Medical Definition of Methylmalonic acidaemia

1. A group of inherited metabolic disorders that result in the accumulation of methylmalonic acid in the bloodstream. This leads to a severe metabolic acidosis (and ketosis) that can be fatal. A defect in the metabolic pathway of methylmalonic acid, vitamin B12 and succinic acid is the underlying cause. An infection can trigger a bout of acidosis. A low protein diet is essential in these patients. Occasionally large doses of vitamin B12 or an alkalinising agent (bicarbonate) will be necessary to treat acute exacerbations of acidosis. Origin: Gr. Haima = blood (27 Sep 1997)

Lexicographical Neighbors of Methylmalonic Acidaemia

methylglutaconyl-CoA hydratase
methylglyoxal
methylglyoxalase
methylglyoxal dehydrogenase
methylguanidine
methylguanidine synthase
methylhexaneamine
methylhistamines
methylhistidines
methylhydrazines
methylic
methylic
methylkinase
methylmalonate semialdehyde
methylmalonic acid
methylmalonic acidaemia (current term)
methylmalonic acidemia
methylmalonic aciduria
methylmalonyl-CoA
methylmalonyl-CoA epimerase
methylmalonyl-CoA mutase
methylmannosides
methylmercuries
methylmercury
methylmercury
methylmercury compounds
methylmethacrylates
methylmorphine
methylnitronitrosoguanidine
methylnitrosourea

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