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Marfan syndrome (MFS) is a multi-systemic genetic disorder that affects the connective tissue. [6] [7] [1] Those with the condition tend to be tall and thin, with long arms, legs, fingers, and toes. [1]
Dural ectasia is common in Marfan syndrome, [3] occurring in 63–92% of people with the syndrome. [11] It may also occur in Ehlers-Danlos Syndrome , neurofibromatosis type I , [ 12 ] ankylosing spondylitis , [ 1 ] and is associated with spondylolisthesis , vertebral fractures, [ 13 ] scoliosis , tumors or trauma .
The Foundation provides information about Marfan syndrome and funds research for the purposes of saving lives and improving the quality of life for people affected by the condition which is a genetic connective tissue disorder. The Foundation also lobbies Congress to fund Marfan syndrome research and engages in its own fundraising activities. [1]
Marfanoid (or Marfanoid habitus) is a constellation of signs resembling those of Marfan syndrome, including long limbs, with an arm span that is at least 1.03 of the height of the individual, and a crowded oral maxilla, sometimes with a high arch in the palate, arachnodactyly, and hyperlaxity.
Lujan–Fryns syndrome (LFS) is an X-linked genetic disorder that causes mild to moderate intellectual disability and features described as Marfanoid habitus, referring to a group of physical characteristics similar to those found in Marfan syndrome. [4] [5] These features include a tall, thin stature and long, slender limbs. [5]
Early Morbidity and Mortality Within 30 days of hospitalization, morbidity and mortality after Bentall procedure are associated with complications stemming from cardiac arrhythmia, pneumonia, acute respiratory distress syndrome (ARDS), sepsis, graft infection, wound infection, neurologic/ cerebrovascular accident and stroke, hemorrhage/ bleeding, myocardial infarction, pericardial effusion ...
A study by Bischoff et al. showed the 10th to 90th percentiles for TUG performance were 6.0 to 11.2 seconds for community-dwelling women between 65 and 85 years of age, and determined that this population should be able to perform the TUG in 12 seconds or less. [6] TUG performance has been found to decrease significantly with mobility impairments.
Diagnosis of NBCCS is made by having two major criteria or one major and two minor criteria. [12] The major criteria consist of the following: more than 2 BCCs or 1 BCC in a person younger than 20 years; odontogenic keratocysts of the jaw; 3 or more palmar or plantar pits; ectopic calcification or early (<20 years) calcification of the falx cerebri