WHAT ARE THE RELATED DISORDERS?

by - December 25, 2017





OKAY GET IT TOGETHER GUYSSS! THIS IS A VERY CONFUSING TOPIC TO UNDERSTAND.






So, as stated from the title, these are the related disorders which are similar to Marfan Syndrome. I am going to tell you roughly the types of syndromes. Some terms might be hard to understand but, not to worry! I will my best to make it simple.




LOEYS-DIETZ SYNDROME
  • Loeys-Dietz syndrome is a genetic disorder of the body’s connective tissue. 
  • It has some features in common with Marfan syndrome, but it also has some important differences. 
  • Loeys-Dietz syndrome is an enlargement of the aorta, the large blood vessel that carries blood from the heart to the rest of the body. 
  • The aorta can weaken and stretch, causing a bulge in the blood vessel wall (an aneurysm). Stretching of the aorta may also lead to a sudden tearing of the layers in the aorta wall (aortic dissection). 
  • This is a life-threatening complication that can occur without warning. In Loeys-Dietz syndrome, aneurysms and dissections also can occur in arteries other than the aorta.

EHLERS-DANLOS SYNDROME

  • Ehlers-Danlos syndrome is a group of genetic connective tissue disorders characterized by unstable, hypermobile joints, loose, “stretchy” skin, and fragile tissues. 
  • Ehlers-Danlos syndrome is caused by a defect in the body’s connective tissue. 
  • Unlike Marfan syndrome, the fragile tissues and skin and unstable joints found in Ehlers-Danlos syndrome are due to defects in a group of proteins called collagen, proteins that add strength and elasticity to connective tissue.
MASS PHENOTYPE

  • MASS Phenotype is a connective tissue disorder that is similar to Marfan syndrome. 
  • It is caused by a similar mutation in the gene called fibrillin-1 that tells the body how to make an important protein found in connective tissue.  
  • Someone with MASS phenotype has a 50 percent chance of passing the gene along to each child.


I know it's a lot to take in. But that is all for now.

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