Ehlers-Danlos Syndrome Flashcards

1
Q

Pathogenesis

A

heterogeneous condition of abnormal elastin and collagen formation (>10 types)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

who gets it?

A

often autosomal dominant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

how does it present?

A
pain 
profound joint hypermobility and instability 
vascular fragility - ease of bruising 
skin hyperelasticity 
scoliosis 
heart problems
autonomic dysfunction 
inner organ involvement
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

how is this management?

A

dislocating joints - bony surgery may be required
bleeding can be a problem
skin healing can be poor - stretched scars/ wound dehisence

How well did you know this?
1
Not at all
2
3
4
5
Perfectly