phenylketonurias/kalín

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Metabolomic Insights into the Nutritional Status of Adults and Adolescents with Phenylketonuria Consuming a Low-Phenylalanine Diet in Combination with Amino Acid and Glycomacropeptide Medical Foods.

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UNASSIGNED Nutrient status in phenylketonuria (PKU) requires surveillance due to the restrictive low-Phe diet in combination with amino acid medical foods (AA-MF) or glycomacropeptide medical foods (GMP-MF). Micronutrient profiles of medical foods are diverse, and optimal micronutrient

Direct determination of phenylalanine in serum extracts of phenylketonuria patients by reversed-phase high-performance liquid chromatography.

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A method for direct determination of phenylalanine (Phe) in serum extract by reversed-phase high-performance liquid chromatography with an octadecylsilane column has been devised. Phe is monitored at 220 nm with a UV detector. The mobile phase is a mixture of methanol and water (10:90) with added

Acid-base status in dietary treatment of phenylketonuria.

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Blood acid-base status, serum electrolytes, and urine pH were examined in 64 infants and children with phenylketonuria (PKU) treated with three different low phenylalanine protein hydrolyzates (Aponti, Cymogran, AlbumaidXP) and two synthetic amino acid mixtures (Aminogran, PAM). The formulas caused

Effects of phenylalanine and its deaminated metabolites on Na+,K+-ATPase activity in synaptosomes from rat brain.

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The effects of phenylalanine (PHE) and its deaminated metabolites phenylpyruvate (PHP), phenyllactate (PHL) and phenylacetate (PHA) on sodium and potassium activated adenosinetriphosphatase (Na+,K+-ATPase) in synaptosomes from rat brain were investigated. At very low concentrations (5-10 microM).

Trace element status of PKU children ingesting an elemental diet.

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We assessed the trace metal status of 10 children (3.5 to 13.3 yr) with phenylketonuria (PKU) who were successively treated for at least 6 months with a semi-synthetic formula restricted in phenylalanine, then for 6 months with a reformulated formula. The reformulated product contained higher

Potentials and limitations of microorganisms as renal failure biotherapeutics.

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Renal insufficiency leads to uremia, a complicated syndrome. It thus becomes vital to reduce waste metabolites and regulate water and electrolytes in kidney failure. The most common treatment of this disease is either dialysis or transplantation. Although these treatments are very effective, they

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