Table 5.

Recommended Surveillance for Individuals with FKBP14-kEDS

System/ConcernEvaluationFrequency
Musculoskeletal Evals by orthopedic physician & specialist in rehab medicine for mgmt of kyphoscoliosis, contractures, & foot deformitiesAs clinically indicated but at least annually
DXA scanEvery 2-5 yrs, or in those w/↓ ambulation
Eyes Routine ophthalmologic evalEvery 2-3 yrs
Ears Formal hearing eval
Cardiovascular Blood pressure measurement 1At each visit
  • Echocardiography w/consideration of cardiac MRI
  • Vascular ultrasonography to evaluate abdominal & peripheral arteries & veins
Every 2-5 yrs starting in early childhood
Neurodevelopment Assessment of developmental progressAt each visit until adolescence

DXA = dual-energy x-ray absorptiometry

1.

Maintenance of blood pressure in the normal range for age is recommended to reduce the risk of arterial rupture.

From: FKBP14 Kyphoscoliotic Ehlers-Danlos Syndrome

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