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Barth Syndrome

Rare congenital X-linked disorder of lipid metabolism. Barth syndrome is transmitted in an X-linked recessive pattern. The syndrome is characterized by muscular weakness, growth retardation, DILATED CARDIOMYOPATHY, variable NEUTROPENIA, 3-methylglutaconic aciduria (type II) and decreases in mitochondrial CARDIOLIPIN level. Other biochemical and morphological mitochondrial abnormalities also exist.
Also Known As:
3-Methylglutaconic Aciduria, Type II; 3-Methylglutaconicaciduria Type 2; 3-Methylglutaconicaciduria Type II; Cardioskeletal Myopathy with Neutropenia and Abnormal Mitochondria; MGA Type 2; MGA Type II; 3 Methylglutaconic Aciduria, Type II; 3 Methylglutaconicaciduria Type 2; 3-Methylglutaconicaciduria Type 2s; 3-Methylglutaconicaciduria Type IIs; MGA Type 2s; MGA Type IIs; Syndrome, Barth; Type 2, 3-Methylglutaconicaciduria; Type 2, MGA; Type 2s, MGA; Type II, MGA; Type IIs, MGA
Networked: 253 relevant articles (15 outcomes, 19 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Mitochondrial Diseases (Mitochondrial Disease)
2. Cardiomyopathies (Cardiomyopathy)
3. Dilated Cardiomyopathy (Cardiomyopathy, Congestive)
4. Muscular Diseases (Myopathy)
5. Neutropenia

Experts

1. Schlame, Michael: 22 articles (01/2022 - 05/2002)
2. Hatch, Grant M: 18 articles (09/2021 - 02/2004)
3. Vaz, Frédéric M: 16 articles (04/2022 - 06/2005)
4. Greenberg, Miriam L: 16 articles (01/2022 - 01/2004)
5. Ren, Mindong: 14 articles (01/2022 - 12/2005)
6. Xu, Yang: 14 articles (01/2022 - 06/2005)
7. Byrne, Barry J: 11 articles (08/2022 - 08/2006)
8. Vernon, Hilary J: 10 articles (01/2022 - 06/2014)
9. Wanders, Ronald J A: 10 articles (01/2022 - 09/2002)
10. Steward, Colin G: 9 articles (05/2021 - 04/2009)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Barth Syndrome:
1. Cardiolipins (Cardiolipin)IBA
2. arginyl- 2,'6'- dimethyltyrosyl- lysyl- phenylalaninamideIBA
3. BezafibrateIBA
4. Reactive Oxygen Species (Oxygen Radicals)IBA
5. Peroxisome Proliferator-Activated Receptors (PPAR)IBA
6. monolysocardiolipinIBA
7. Mitochondrial DNA (mtDNA)IBA
8. PhosphatidylserinesIBA
9. AntioxidantsIBA
10. TEMPOIBA

Therapies and Procedures

1. Therapeutics
2. Secondary Prevention
3. Endurance Training
4. Heart Transplantation (Grafting, Heart)
5. Mitral Valve Annuloplasty