Abstract
Exocrine pancreatic insufficiency (PI) affects a majority of the population with cystic fibrosis (CF). A subset is born with pancreatic sufficiency (PS), but it can develop to PI later in life. This chapter on PI and nutrition in CF begins with perspectives from a patient with CF and PI and then reviews historical observations, pathophysiology, definition of severe PI, clinical manifestations, diagnosis, screening and treatment of PI, fat-soluble vitamin deficiencies, and bone health. The chapter highlights recommendations and evidence for treating PI with pancreatic enzyme replacement therapy, the approach to assessing the effectiveness of therapy, and the emergence of CFTR modulators, which may improve the management of PI and malnutrition. Finally, the chapter provides a summary of key points for the screening and treatment of PI and briefly discusses future directions.KeywordsExocrine pancreatic insufficiencyCystic fibrosisPancreatic sufficiencyPancreatic function testFecal elastasePancreatic enzyme replacement therapyBone healthFat-soluble vitaminsNutritionModulatorsCorrectorsPotentiators
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