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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:
MGA Type 2; 3 methylglutaconicaciduria type 2; 3-methylglutaconicaciduria type 2s; 3-methylglutaconicaciduria type IIs; MGA type IIs; Syndrome, Barth; type 2, 3-methylglutaconicaciduria; type 2s, 3-methylglutaconicaciduria; type II, 3-methylglutaconicaciduria; type II, MGA; type IIs, 3-methylglutaconicaciduria; type IIs, MGA; 3-methylglutaconicaciduria type 2; 3-methylglutaconicaciduria type II; MGA type II
Networked: 116 relevant articles (3 outcomes, 5 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Neutropenia
2. Dilated Cardiomyopathy (Cardiomyopathy, Congestive)
3. Muscular Diseases (Myopathy)
4. Cardiomyopathies (Cardiomyopathy)
5. Myotonic Dystrophy (Dystrophia Myotonica)

Experts

1. Hatch, Grant M: 10 articles (04/2014 - 02/2004)
2. Greenberg, Miriam L: 8 articles (04/2014 - 01/2004)
3. Wanders, Ronald J A: 8 articles (09/2013 - 09/2002)
4. Schlame, Michael: 8 articles (04/2012 - 05/2002)
5. Steward, Colin G: 6 articles (09/2015 - 04/2009)
6. Houtkooper, Riekelt H: 6 articles (02/2014 - 11/2008)
7. Vaz, Frédéric M: 6 articles (02/2014 - 06/2005)
8. Finsterer, Josef: 6 articles (11/2013 - 08/2003)
9. Chen, Shuliang: 5 articles (02/2014 - 01/2004)
10. Barth, Peter G: 5 articles (11/2013 - 09/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Barth Syndrome:
1. Cardiolipins (Cardiolipin)IBA
2. Reactive Oxygen Species (Oxygen Radicals)IBA
3. TEMPOIBA
4. Linoleic Acid (Linoleate)IBA
5. Messenger RNA (mRNA)IBA
6. monolysocardiolipinIBA
7. Cyclic neutropeniaIBA
8. Proteins (Proteins, Gene)IBA
9. Cataract and cardiomyopathyIBA
10. AMP Deaminase (Myoadenylate Deaminase)IBA

Therapies and Procedures

1. Heart Transplantation (Grafting, Heart)
2. Mitral Valve Annuloplasty
3. Transplants (Transplant)
4. Transplantation (Transplant Recipients)