Abstract
Low-protein diets (LPDs) are the mainstream treatment for inborn errors of intermediary protein metabolism (IEIPM), but dietary management differs worldwide. Most studies have investigated pediatric populations and their goals such as growth and metabolic balance, showing a tendency toward increasing overweight and obesity. Only a few studies have examined nutritional status and dietary intake of adult IEIPM patients on LPDs. We assessed nutritional parameters (dietary intake using a 7-day food diary record, body composition by bioimpedance analysis, and biochemical serum values) in a group of 18 adult patients with urea cycle disorders (UCDs) and branched chain organic acidemia (BCOA). Mean total protein intake was 0.61 ± 0.2 g/kg/day (73.5% of WHO Safe Levels) and mean natural protein (PN) intake was 0.54 ± 0.2 g/kg/day; 33.3% of patients consumed amino acid (AA) supplements. A totally of 39% of individuals presented a body mass index (BMI) > 25 kg/m2 and patients on AA supplements had a mean BMI indicative of overweight. All patients reported low physical activity levels. Total energy intake was 24.2 ± 5 kcal/kg/day, representing 72.1% of mean total energy expenditure estimated by predictive formulas. The protein energy ratio (P:E) was, on average, 2.22 g/100 kcal/day. Plasmatic levels of albumin, amino acids, and lipid profiles exhibited normal ranges. Phase angle (PA) was, on average, 6.0° ± 0.9°. Fat mass percentage (FM%) was 22% ± 9% in men and 36% ± 4% in women. FM% was inversely and significantly related to total and natural protein intake. Data from IEIPM adults on LPDs confirmed the pediatric trend of increasing overweight and obesity despite a low energy intake. A low protein intake may contribute to an increased fat mass. Nutritional parameters and a healthy lifestyle should be routinely assessed in order to optimize nutritional status and possibly reduce risk of cardiovascular degenerative diseases in adult UCD and BCOA patients on LPDs.
Highlights
Low-protein diets (LPDs) are the main treatment for inborn errors of intermediary protein metabolism (IEIPM), such as urea cycle disorders (UCDs) and branched chain organic acidemia (BCOA).There is a consensus on limiting the natural protein intake, both in pediatric and adult populations, while the use of amino acid formulas in UCDs and BCOA is still discussed.LPDs are individualized for each patient, considering clinical status and individual tolerance to toxic metabolites [1]
Dietary intake, and body composition, and we investigated the relationship of these parameters to nutritional outcomes to better understand the possible long-term nutritional implications of LPDs
Dietary intake, and body composition in adult patients with UCDs and BCOA on LPDs since a pediatric age
Summary
Low-protein diets (LPDs) are the main treatment for inborn errors of intermediary protein metabolism (IEIPM), such as urea cycle disorders (UCDs) and branched chain organic acidemia (BCOA).There is a consensus on limiting the natural protein intake, both in pediatric and adult populations, while the use of amino acid formulas in UCDs and BCOA is still discussed.LPDs are individualized for each patient, considering clinical status and individual tolerance to toxic metabolites [1]. Recent guidelines [2,3] for the management of patients with UCDs and BCOA have advised a low-protein diet and referred to the World Health Organization (WHO), Food and Agriculture Organization of the United Nations (FAO), and United Nations University (UNU) protein and energy requirements [4] The use of amino acid formulas is advised when protein requirements are not reached with only natural protein, calculated as the provision of 20–30% of the total protein intake. The amount of these formulas is still debated among different metabolic centers [2,3,5]
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