|Keywords:||Cystic fibrosis; Dietary intake; Body growth; Pancreatic enzyme replacement therapy; Coefficient of fat absorption; Vitamin A; Retinol; Vitamin E; Alpha-tocopherol; Immunoglobulin-G|
|Full text PDF:||http://dspace.library.uu.nl:8080/handle/1874/306829|
Cystic fibrosis (CF) is a life-threatening genetic disorder that affects mostly the lungs but also the pancreas, liver and intestine. CF is characterised by chronic pulmonary inflammation resulting in a gradual, progressive decline in pulmonary function. The vast majority of CF patients also have an exocrine pancreatic insufficiency, resulting in inadequate digestion and leading to fat malabsorption and malnutrition. Lung disease and nutritional status are tightly intertwined in which malnutrition, as a result of energy losses due to malabsorption, inadequate caloric intake and increased energy metabolism, is associated with poor clinical outcomes. Therefore, regular evaluation of nutritional status is an integral part of CF care. CF-specific guidelines give recommendations for the monitoring of nutritional intake and growth, strategies to prevent malnutrition and interventions in patients with nutritional failure, as well as for the control of intestinal fat malabsorption. The aim of this thesis was to increase the knowledge on the effectiveness of nutritional interventions; the daily practice of caloric, fat, vitamin A and vitamin E intake; and pancreatic enzyme replacement therapy, along with their relationship to either growth or pulmonary function in paediatric patients with CF. It also provides insight into caloric, fat, vitamin A and vitamin E intake, and pancreatic enzyme replacement therapy, compared to CF-specific recommendations. Moreover, the use of weight and height measurements, corrected for genetic potential and the bone maturation of children and adolescents with CF, was evaluated. As the outlook for patients with cystic fibrosis (CF) has dramatically improved over the past several years, CF-care guidelines should evolve accordingly. Children and adolescents with CF had a caloric intake below recommended although significantly higher than healthy controls.In this respect, it now seems to be more prudent to advise a caloric intake slightly above the age-specific intake, with individual adjustments for patients with deviant weight- and height-growth. Moreover, a reduction in saturated fat intake seems appropriate to avoid an increased risk for cardiovascular disease later in life. The pancreatic enzyme replacement therapy has to be determined individually, as there is an enormous variability in the response to the therapy among patients, with no clear correlation between coefficient of fat absorption and pancreatic enzyme dosages. In order to prevent under- or overestimation of the nutritional status, a nutritional status assessment, including weight and height measurements, should be complemented with routine evaluation of height measurements adjusted for genetic components and in those with a delayed height-growth, evaluation of the bone age is advised. Lastly, vitamin A and E supplementation at half the CF-specific recommended dosages resulted in appropriate serum levels; therefore, dosages of 50% of the recommendations could be used, at least initially.