Cystic fibrosis and fat malabsorption: Pathophysiology of the cystic fibrosis gastrointestinal tract and the impact of highly effective CFTR modulator therapy.

Autor: McDonald CM; Cystic Fibrosis Center, Primary Children's Hospital, Salt Lake City, Utah, USA., Reid EK; Cystic Fibrosis Center, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA., Pohl JF; Pediatric Gastroenterology, Primary Children's Hospital, Salt Lake City, Utah, USA., Yuzyuk TK; Pathology, University of Utah School of Medicine, Salt Lake City, Utah, USA.; ARUP Institute for Clinical & Experimental Pathology, Salt Lake City, Utah, USA., Padula LM; Pediatric Specialty, University Health, San Antonio, Texas, USA., Vavrina K; Pediatric Specialty, University Health, San Antonio, Texas, USA., Altman K; Gunnar Esiason Adult Cystic Fibrosis and Lung Center, Columbia University Medical Center, New York, New York, USA.
Jazyk: angličtina
Zdroj: Nutrition in clinical practice : official publication of the American Society for Parenteral and Enteral Nutrition [Nutr Clin Pract] 2024 Apr; Vol. 39 Suppl 1, pp. S57-S77.
DOI: 10.1002/ncp.11122
Abstrakt: Cystic fibrosis (CF) is a progressive, genetic, multi-organ disease affecting the respiratory, digestive, endocrine, and reproductive systems. CF can affect any aspect of the gastrointestinal (GI) tract, including the esophagus, stomach, small intestine, colon, pancreas, liver, and gall bladder. GI pathophysiology associated with CF results from CF membrane conductance regulator (CFTR) dysfunction. The majority of people with CF (pwCF) experience exocrine pancreatic insufficiency resulting in malabsorption of nutrients and malnutrition. Additionally, other factors can cause or worsen fat malabsorption, including the potential for short gut syndrome with a history of meconium ileus, hepatobiliary diseases, and disrupted intraluminal factors, such as inadequate bile salts, abnormal pH, intestinal microbiome changes, and small intestinal bacterial overgrowth. Signs and symptoms associated with fat malabsorption, such as abdominal pain, bloating, malodorous flatus, gastroesophageal reflux, nausea, anorexia, steatorrhea, constipation, and distal intestinal obstruction syndrome, are seen in pwCF despite the use of pancreatic enzyme replacement therapy. Given the association of poor nutrition status with lung function decline and increased mortality, aggressive nutrition support is essential in CF care to optimize growth in children and to achieve and maintain a healthy body mass index in adults. The introduction of highly effective CFTR modulator therapy and other advances in CF care have profoundly changed the course of CF management. However, GI symptoms in some pwCF may persist. The use of current knowledge of the pathophysiology of the CF GI tract as well as appropriate, individualized management of GI symptoms continue to be integral components of care for pwCF.
(© 2024 American Society for Parenteral and Enteral Nutrition.)
Databáze: MEDLINE
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