The effects of low protein products availability on growth parameters and metabolic control in selected amino acid metabolism disorders patients

Abstract

BackgroundIn Saudi Arabia, a diet for life policy has been adopted in the management of amino acid metabolism disorders for years. However, the specially designed low protein products/medical foods - which are one of the important treatment tools - were not available up until several years ago in Saudi Arabia (SA). Our aim was to measure the compliance and quality of life in patients affected with these disorders followed in the metabolic nutrition clinic at King Faisal Specialist Hospital & Research Centre (KFSH&RC), Riyadh, SA.MethodologyWe used a non-randomized retrospective/prospective study which utilized the growth parameters, biochemical data of patients plus questionnaires collected from patients and their family/caregivers. A total of n = 182 patients affected with selected amino acid metabolism disorders were enrolled. Some were excluded n = 84 for various reasons. Sample analyzed were: Phenylketonuria (PKU) (44), Maple Syrup Urine Disease (MSUD) (30), Tyrosinemia (TYR) (17) and Homocystinuria (HCU) (7). Tandem Mass Spectrometry (TMS) used to quantitate plasma amino acid concentrations. Data was obtained using (COMPLE) Microsoft-Access which was designed by the metabolic nutrition clinic at KFSH&RC-Riyadh. Student's paired t-test was used to investigate relationship between variables.ResultsThe main findings were the improvement of selected amino acid levels pre and post the usage of medical foods. In PKU patients, the TMS Phenylalanine (PHE) levels post usage was significantly decreased (P value < .0001). This was also the case in MSUD patients with significant decrease in Leucine & Isoleucine levels (P value .0008) but not in Valine levels (P value .1148) as 36.7% of them received Valine supplements while enrolled in the study.ConclusionLow protein products availability was successful in improving outcomes for selected amino acid metabolic disorders. However, due to compliance issues and impracticality of the diet, the results were not significant in all enrolled patients.

Exploring the Barriers and Motivators to Dietary Adherence among Caregivers of Children with Disorders of Amino Acid Metabolism (AAMDs): A Qualitative Study

“…It is now well established from a variety of studies that individualized dietary therapy plays an important role in the treatment of AAMD, as it is characterized by a disruption of the metabolism of amino acids, which are the main constituents of the daily diet [6,7]. The current treatment for this group of disorders includes (a) the provision of a suitable protein substitute free of the offending substrate; (b) a lifelong protein restriction of natural protein intake through the exclusion of all high protein food, such as eggs, milk, cheese, meat, poultry, fish, dried beans, and legumes; and (c) the provision of special low-protein food to meet the energy requirement [8,9].…”

Current Practices and Challenges in the Diagnosis and Management of PKU in Latin America: A Multicenter Survey

“…Even when available, they were usually not affordable, since these were rarely subsidized by the government. Specially designed low-protein products are important for satiety and diet variety [ 25 ], and were also proven valuable in improving metabolic control and growth in patients with PKU [ 26 ]. However, they inflict a significant financial burden to the PKU diet: in an American study, low-protein foods represented the highest annual out-of-pocket costs (child = US$1651.00; adult = US$967.00) when compared to other categories of care [ 6 ].…”

Effect of Special Low-Protein Foods Consumption in the Dietary Pattern and Biochemical Profile of Patients with Inborn Errors of Protein Metabolism: Application of a Database of Special Low-Protein Foods

“…The dietary approach for managing these metabolic disorders typically involves calculating and measuring the natural protein source, adding carbohydrates and fats to the diet to increase caloric intake and prevent catabolism, and supplementing with calculated amounts of PFAAs as needed, along with SLPFs that provide energy and variety to the diet [8]. SLPFs are considered essential in managing these disorders, as they not only meet energy needs but also help maintain anabolism and improve the variety of the diet, thereby helping to maintain metabolic control within target ranges [9].…”