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Loeys-Dietz Syndrome

An autosomal dominant aneurysm with multisystem abnormalities caused by increased TGF-BETA signaling due to mutations in type I or II of TGF-BETA RECEPTOR. Additional craniofacial features include CLEFT PALATE; CRANIOSYNOSTOSIS; HYPERTELORISM; or bifid uvula. Phenotypes closely resemble MARFAN SYNDROME; Marfanoid craniosynostosis syndrome (Shprintzen-Goldberg syndrome); and EHLERS-DANLOS SYNDROME.
Also Known As:
Loeys-Dietz Syndrome, Type 1a; Loeys Dietz Aortic Aneurysm Syndrome; Loeys Dietz Syndrome; Loeys Dietz Syndrome, Type 1a; Syndrome, Loeys-Dietz; Loeys-Dietz Aortic Aneurysm Syndrome
Networked: 222 relevant articles (0 outcomes, 11 trials/studies)

Disease Context: Research Results

Related Diseases

1. Marfan Syndrome (Marfan's Syndrome)
2. Thoracic Aortic Aneurysm
3. Aneurysm (Aneurysms)
4. Ehlers-Danlos Syndrome (Syndrome, Ehlers-Danlos)
5. Bicuspid Aortic Valve Disease

Experts

1. Dietz, Harry C: 17 articles (07/2022 - 04/2006)
2. Morisaki, Takayuki: 10 articles (01/2022 - 10/2009)
3. Morisaki, Hiroko: 8 articles (01/2022 - 10/2009)
4. Braverman, Alan C: 7 articles (01/2021 - 08/2006)
5. Loeys, Bart L: 7 articles (06/2013 - 08/2006)
6. Loeys, Bart: 6 articles (12/2021 - 04/2006)
7. Komuro, Issei: 6 articles (11/2019 - 12/2015)
8. Takeda, Norifumi: 6 articles (11/2019 - 12/2015)
9. Matsuda, Hitoshi: 5 articles (01/2022 - 10/2009)
10. Sasaki, Hiroaki: 5 articles (01/2022 - 10/2009)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Loeys-Dietz Syndrome:
1. Transforming Growth Factor-beta Type I ReceptorIBA
2. Transforming Growth Factor-beta Type II ReceptorIBA
3. Transforming Growth Factors (Transforming Growth Factor)IBA
4. Transforming Growth Factor beta (TGF-beta)IBA
5. 1,3,4,6- tetra- O- acetyl- 2- azido- 2- deoxyglucopyranose (TAAD)IBA
6. Fibrillin-1IBA
7. LigandsIBA
8. dicarboxydine (DCD)IBA
9. Transforming Growth Factor beta Receptors (TGF beta Receptors)IBA
10. Transforming Growth Factor beta3 (TGF beta 3)IBA

Therapies and Procedures

1. Replantation
2. Electrodes (Electrode)
3. Anesthesia
4. Heart Transplantation (Grafting, Heart)
5. Stents