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- W2026565593 abstract "Orotic aciduria, an indicator of adequacy of treatment in lysinuric protein intolerance (LPI). The basic defect in LPI is in the transport of the diamino acids in kidney tubuli, jejunum and liver cells. This leads to a deficiency in the liver of the urea cycle intermediates arginine and ornithine, a malfunction of the cycle and an accumulation of ammonia. Like in the other conditions in which hyperammonemia is associated with accumulation of carbamyl phosphate, urinary excretion of orotic acid is excessive. Orotic acid is an intermediate of the synthesis of the pyrimidines from carbamyl phosphate. We measured orotic acid excretion in controls and patients with LPI. The controls in all situations excreted less than 20 μg/kg/hr. The patients excreted normal amounts during fasting (4.6, 2-8, 5 for mean, range, number of subjects). Their excretion was increased in 24-hr urines during a self-chosen low-protein diet (125, 3-366, 7), in 4 to 6 hr urines after a milk load with 0.5 g of protein/kg (488, 251-1747, 3), in 2-hr urines after oral ammonium lactate, 2.5 mmoles/kg (212, 15-1126, 10), and in 6-hr urines after iv alanine, 6.6 mmoles/kg in 90 min (790, 47-1831, 11). If the loads were given with an iv infusion of arginine, ornithine or citrulline, the orotic aciduria did not appear. Given orally, citrulline was the most efficient of the three in preventing orotic aciduria. Orotic aciduria thus is a reliable indicator of the function of the urea cycle in LPI and enables us to monitor the home treatment." @default.
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- W2026565593 date "1980-02-01" @default.
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- W2026565593 title "Orotic aciduria, an indicator of adequacy of treatment in lysinuric protein intolerance (LPI): 76" @default.
- W2026565593 doi "https://doi.org/10.1203/00006450-198002000-00103" @default.
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