Special low protein foods for phenylketonuria: availability in Europe and an examination of their nutritional profile

Background Special low protein foods (SLPF) are essential in the nutritional management of patients with phenylketonuria (PKU). The study objectives were to: 1) identify the number of SLPF available for use in eight European countries and Turkey and 2) analyse the nutritional composition of SLPF available in one of these countries. Methods European Nutritionist Expert Panel on PKU (ENEP) members (Portugal, Spain, Belgium, Italy, Germany, Netherlands, UK, Denmark and Turkey) provided data on SPLF available in each country. The nutritional composition of Portuguese SLPF was compared with regular food products. Results The number of different SLPF available in each country varied widely with a median of 107 [ranging from 73 (Portugal) and 256 (Italy)]. Food analysis of SLPF available from a single country (Portugal) indicated that the mean phenylalanine content was higher in low protein baby cereals (mean 48 mg/100 g) and chocolate/energy bars/jelly (mean 41 mg/100 g). The energy content of different foods from a sub-group of SLPF (cookies) varied widely between 23 and 96 kcal/cookie. Low protein bread had a high fat content [mean 5.8 g/100 g (range 3.7 to 10)] compared with 1.6 g/100 g in regular bread. Seven of the 12 SLPF sub-groups (58 %) did not declare any vitamin content, and only 4 (33 %) identified a limited number of minerals. Conclusions Whilst equal and free access to all SLPF is desirable, the widely variable nutritional composition requires careful nutritional knowledge of all products when prescribed for individual patients with PKU. There is a need for more specific nutritional standards for special low protein foods.

Nutritional requirements of the critically ill patient

Objective: Given the inaccessibility of indirect calorimetry, intensive care units generally use predictive equations or recommendations that are established by international societies to determine energy expenditure. The aim of the present study was to compare the energy expenditure of critically ill patients, as determined using indirect calorimetry, to the values obtained using the Harris-Benedict equation. Methods: A retrospective observational study was conducted at the Intensive Care Unit 1 of the Centro Hospitalar do Porto. The energy requirements of hospitalized critically ill patients as determined using indirect calorimetry were assessed between January 2003 and April 2012. The accuracy (± 10% difference between the measured and estimated values), the mean differences and the limits of agreement were determined for the studied equations. Results: Eighty-five patients were assessed using 288 indirect calorimetry measurements. The following energy requirement values were obtained for the different methods: 1,753.98±391.13 kcal/ day (24.48 ± 5.95 kcal/kg/day) for indirect calorimetry and 1,504.11 ± 266.99 kcal/day (20.72±2.43 kcal/kg/day) for the HarrisBenedict equation. The equation had a precision of 31.76% with a mean difference of -259.86 kcal/day and limits of agreement between -858.84 and 339.12 kcal/day. Sex (p=0.023), temperature (p=0.009) and body mass index (p< 0.001) were found to significantly affect energy expenditure Conclusion: The Harris-Benedict equation is inaccurate and tends to underestimate energy expenditure. In addition, the Harris-Benedict equation is associated with significant differences between the predicted and true energy expenditure at an individual level