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DeCS

Descriptor English:

Marfan Syndrome

Descriptor Spanish:

Síndrome de Marfan

Descriptor Portuguese:

Síndrome de Marfan

Synonyms English:

Marfan Syndrome, Type I
Marfan's Syndrome
Marfans Syndrome
Syndrome, Marfan
Syndrome, Marfan's

Tree Number:

Definition English:

An autosomal dominant disorder of CONNECTIVE TISSUE with abnormal features in the heart, the eye, and the skeleton. Cardiovascular manifestations include MITRAL VALVE PROLAPSE, dilation of the AORTA, and aortic dissection. Other features include lens displacement (ectopia lentis), disproportioned long limbs and enlarged DURA MATER (dural ectasia). Marfan syndrome is associated with mutations in the gene encoding fibrillin, a major element of extracellular microfibrils of connective tissue.

See Related English:

History Note English:

1985; use ARACHNODACTYLY 1963-1984

Allowable Qualifiers English:

Record Number:

8554

Unique Identifier:

D008382

Similar:

DeCS