigation of patients for cystic fibrosis and other pancreatic disorders.
Explore the source record for details and available documents.