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isovaleric Acidemia

Also Known As:
Acidemia, isovaleric; Isovaleric acid CoA dehydrogenase deficiency; Isovaleric acidemia; Isovaleryl CoA carboxylase deficiency
Networked: 49 relevant articles (1 outcomes, 2 trials/studies)

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Bio-Agent Context: Research Results

Experts

1. Wasant, Pornswan: 2 articles (01/2015 - 06/2008)
2. Vatanavicharn, Nithiwat: 2 articles (01/2015 - 06/2008)
3. Kuptanon, Chulaluck: 2 articles (01/2015 - 06/2008)
4. Dai, Fang: 2 articles (04/2011 - 06/2010)
5. Ye, Jun: 2 articles (08/2008 - 05/2005)
6. Sobey, Jenna Helmer: 1 article (02/2015)
7. Nguyen, Thanh: 1 article (02/2015)
8. Kiberenge, Roy: 1 article (02/2015)
9. Austin, Thomas: 1 article (02/2015)
10. Lam, Humphrey: 1 article (02/2015)

Related Diseases

1. Pancreatitis
2. Propionic Acidemia
04/01/1996 - "Acylcarnitines in amniotic fluid samples were analyzed for the prenatal diagnosis of propionic acidemia, methylmalonic aciduria, isovaleric acidemia, and glutaric aciduria by electrospray tandem mass spectrometry. "
04/16/2003 - "The organic acidurias include propionic aciduria, methylmalonic aciduria (MMA), isovaleric acidemia, lactic acidemia and glutaric acidemia type I. However, the association of MMA with electrical activity of the brain characterised by a hypsarrhythmic pattern, refractory convulsions and psychomotor retardation is very rare. "
06/10/1992 - "The feasibility of this new and affordable procedure has been confirmed by identifying urinary acylcarnitines in cases of medium-chain acyl-coenzyme A dehydrogenase deficiency, propionic acidemia and isovaleric acidemia."
10/01/2007 - "Among the 166 neonates studied (10 aged 1 day, 79 aged 2-7 days and 77 aged 8-28 days), significant abnormalities on TMS suggestive of IEM were detected in 38 babies (23%), most common diseases diagnosed were maple syrup urine disease (10 neonates), propionic acidemia (8 neonates), urea cycle diseases (6 neonates) and isovaleric acidemia (4 neonates). "
05/01/2005 - "Forty patients (4.0%) were diagnosed as organic acidemias among the 1000 patients, including 20 methylmalonic acidemia, 6 propionic acidemia, 3 isovaleric acidemia, 3 glutaric acidemia type I, 3 glutaric acidemia type II, 2 biotinidas deficiency, 1 3-methylcrotonyl-CoA carboxylase deficiency, 1 3-hydroxy-3-methylglutaryl-CoA lyase deficiency, and 1 beta-keto thiolase deficiency. "
3. Maple Syrup Urine Disease
4. Multiple Acyl Coenzyme A Dehydrogenase Deficiency
5. Hyperammonemia

Related Drugs and Biologics

1. Protons (Proton)
2. Amino Acids
3. Methylmalonic acidemia
4. Carnitine (L-Carnitine)
5. Medium chain acyl CoA dehydrogenase deficiency
6. Acyl-CoA Dehydrogenase (Medium-Chain Acyl-Coenzyme A Dehydrogenase)
7. Transferases
8. Oxidoreductases
9. Coenzyme A (CoA)
10. acylcarnitine

Related Therapies and Procedures

1. Perioperative Period