Nutritional intervention and follow-up for patients with cystic fibrosis / 中华临床营养杂志

ABSTRACT

Objective To investigate effect of standardized nutritional assessment, intervention, and monitoring on nutritional status of patients with cystic fibrosis (CF).Methods We recruited 6 consecutive patients with CF diagnosed from June 2005 to June 2015 in Peking Union Medical College Hospital, all given nutritional assessment and intervention.The clinical data and nutritional status of the patients were analyzed.Four cases were followed up, and their compliance and effect of nutritional intervention on their nutritional status were evaluated.Results The mean age of diagnosis of the patients was (19 ± 6) years.All the patients had recurrent onset of pulmonary infection and hypoxemia with varying degrees.The nutritional status was impaired in all the cases to different extent, with body mass index (BMI) of 3 patients under 20 years of age being lower than the third percentile of the standard growth curve for adolescents of the same age and sex.Mild microcytic hypochromic anemia existed in 2 cases, hypoalbuminemia in 2 cases (serum albumin ＜ 35 g/L in absence of liver or renal dysfunction), and decreased prealbumin level in 4 patients.The serum concentrations of Na, Cl, K, and Ca were approximately normal in all the 6 cases.Vitamin D level was detected in only 2 cases, all revealing vitamin D deficiency.High energy and high protein diet was suggested to all the patients, plus prescription of oral nutritional supplements for 5 patients.Diagnostic pancreatic enzyme replacement therapy (PERT) was performed in 3 patients who were suspected with exocrine pancreatic insufficiency (PI), together with fatsoluble vitamins supplementation including vitamin D3.Four CF patients were followed up for 11 to 39 months, of whom 1 was lost to follow-up after 11 months.During the follow-up period, 3 patients had progressive weight loss or growth retardation, and the other one patient failed to increase body weight.Dietary review of the 3 patients with available follow-up data revealed that actual energy intake and protein intake were only 46.1％-65.9％ and 44.1％-63.2％ of recommendation levels, respectively.By enhancing dietary education and guidance in follow-up, nutritional status of the patients was improved.Conclusion Intensive nutrition management, standardized nutritional intervention and monitoring, dietary guidance or enteral nutrition adjustment may constitute critical measures for improving nutritional status of CF patients.