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Arachnodactyly, Contractural Beals Type



Arachnodactyly, Contractural Beals Type Causes


Studies have shown that the cause of Contractural Beals Type Arachnodactyly is a mutation or alternation in the gene FBN2.


Arachnodactyly, Contractural Beals Type Definition


Contractural Beals Type Arachnodactyly is a very rare genetic disorder also known as Beals Syndrome. It is characterized by permanently flexed fingers, abnormally shaped ears and the permanent fixation of affected joints like hips, fingers, knees and elbows that is in a flexed position.


Arachnodactyly, Contractural Beals Type Diagnosis


Diagnosis of Contractural Beals Type Arachnodactyly is based on the clinical signs present. Molecular genetic testing could also confirm the disease.


Arachnodactyly, Contractural Beals Type Symptoms and Signs


Symptoms of Contractural Beals Type Arachnodactyly may include one of the following skeletal abnormalities: ? Facial abnormalities ? Muscular hypoplasia ? Pectus Deformities ? Reduced Bone Mass (Osteopenia) ? Curvature of the spine (Scoliosis) ? Long and slender toes/fingers (Arachnodactyly) ? Crumpled appearance of abnormally shaped ear ? Congenital contractures due to delay in motor development


Arachnodactyly, Contractural Beals Type Treatment


Treatment options include physical therapies to improve joint mobility or braces to provide stability. Heart monitoring is also necessary to check for cardiovascular complications that may arise due to the symptoms present.


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