Nutritional assessment in adults with cystic fibrosis.

Objectives: Clinical experience with patients with cystic fibrosis (CF) suggests a nutritional risk in this population. In addition to the lung pathology, a main pathophysiologic concern is the viscous mucus blocking pancreatic ducts, leading to reduced production of pancreatic enzymes. Therefore, maldigestion and consequently malabsorption (particularly fat and fat-soluble vitamins) occur, resulting in steatorrhea, vitamin deficiencies, and subsequently manifest malnutrition. The aim of this study was to investigate the nutritional status and determine the prevalence of malnutrition in an adult Swiss CF cohort.

Methods: This was an observational cohort study in which the nutritional status and dietary habits of patients with CF and healthy controls were compared. Assessment was based on the nutritional risk screening (NRS-2002), dietary habits (7-d dietary record), body composition (bioelectrical impedance analysis), anthropometrics, resting energy expenditure (REE; indirect calorimetry), and physical or mental function (hand-grip strength, Short Form-36 v2).

Results: Nineteen patients (15 men, mean age 32 y) and 15 controls (8 men, mean age 49 y) were included. Eight patients (42%) were at nutritional risk (NRS-2002 ≥3). Patients had higher energy intake/body weight (P = 0.021) with lower body fat percentage (P < .001), body mass index (P = 0.030), and physical/mental health scores (P < 0.001) than controls. Energy intake was higher than REE in patients (P = 0.003), but not in controls (P = 0.373).

Conclusions: Prevalence of malnutrition was high in this CF cohort, coinciding with low body fat percentage and low body mass index despite high energy and protein intake. Energy requirements of patients with CF should be estimated as approximately twice the Harris-Benedict REE and 1.7 times indirect calorimetry REE, while ensuring adequate intake of pancreatic enzymes.