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- W2523890399 abstract "Although CF is a genetically transmitted disease, various immunologic phenomena have been documented to occur during its inexorable clinical course. Abundant and abnormally sticky mucous secretions block ducts in the gastrointestinal and respiratory tracts and account for both slowing of mucociliary clearance as well as obstruction, inflammation, and chronic infection. Colonization and infection with P. aeruginosa as well as other strains of bacterial organisms may be responsible for the clinical progression of CF lung disease and have been associated with immune responses that may seem contradictory in nature. Most CF patients demonstrate hyperimmune responses to bacterial antigens such as hypergammaglobulinemia, leukocytosis, increased lymphocyte stimulation, elevated complement levels, and immune complex production. Other CF patients may have hypogammaglobulinemia and still others show a depressed lymphocyte responses to Pseudomonas. Many of the variations in immune responses parallel progression of the chronic obstructive lung disease and its associated state of malnutrition. Classic allergic manifestations may also occur, some of which are IgE medicated such as atopy, reactive airways disease, and a higher prevalence of allergic bronchopulmonary aspergillosis than is seen in any other childhood lung disease. In addition, non--IgE-mediated phenomena in the presence of immune complexes in serum, sputum, and lung tissue have been reported. Treatment of CF is primarily supportive but includes specific measures directed at the control of pulmonary infection, augmentation of mucus clearance, and various nutritional therapies. The use of immunologically boosting or suppressing therapies are currently under investigation in multicenter studies." @default.
- W2523890399 created "2016-10-07" @default.
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- W2523890399 date "1992-05-01" @default.
- W2523890399 modified "2023-09-25" @default.
- W2523890399 title "IMMUNOLOGIC FEATURES OF THE LUNGS IN CYSTIC FIBROSIS" @default.
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- W2523890399 doi "https://doi.org/10.1016/s0889-8561(22)00107-2" @default.
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