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These crystals are brown spheres with spiky thorns ... with concentric rings like a tree trunk. Leucine crystals typically aren’t found in healthy urine. They’re found in acidic urine.
Plasma levels of amino acids (Table 1) and apparent whole-body leucine oxidation levels 5 (low levels indicating MSUD) were measured before and after transplantation in both patients and in a ...
“Maple Syrup Urine Disease (MSUD) is a neonatal condition caused by an enzyme deficiency within the cells, which hinders the metabolism of branched-chain amino acids, specifically ...
MSUD is an inherited metabolic disease due to a deficiency in branched chain ketoacid dehydrogenase that leads to the accumulation of BCAA (leucine, isoleucine, and valine) and their keto-acid ...