{"title":"囊性纤维化的上皮细胞功能障碍:对气道疾病的影响。","authors":"T F Boat, P W Cheng","doi":"10.1111/apa.1989.78.s363.25","DOIUrl":null,"url":null,"abstract":"<p><p>An important pathophysiologic factor in CF airways is the failure to clear poorly hydrated secretions. The water deficit in CF mucous secretions can now be ascribed to a fundamental defect of epithelial cell regulatory processes which promotes sodium reabsorption from surface liquids and interferes with chloride secretion onto the luminal surface. In addition, it is now known that CF airway epithelial cells oversulfate high molecular weight glycoconjugates, both secreted and cell surface-associated. Oversulfation of glycoconjugates may contribute to the altered clearance properties of CF airways mucus and in addition could favor colonization of airways by organisms such as P. aeruginosa.</p>","PeriodicalId":75408,"journal":{"name":"Acta paediatrica Scandinavica. Supplement","volume":"363 ","pages":"25-9; discussion 29-30"},"PeriodicalIF":0.0000,"publicationDate":"1989-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1111/apa.1989.78.s363.25","citationCount":"38","resultStr":"{\"title\":\"Epithelial cell dysfunction in cystic fibrosis: implications for airways disease.\",\"authors\":\"T F Boat, P W Cheng\",\"doi\":\"10.1111/apa.1989.78.s363.25\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>An important pathophysiologic factor in CF airways is the failure to clear poorly hydrated secretions. The water deficit in CF mucous secretions can now be ascribed to a fundamental defect of epithelial cell regulatory processes which promotes sodium reabsorption from surface liquids and interferes with chloride secretion onto the luminal surface. In addition, it is now known that CF airway epithelial cells oversulfate high molecular weight glycoconjugates, both secreted and cell surface-associated. Oversulfation of glycoconjugates may contribute to the altered clearance properties of CF airways mucus and in addition could favor colonization of airways by organisms such as P. aeruginosa.</p>\",\"PeriodicalId\":75408,\"journal\":{\"name\":\"Acta paediatrica Scandinavica. Supplement\",\"volume\":\"363 \",\"pages\":\"25-9; discussion 29-30\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"1989-01-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://sci-hub-pdf.com/10.1111/apa.1989.78.s363.25\",\"citationCount\":\"38\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Acta paediatrica Scandinavica. Supplement\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1111/apa.1989.78.s363.25\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Acta paediatrica Scandinavica. Supplement","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1111/apa.1989.78.s363.25","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Epithelial cell dysfunction in cystic fibrosis: implications for airways disease.
An important pathophysiologic factor in CF airways is the failure to clear poorly hydrated secretions. The water deficit in CF mucous secretions can now be ascribed to a fundamental defect of epithelial cell regulatory processes which promotes sodium reabsorption from surface liquids and interferes with chloride secretion onto the luminal surface. In addition, it is now known that CF airway epithelial cells oversulfate high molecular weight glycoconjugates, both secreted and cell surface-associated. Oversulfation of glycoconjugates may contribute to the altered clearance properties of CF airways mucus and in addition could favor colonization of airways by organisms such as P. aeruginosa.