METABOLIC disorders / 3 Flashcards
What are the 2 types of osteochondrosis
- Scheuermann’s Disease
2. Calve’s Disease
What is osteochondrosis
Interruption of blood supply of epiphysis of bone
What is osteochondrosis of primary center of ossification in the spine
Calve’s disease
What is osteochondrosis of secondary center of ossification on the spine
Scheuermann’s disease
What does Scheuermann’s result in
Kyphotic posture
What ages are effected by Calve’s disease
2-8
How many vertebrae is Calve’s disease limited to
1
What does Calve’s cause
Avascular necrosis of langerhan cells which creates granulomas
What are the types of osteoporosis
Type I and II
What is type I osteoporosis
Postmenopausal osteoporosis
Who gets type I osteoporosis
5-20% of women between 50-70
What is type II osteoporosis
Senile osteoporosis
Who gets type II osteoporosis
Women more than men over 70
What percent of women over the age of 45 will be affected by osteoporosis
30-45%
When does bone mass peak
25-35
How much bone mass loss occurs during the first 5 years of menopause
11%
How much bone mass loss occurs during the following 20 years of menopause
5%
How much calcium do premenopausal women need
Over 1000 mg
How much calcium do postmenopausal women need
Over 1500 mg
What is osteomalacia
Softening of bone without loss of bone matrix
What causes osteomalacia (2)
- Insufficient intestinal calcium
2. Increased renal phosphate losses
What is scurvy
Vitamin C deficiency which leads to failure of osteoblastic formation of bone matrix
What does scurvy cause
Hemorrhagic manifestations and abnormal formation of bones and teeth
What is arthrogryposis multiplex congenita
Nonprogressive neuromuscular syndrome characterized by multiple congenital contractures in and intact skeleton
What does arthrogryposis multiplex congenita cause
Contractures in flexion or extension, amyoplasia
What is distal arthrogryposis
Primarily affectin hands and feet
What is the incidence of arthrogryposis multiplex congenita per birth
1 in 3000/4000
What is osteogenesis imperfecta defined as
Brittle bones
How does osteogenesis imperfecta occur
Due to mutations in type I collagen affecting bone, teeth, and ligaments
How is osteogenesis imperfecta diagnosed
Skin biopsy revealing collagen mutation
What is another name for achondroplasia
Dwarfism
How do you diagnose achondroplasia
DNA test performed before birth
How many mutated genes are required for achondroplasia
2
What are indicators of achondroplasia
Slow motor development, walking doesn’t occur until 24-36 months, obesity, and otitis media
What is otitis media
Middle ear infection
How does achondroplasia present on an X-Ray
Large skull, narrow foramen magnum, short vertebral bodies, narrow spinal canal, fibular overgrowth, broad hands short metacarpals, and ribs are short
Achondroplasia is failure of what
Longitudinal growth in cartilage of epiphyseal plate
Marfan syndrome is hyper or hypochondroplasia
Hyperchondroplasia
Marfan syndrome has a defect in what
The gene that produces fibrilin
What does fibrilin do
Gives connective tissue its elasticity and strength
Clinical picture of Marfan syndrome
Tall, excessive length of limbs and trunk, hypermobility, chest wall abnormalities
What are the Ghent criteria (6)
- Enlarged aorta
- Aortic dissection
- Dislocation of lens
- Dural ectasia
- Skeletal problems
- Abnormal gene that causes Marfan
