Pancretic disease in cystic fibrosis and alpha-1 antitrypsin deficiency

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dc.contributor.authorKarabiber, Hamza
dc.contributor.authorSelimoglu, Mukadder Ayse
dc.date.accessioned2024-08-04T20:31:08Z
dc.date.available2024-08-04T20:31:08Z
dc.date.issued2009
dc.departmentİnönü Üniversitesien_US
dc.description.abstractCystic fibrosis and alpha-1 antitrypsin deficiency are two important autosomal recessive multi-organ diseases. They are complex and severe diseases that involve various organs including pancreas. Pancreatic disease in cystic fibrosis varies from complete loss of exocrine and endocrine functions to nearly normal pancreatic function. A strong correlation between genotype and phenotype is recognized. Pancreatic involvement is characterized by malabsorption of fat and protein, and growth failure. Fat malabsorption leads to special problems with the fat-soluble vitamins. Enzyme and fat-soluble vitamin replacements are the milestones of the malabsorption treatment. Gene therapy offers the best hope for a cure of cystic fibrosis. Although an association between alpha 1-antitrypsin deficiency and chronic pancreatitis has been reported in several case reports, in prospective studies it has been shown that pancreatitis prevalence in patients with alphal-antitrypsin deficiency was not different from that of healthy controls. Thus it was concluded that alpha-1 antitrypsin deficiency was not related to the pathogenesis of idiopathic or hereditary chronic pancreatitis. However, protective role of alpha-1 antitrypsin against pancreatic auto digestion suggests that the deficiency of that protein may worsen the pancreatic disease rather than causing it. Early detection and management of pancreatic insufficiency is essential to optimize health and outcomes in cystic fibrosis and alpha-1 antitrypsin deficiency patients. (Turk Arch Ped 2009; 44: 7-11)en_US
dc.identifier.endpage11en_US
dc.identifier.issn1306-0015
dc.identifier.issn1308-6278
dc.identifier.issue1en_US
dc.identifier.scopus2-s2.0-62249096944en_US
dc.identifier.scopusqualityN/Aen_US
dc.identifier.startpage7en_US
dc.identifier.urihttps://hdl.handle.net/11616/94762
dc.identifier.volume44en_US
dc.identifier.wosWOS:000266151500003en_US
dc.identifier.wosqualityN/Aen_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.language.isotren_US
dc.publisherAvesen_US
dc.relation.ispartofTurk Pediatri Arsivi-Turkish Archives of Pediatricsen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectAlfa-1 antitripsin deficinecyen_US
dc.subjectcystic fibrosisen_US
dc.subjectpancreasen_US
dc.titlePancretic disease in cystic fibrosis and alpha-1 antitrypsin deficiencyen_US
dc.typeArticleen_US

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