title: Nutrition Management of Cystic Fibrosis in the 21st Century.
abstract: Despite significant advancements made in life expectancy over the past century, cystic fibrosis remains a life-threatening genetic disease that affects the gastrointestinal tract, and it has significant impact on the nutrition status of those with the disease. Nutrition management includes a high-calorie/high-fat diet, pancreatic enzyme replacement therapy, vitamin and mineral replacement, and enteral support as needed. As patients are living longer, clinicians may encounter patients with cystic fibrosis in obstetrician offices, endocrine clinics, or hospital settings, owing to lung transplantation or for treatment for distal intestinal obstruction syndrome.
authors.affiliation: Rainbow Babies and Children's Hospital Case Medical Center, Cleveland, Ohio Terri.Schindler@UHhospitals.org.
authors.forename: Teresa
authors.initials: T
authors.lastname: Schindler
authors.affiliation: Medical University of South Carolina, Charleston, South Carolina.
authors.forename: Suzanne
authors.initials: S
authors.lastname: Michel
authors.affiliation: National Jewish Health, Denver, Colorado.
authors.forename: Alexandra W M
authors.initials: AW
authors.lastname: Wilson
mesh_terms: D003550:Cystic Fibrosis / Q000150:complications / Q000178:diet therapy* / Q000601:surgery; D003920:Diabetes Mellitus / Q000209:etiology; D059305:Diet, High-Fat; D002149:Energy Intake; D004750:Enteral Nutrition / Q000379:methods; D010188:Exocrine Pancreatic Insufficiency / Q000188:drug therapy / Q000209:etiology; D005260:Female; D005765:Gastrointestinal Agents / Q000627:therapeutic use; D006801:Humans; D007415:Intestinal Obstruction / Q000209:etiology; D016040:Lung Transplantation; D008297:Male; D044342:Malnutrition / Q000178:diet therapy* / Q000209:etiology; D009752:Nutritional Status; D010194:Pancreatin / Q000627:therapeutic use; D011247:Pregnancy; D011248:Pregnancy Complications / Q000209:etiology; D014815:Vitamins / Q000627:therapeutic use
chemical_list: D005765:Gastrointestinal Agents; D014815:Vitamins; D010194:Pancreatin
keywords: cystic fibrosis; diabetes mellitus; exocrine pancreas insufficiency; lung diseases; nutrition therapy; pancreas