Organic acid disorders definition
Organic acid disorders means disorders of metab- olism characterized by the accumulation of nonamino organic acids and toxic intermediates. This may lead to met- abolic crisis with ketoacidosis, hyperammonemia and hypo- glycemia resulting in severe neurological and physical dam- age and possibly death. For the purpose of this chapter organic acid disorders include: 3-OH 3-CH3 glutaric acid- uria (HMG), beta-ketothiolase deficiency (BKT), glutaric acidemia type I (GA 1), isovaleric acidemia (IVA), methyl- malonic acidemia (CblA,B), methylmalonic acidemia (mutase deficiency) (MUT), multiple carboxylase deficiency (MCD), and propionic acidemia (PROP).
Organic acid disorders means 3-OH 3-CH3 glutaric aciduria (HMG), beta-ketothiolase deficiency (BKT), glutaric acidemia type I (GA 1), isovaleric acidemia (IVA), methylmalonic acidemia (CblA,B), methylmalonic acidemia (mutase deficiency) (MUT), multiple carboxylase deficiency (MCD), and propionic acidemia (PROP). These disorders can lead to metabolic crises resulting in severe nerve damage, physical damage, and possibly death.
Organic acid disorders means disorders of metabolism char- acterized by the accumulation of nonamino organic acids and toxic in- termediates. This may lead to metabolic crisis with ketoacidosis, hy- perammonemia and hypoglycemia resulting in severe neurological and physical damage and possibly death. For the purpose of this chapter organic acid disorders include: 3-OH 3-CH3 glutaric aciduria (HMG), beta-ketothiolase deficiency (BKT), glutaric acidemia type I (GA 1), isovaleric acidemia (IVA), methylmalonic acidemia (CblA,B), methylma- lonic acidemia (mutase deficiency) (MUT), multiple carboxylase defi- ciency (MCD), and propionic acidemia (PROP).
More Definitions of Organic acid disorders
Organic acid disorders means disorders of metab- olism characterized by the accumulation of nonamino organic acids and toxic intermediates. This may lead to met- abolic crisis with ketoacidosis, hyperammonemia and hypo- glycemia resulting in severe neurological and physical dam- age and possibly death. For the purpose of this chapter organic acid disorders include: 3-OH 3-CH3 glutaric aciduria (HMG), beta-ketothiolase deficiency (BKT), glutaric acide- mia type I (GA 1), isovaleric acidemia (IVA), methylmalonic