scholarly journals Impact on Isoleucine and Valine Supplementation When Decreasing Use of Medical Food in the Nutritional Management of Methylmalonic Acidemia

Nutrients ◽  
2020 ◽  
Vol 12 (2) ◽  
pp. 473
Author(s):  
Laurie E. Bernstein ◽  
Casey Burns ◽  
Morgan Drumm ◽  
Sommer Gaughan ◽  
Melissa Sailer ◽  
...  

Background: Methylmalonic acidemia (MMA) is an autosomal recessive disorder treated with precursor-free medical food while limiting natural protein. This retrospective chart review was to determine if there was a relationship between medical food, valine (VAL) and/or isoleucine (ILE) supplementation, total protein intake, and plasma amino acid profiles. Methods: A chart review, of patients aged 31 days or older with MMA treated with dietary intervention and supplementation of VAL and/or ILE and followed at the Children’s Hospital Colorado Inherited Metabolic Diseases Clinic. Dietary prescriptions and plasma amino acid concentrations were obtained at multiple time points. Results: Baseline mean total protein intake for five patients was 198% of Recommended Dietary Allowance (RDA) with 107% natural protein and 91% medical food. Following intervention, total protein intake (p = 0.0357), protein from medical food (p = 0.0142), and leucine (LEU) from medical food (p = 0.0276) were lower, with no significant change in natural protein intake (p = 0.2036). At baseline, 80% of patients received VAL supplementation and 100% received ILE supplementation. After intervention, only one of the cohort remained on supplementation. There was no statistically significant difference in plasma propiogenic amino acid concentrations. Conclusions: Decreased intake of LEU from medical food allowed for discontinuation of amino acid supplementation, while meeting the RDA for protein.

Nutrients ◽  
2018 ◽  
Vol 10 (10) ◽  
pp. 1543 ◽  
Author(s):  
Naomi Yoshii ◽  
Koji Sato ◽  
Riki Ogasawara ◽  
Yusuke Nishimura ◽  
Yasushi Shinohara ◽  
...  

Dietary protein intake is critical for the maintenance of skeletal muscle mass. Plasma amino acid concentrations increase with protein intake and increases in muscle protein synthesis are dependent on leucine concentrations. We aimed to investigate the effect of a mixed meal and free amino acids intake on plasma leucine concentrations. In this randomized crossover study, 10 healthy young men (age 25 ± 1 years, height 1.73 ± 0.02 m, weight 65.8 ± 1.5 kg) underwent tests under different conditions—intake of 2 g of leucine (LEU), intake of a mixed meal (protein 27.5 g, including 2.15 g of leucine, protein: fat: carbohydrate ratio—22:25:53) only (MEAL), intake of 2 g of leucine immediately after a mixed meal (MEAL-LEU) and intake of 2 g of leucine 180 min after a mixed meal (MEAL-LEU180). Blood samples were collected within 420 min (240 min for LEU only) after intake and changes in amino acid concentrations were evaluated. Although the maximum plasma leucine concentration increased to 442 ± 24 µM for LEU, it was lower at 347 ± 16 µM (p < 0.05 vs. LEU) for MEAL-LEU, 205 ± 8 µM (p < 0.05 vs. LEU) for MEAL. The maximum plasma leucine concentration for MEAL-LEU180 increased to 481 ± 27 µM and compared to LEU there was no significant difference (p > 0.1). The observation that rapid elevations in plasma leucine concentrations are suppressed when leucine is ingested at the same time as a meal suggests that the timing of its intake must be considered to maximize the anabolic response.


2021 ◽  
Vol 5 (Supplement_2) ◽  
pp. 415-415
Author(s):  
Mariah Jackson ◽  
Fang Niu ◽  
Lynette Smith ◽  
Laura Bilek ◽  
Nancy Waltman ◽  
...  

