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isovaleric Acidemia

A rare hereditary, autosomal recessive metabolic disorder. Two forms of isovaleric acidemia, possibly allelic, are recognized: the acute neonatal form, leading to massive ACIDOSIS and rapid death in the first days of life, and a chronic form in which periodic attacks of severe KETOSIS occur with asymptomatic intervening periods. Mutations in the IVD gene have been identified. OMIM: 243500
Also Known As:
Acidemia, isovaleric; Isovaleric Acid-CoA Dehydrogenase Deficiency; Isovaleric acid CoA dehydrogenase deficiency; Isovaleric acidemia; Isovaleryl CoA carboxylase deficiency; Isovaleryl-CoA Dehydrogenase Deficiency
Networked: 144 relevant articles (4 outcomes, 8 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Methylmalonic acidemia
2. Propionic Acidemia
3. Maple Syrup Urine Disease
4. Medium chain acyl CoA dehydrogenase deficiency
5. Multiple Acyl Coenzyme A Dehydrogenase Deficiency

Experts

1. Vockley, Jerry: 6 articles (09/2021 - 12/2004)
2. Vockley, J: 4 articles (12/2008 - 02/2000)
3. Ensenauer, Regina: 3 articles (03/2018 - 12/2004)
4. Sakamoto, Osamu: 3 articles (06/2017 - 12/2011)
5. Ye, Jun: 3 articles (08/2008 - 05/2005)
6. Yamaguchi, Seiji: 2 articles (05/2022 - 04/2010)
7. Ibarra-González, Isabel: 2 articles (06/2021 - 02/2020)
8. Vela-Amieva, Marcela: 2 articles (06/2021 - 02/2020)
9. Chinen, Yasutsugu: 2 articles (06/2017 - 01/2015)
10. Mienie, L J: 2 articles (10/2016 - 11/2007)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to isovaleric Acidemia:
1. Anti-Bacterial Agents (Antibiotics)IBA
2. TritiumIBA
3. Isovaleryl-CoA Dehydrogenase (Isovaleryl-Coenzyme A Dehydrogenase)IBA
4. Glycine (Aminoacetic Acid)FDA LinkGeneric
5. 3-methylbutyrylcarnitineIBA
6. pivaloylcarnitineIBA
7. isovaleryl-coenzyme AIBA
8. Amino AcidsFDA Link
9. AcidsIBA
10. EnzymesIBA

Therapies and Procedures

1. Therapeutics
2. General Anesthesia
3. Perioperative Period
4. Protein-Restricted Diet (Diet, Protein Restricted)
5. Lipectomy (Liposuction)