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• W2130856821 abstract "This report describes a patient suffering from intolerance to amino acids, mainly l-methionine, while on prolonged total parenteral nutrition. The patient displayed tachycardia, tachypnea, central scotomas, night blindness, and irritability, leading to coma while on total parenteral nutrition. The symptoms disappeared with discontinuation of the administered l-amino acid solutions. Biochemical abnormalities included high plasma methionine (110 to 130 µmol/L, normal 10 to 55 µmol/L) and low serum uric acid levels (0.5 to 1.4 mg/dl) associated with increased urinary excretion of sulfite (strong positive colorimetric reaction on Machery Nagel strips versus absent reaction in controls), thiosulfate (4.4 to 9.5 mM/d day or 30 to 50% of total sulfur excretion versus normal = 0.4 ± 0.1 mM/day or <2%), hypoxanthine ( 150 to 750 mg/24 h, normal <50 mg/24 h) and xanthine (700 to 2100 mg/24 h, normal <50 mg/24 h), with a decreased urinary excretion of uric acid (<100 mg/24 h, normal 300 to 520 mg/24 h) and inorganic sulfate (2.9 to 5.5 mM/day or 10 to 40% of total sulfur excretion versus 18.3 ± 1.2 mM/day in normal or 80% of total sulfur excretion). These abnormalities indicated that the underlying defect in sulfur amino acid metabolism occurred at the level of transformation of sulfite to sulfate, while that in uric acid production was at the level of transformation of xanthine and hypoxanthine to uric acid. The two enzymes catalyzing these reactions are sulfite oxidase, and xanthine oxidase, two metallo-enzymes containing molybdenum as part of their prosthetic groups. Treatment with ammonium molybdate (300 µg/day) improved the clinical condition, reversed the sulfur handling defect, and normalized uric acid production. This may represent the first case of diet-induced molybdenum deficiency in man." @default.
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• W2130856821 date "1981-11-01" @default.
• W2130856821 modified "2023-10-14" @default.
• W2130856821 title "Amino acid intolerance during prolonged total parenteral nutrition reversed by molybdate therapy" @default.
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• W2130856821 doi "https://doi.org/10.1093/ajcn/34.11.2551" @default.
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