propionicacidemia /pro·pi·on·ic·ac·i·de·mia/ (pro″pe-on″ik-as″ĭ-de´me-ah)
propionicacidemia
1. an aminoacidopathy characterized by excess of propionic acid and glycine in the blood and urine, ketosis, acidosis, and often neurologic complications, due to deficiency of an enzyme involved in amino acid and fatty acid catabolism.
2. excess of propionic acid in the blood.
pro·pi·on·ic·ac·i·de·mi·a (pr  p - n -k s-d m- )n. An abnormally high concentration of propionic acid in the blood, caused by the deficiency of an enzyme and characterized by vomiting, lethargy, ketoacidosis, and leukopenia. |
propionicacidemia
[prō′pē·on′ikas′idē′mē·ə]
Etymology: Gk, pro + pion, fat; L, acidus, sour; Gk, haima, blood
a rare inherited metabolic defect caused by the failure of the body to metabolize the amino acids threonine, isoleucine, and methionine, characterized by lethargy and mental and physical retardation. Acidosis results from the accumulation of propionic acid in the body. A diet low in these amino acids is difficult to achieve but is the only treatment. propionicacidemic, adj.
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