08/02 conditions Flashcards
1
Q
Marfan’s
A
- Inherited disorder that affects connective tissue
2
Q
Marfan’s cause
A
- Defect in gene that enables body to produce protein to help give connective tissue elasticity and strength
- Inherited from parent with disorder (50-50 chance)
- 25% of cases come from mutation
3
Q
Marfan’s risks
A
- Men and women affected equally
- Occurs among all races
- Greatest risk is having parent with disorder
4
Q
Marfan’s S+S
A
- Variable, even among members of same family
- Tall and slender build
- Disproportionally long arms, legs, and fingers
- Heart murmurs
- Extreme near-sightedness
- Scoliosis
- Flat feet
5
Q
Marfan’s prognosis
A
- Complications- CV (aortic aneurysm, aortic dissection and valve malformations), eye (lens dislocation, retinal problem), skeletal (inc risk of scoliosis, sunken or protruding chest sternum, foot and low back P)
- During pregnancy extra stress on aorta can increase risk of rupture/dissection
6
Q
Elhers-Danlos
A
- Inherited disorder that affects connective tissue- primarily skin, Jts, blood vessel walls
7
Q
Elhers-Danlos cause
A
- Variety of genetics
- 50% chance of passing onto children if you have hypermobile Ehlers-Danlos
8
Q
Elhers-Danlos S+S
A
- Overly flexible Jts- connective tissue is loose
- Stretchy skin
- Fragile skin- damage often wont heal
9
Q
Elhers-Danlos prognosis
A
- Can result in dislocations and early onset arthritis
- Vascular Ehlers-Danlos can inc risk of fatal ruptures of blood vessels
10
Q
Lupus
A
- Immune system attacks own tissue and organs
11
Q
Lupus S+S
A
- Can develop suddenly or slowly, very variable
- Most people have episodes/flares
- Fatigue
- Fever
- Jt P, swelling, stiffness
- Butterfly shaped rash on face that covers cheeks and bridge of nose
- Skin lesions that appear worse with sun exposure
- Shortness of breath
- Chest P
- Dry eyes
12
Q
Lupus prognosis
A
- Corticosteroids (prednisone) helps reduce swelling, tenderness and P
13
Q
Systematic sclerosis
A
- Group of rare diseases that involve hardening and tightening of skin
- May also cause problems in blood vessels, internal organs and digestive tract
14
Q
Systematic sclerosis
A
- Overproduction and accumulation of collagen in body tissues
- No exact cause, believed to be auto-immune
- Combination of factors including immune system problems, genetics and enviro triggers
15
Q
Systematic sclerosis population
A
- Mostly affects women
- 30-50