Abstract Objectives Determine the extent to which baseline protein and amino acid intake is independently associated with measures of baseline bone outcomes in a cohort of post-menopausal women. Methods This study was a secondary analysis of participants in the Heartland Osteoporosis Prevention Study (HOPS) randomized controlled trial evaluating the effect of different osteoporosis interventions on post-menopausal women in the Midwest. Diets were evaluated via a Harvard Willett Semi-quantitative Food Frequency Questionnaire (FFQ) at baseline. Dual energy x-ray absorptiometry (DXA) scans were used to collect bone mineral density (BMD) and trabecular bone score (TBS) measurements. Single and multivariate linear regression of total protein intake (g/d and g/kg/d), proline (g), and lysine (g) with BMD and TBS locations were conducted. Student T-tests were used to compare the bone outcome differences between groups with protein intake above and below 1.2g/kg. Results There were 249 participants available for analysis. Mean total protein intake was 82.7 g with 62% consuming &lt; 1.2/kg/d. After adjustment, for every additional gram intake of protein, lysine and proline, Hip BMD increased (Protein: β = 0.0005, P = 0.047; Lysine: β = 0.006, P = 0.04; Proline: β = 0.008, P = 0.04). Whole body BMD and TBS-1 were significantly associated with proline intake (Whole body BMD: β = 0.008, P = 0.04; TBS-1: β = −0.007, P = 0.02). After adjustment, women who consumed protein above 1.2 g/kg, had a Hip BMD 0.03 g/cm2 higher than those who consume protein below 1.2 g/kg (β = 0.03, P = 0.046). Lumbar BMD, Total L1-L4 BMD, TBS-2, TBS-3, TBS-4, and Total TBS were not significantly associated with protein or amino acid intakes after adjustment of covariates. Conclusions Higher protein intakes were associated with higher Hip BMD. Proline may play a controversial role in bone outcomes. Future research should investigate the role of dietary factors on BMD vs TBS. Funding Sources The original research was funded by the National Institute of Nursing Research of the National Institutes of Health under award number R01NR015029. No additional funding was used for the secondary analysis.


1985 ◽  
Vol 63 (5) ◽  
pp. 487-494 ◽  
Author(s):  
N. Theresa Glanville ◽  
G. Harvey Anderson

The effect of diabetes (streptozotocin, 65 mg/kg ip), dietary protein intake (15–60%), and plasma amino acid concentrations on brain large neutral amino acid levels in rats was examined. After 20 days, the plasma concentrations of methionine and the branched chain amino acids (BCAA), valine, isoleucine, and leucine were increased in diabetic rats. In brain tissue, methionine and valine levels were increased but threonine, tyrosine, and tryptophan concentrations were depressed. Increased protein consumption promoted a diabetic-like plasma amino acid pattern in normal rats while enhancing that of diabetic animals. However, with the exception of threonine, glycine, valine, and tyrosine, there was little effect on brain amino acid levels. A good association was found between the calculated brain influx rate and the actual brain concentration of threonine, methionine, tyrosine, and tryptophan in diabetic animals. There was no correlation, however, between brain influx rate and brain BCAA levels. Thus, the brain amino acid pattern in diabetes represents the combined effects of insulin insufficiency and composition of the diet ingested on plasma amino acid levels as well as metabolic adaptation within the brain itself.


1982 ◽  
Vol 243 (1) ◽  
pp. R99-R103 ◽  
Author(s):  
D. J. Johnson ◽  
G. H. Anderson

The level of each plasma amino acid in male weanling rats was examined in relation to their intake from gluten, casein, synthetic amino acid, or zein diets. Plasma levels of valine, leucine, and isoleucine were found to be linearly related to the intake per gram diet regardless of the dietary source. Although levels of most other amino acids were not linearly related to intake, they were predictable if both the concentration of the amino acid in the diet and chronic level of protein intake were known. That is, a relationship, which applied to all diets, was found between the concentration of amino acid in the plasma, the concentration in the diet, and the total protein or amino acid nitrogen in the diet.


1988 ◽  
Vol 77 (1) ◽  
pp. 60-66 ◽  
Author(s):  
E. KINDT ◽  
H. A. LUNDE ◽  
L. R. GJESSING ◽  
S. HALVORSEN ◽  
S. O. LIE

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