54 Primary Disorders of Connective Tissue

Question 1

Criteria used to define patients with benign hypermobile joint syndrome

Answer 1

Brighton criteria

Question 2

M:F Benign hypermobile joint syndrome

Answer 2

F

Question 3

Number of EDS subtypes

Answer 3

13

Question 4

Heterogeneous group of disorders characterized by soft tissue laxity of varying degree, particularly affecting the dermis and joints

Answer 4

EDS

Question 5

Collagen types found to be defective in EDS subtypes

Answer 5

Type I, III, and V collagen

Question 6

Clinical EDS types with defect in type I collagen

Answer 6

1) Rare type of classical EDS
2) Cardiac valvular EDS
3) Rare type of vascular EDS

Question 7

Clinical EDS types with defect in type III collagen

Answer 7

Vascular EDS

Question 8

Clinical EDS types with defect in type V collagen

Answer 8

Classical EDS