Nutritional assessment and management in children with cystic fibrosis / Mai Mahdy Goda Ibrahim ; Supervised Nehal Mohamed Elkoofy , Mona Mohamed Fathy , Dina Hossam Eldine Hamed

Thesis (M.Sc.) - Cairo University - Faculty of Medicine - Department of Pediatrics

Introduction: Cystic fibrosis (CF) is a genetic, autosomal recessive disorder. Malnutrition and lung disease are 2 of the main burdens of CF. Approximately 1 in every 3500 babies born in the United States has CF. While it is most common in caucasians, CF can affect all racial and ethnic groups. There are over 1800 identified mutations since the gene, cystic fibrosis transmembrane conductance regulator (CFTR), was initially discovered in 1989. A hallmark of cystic fibrosis (CF) is malnutrition leading to poor weight gain. Many factors contribute to inadequate weight gain and malnutrition in CF as Pancreatic insufficiency and a variety of gastrointestinal disorders, including gastroesophageal reflux, constipation, bacterial overgrowth, and distal intestinal obstruction syndrome (DIOS), can decrease caloric intake and nutrient absorption. Also worsening lung disease increases caloric needs while decreasing appetite in some cases. The prevalence of malnutrition is decreasing in the CF population primarily from interventions focusing on early assessment and management of malnutrition. The goals of nutrition intervention are to promote optimal growth and development, enhance quality of life and improve survival. To reach these patients with CF need to follow an unrestricted diet that is high in calories, have optimal pancreatic enzyme replacement therapy (PERT) and use supplemental nutrition when appropriate. Methods: Interventional observational study was conducted on fifty CF patients with a mean age of 3.6 years ±3.0 SD. The patients were recruited from the cystic fibrosis clinic, children's hospital, Cairo university, from March, 2016 to February, 2017

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