08/02 conditions Flashcards

1
Q

Marfan’s

A
  • Inherited disorder that affects connective tissue
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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
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3
Q

Marfan’s risks

A
  • Men and women affected equally
  • Occurs among all races
  • Greatest risk is having parent with disorder
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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
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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
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6
Q

Elhers-Danlos

A
  • Inherited disorder that affects connective tissue- primarily skin, Jts, blood vessel walls
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7
Q

Elhers-Danlos cause

A
  • Variety of genetics
  • 50% chance of passing onto children if you have hypermobile Ehlers-Danlos
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8
Q

Elhers-Danlos S+S

A
  • Overly flexible Jts- connective tissue is loose
  • Stretchy skin
  • Fragile skin- damage often wont heal
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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
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10
Q

Lupus

A
  • Immune system attacks own tissue and organs
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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
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12
Q

Lupus prognosis

A
  • Corticosteroids (prednisone) helps reduce swelling, tenderness and P
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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
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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
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15
Q

Systematic sclerosis population

A
  • Mostly affects women
  • 30-50
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16
Q

Systematic sclerosis risks

A
  • Genetics
  • Enviro triggers- medication/drugs, exposure to chemicals
  • Immune system problems
17
Q

Systematic sclerosis S+S

A
  • Variable dependent on location affected
  • Skin-related- hardening and tightening, firstly affects fingers, hands, feet and face, early stages are swelling and itchiness, affected skin can become lighter or darker in colour and may look shiny due to tightness
  • Raynaud’s- vasoconstriction of vessels in fingers and toes in response to cold/emotional stress, digits turn white, blue or red and feel painful or numb
  • Digestive problems- heartburn, difficulty swallowing, bloating, diarrhoea, constipation
  • Heart and lung- shortness of breath, decreased exercise tolerance, dizziness
18
Q

Systematic sclerosis prognosis

A
  • Ranges in severity
  • Pain relief through NSAIDs
  • Ease itching with moisturisers
19
Q

Polymyositis

A
  • Common inflammatory disease that causes muscle weakness affecting both sides of body
20
Q

Polymyositis cause

A
  • Unknown
  • Shares characteristics with autoimmune disorders
21
Q

Polymyositis population

A
  • Adults 30-50
  • Women affected more than men
22
Q

Polymyositis risks

A
  • Higher if you have lupus, RA, scleroderma or Sjogren’s syndrome
23
Q

Polymyositis S+S

A
  • Muscle weakness, closest to trunk (hips, shoulders, upper arms and neck)
  • Weakness is bilateral and tends to gradually worsen
24
Q

Polymyositis prognosis

A
  • Complications- difficulty swallowing, aspiration pneumonia, breathing problems
  • Azathioprine and methotrexate
25
Q

Dermatomyositis

A
  • Uncommon inflammatory disease marked by muscle weakness and distinctive skin rash
26
Q

Dermatomyositis cause

A
  • Unknown
  • Characteristic of autoimmune
  • Genetics and enviro factors play role- e.g., viral infections, sun exposure, certain medication, smoking
27
Q

Dermatomyositis population

A
  • Adults and children
  • Adults- late 40s-60
  • Children- 5-15
  • Females
28
Q

Dermatomyositis S+S

A
  • Can appear suddenly or gradually
  • Skin changes- violet-coloured or dusky red rash, commonly on face and eyelids, knuckles, elbows, knees, chest and back. Can be itchy and painful (usually first sign)
  • Muscle weakness- closest to trunk, bilateral, gradually worsens
29
Q

Dermatomyositis prognosis

A
  • No cure
  • Corticosteroids
  • Sun cream
  • Antimalarial medication
30
Q

Vasculitis

A
  • Inflammation of blood vessels
  • Causes thickening which reduces width of passageway for blood
  • Can lead to organ/tissue damage due to reduced flow