Osteogenesis imperfecta
-results in thin poorly developed bones, short limbs, hearing loss due to otoscelrosis, prone to multiple fractures
-occurrence: 20,000-50,000
defective synthesis of collagen I alpha chains
What is the cause of Osteogenesis imperfecta?
Developmental hip dysplasia
-unstable, subluxated, or complete dislocated
-occurrence: 1 in 100 have instability; 1 in 1000 have complete dislocation
-more frequent in girls
scoliosis
-lateral deviation of spine
-girls are affected most of the time
b
Scoliosis is defined as _________
a. cryptogenic
b. idiopathic
c. agnogenic
osteopenia
-lack of bone mass
-reduction in bone mass greater than expected
-includes all metabolic bone diseases
osteoporosis
Which is more debilitating, osteopenia or osteoporosis ?
osteoporosis
-loss of mineralized bone mass causing porosity
-associated with aging, gender, genetics, activity level, and nutrition
-susceptible to fractures
postmenopausal caucasian and asian women
Which demographic has the highest rate of osteoperosis?
TNF, IL-1, IL-6 cytokines
Decreased estrogen is associated with increased ___________ that stimulate osteoclast precursors.
estrogen replacement therapy, SERMs
Metabolic treatment for women?
testosterone, biphosphonates, calcitonin
Metabolic treatment for men?
biphosphonates
-analogs of endogenous inorganic pyrophosphates
-bind to hydroxyapatite to prevent bone reabsorption by inhibiting osteoclast activity
calcitonin
-endogenous peptide that partially inhibits osteoclastic activity
osteomalacia
-softening of bones not involving bone matrix loss
-inadequate mineralization of bone (adults)
rickets
-failure/delay in calcification of growth plate
-skeletal pain and deformities, bow legged
Paget disease
-local areas of excessive bone turnover lesions with increased osteoblastic deposition of bone
-chaotic in fashion; causes bone deformities
-2nd most common bone disease
first degree family member w disease, paramyxovirus particles in osteoclasts
What are the causes of Paget disease?
bisphosphonates and calcitonin
Treatment for Paget disease?