Ch 20 Flashcards
- Musculoskeletal system aka ___
- Tissues include (3)
- Integrates with ___
- Locomotor system
- Osseous, muscular, and connective
- Nervous System
MSK has what three jobs?
Form, function, protection (kinetics/ biomechanical movement)
MSK - ___ system and ___ storage
hematopoietic, mineral
- Define dysostosis
2. what are the four characteristics w/ it
- developmental anomaly of bone
- abnormal mesenchymal migration
- defective ossification of fetal cartilage
- sporadic (isolated) or part of syndrome
- homeobox gene alterations
Define homeobox gene
structural development during embryologic development
What are three subtypes of dysostosis?
- Aplasia
- Supernumerary digits
- abnormal fusion of bones
Explain aplasia
absent of incomplete development, abnormal function
- Define Dysplasia
2. give examples of both bone and cartilage
- mutations interfere with growth or homeostasis (dwarfism)
2. B = osteodysplasia, C = chondrodysplasia
- Dysostosis: ___ syndrome
- it is ___ with ___
- ___ deformity
- Klippel Feil
- idiopathic w/ congenital fusion (any 2 C vertebrae)
- Sprengel’s
Syndactyly: the MC ___
congenital malformation of the limbs
- Osteogenesis imperfecta aka ___
- Mutations of ___
- it is ___ dominant
- abnormal collagen leads to ___
- It can affect (5)
- brittle bone disease
- type 1 collagen
- autosomal
- premature breakdown
- ECM :eyes, inner ear bones, skin, joints, and teeth
O.I. - Range of severity has two types:
- Type 1 = ___
- Type 2 = ___
- normal lifespan
2. lethal in utero (cerebral hemorrhage, respiratory failure)
Type I of O.I. -
- Abnormal collagen of ___
- diagnosed in ___
- what can occur (6)?
- sclera
- childhood
- fractures, extremity bowing, scoliosis, ligamentous laxity (dislocations), hearing loss, short stature
- O.I. has what sign?
2. What were they treated with?
- Zebra stripe
2. bisphosphonates (cyclical)
What is the MC form of dwarfism?
Achondroplasia
Achondroplasia -
- decreased ___ equals decreased ___ (MC in (2))
- ___ mutation which inhibits ___
- % are spondaneous or autosomal dominant
- What are four signs?
- Cartilage synthesis, growth plate expansion, femur and humerus
- Fibroblast growth factor receptor (FGFR3), epiphyseal (growth plate) chondrocytes
- 75% and 25%
- Short proximal extremities, lower extremity bowing, frontal bossing, midface hypoplasia (low nasal bridge)
Causes of dwarfism (4)?
various
- Turner syndrome
- hypothyroidism
- malnutrition
- O.I.
Achondroplasia -
- ratio
- What can happen to the spine (5)?
- How can it cause death?
- What is a sign of achondroplasia dealing with the hand?
- 1: 10,000
- hyper lordosis or kyphosis at birth, bullet vertebrae, scoliosis, spinal stenosis (foramen magnum stenosis, spinal canal stenosis)
- Brain stem compression or cardiovascular abnormalities
- trident hand
- What is the name given to the fatal type of achondroplasia?
- Ratio?
- Explain
- Thanatophori dwarfism
- 1: 20,000 births
- extremely small thorax and shortened long bones (perinatal respiratory failure)
Osteopetrosis -
- Two ways of describing?
- ___ stenosis leads to ___
- ___ fills and leads to deranged ___
- Treatment?
- decreased osteoclast mediated bone resorption or skeletal sclerosis (stone like which increases fractures)
- foraminal, cranial nerve palsies
- medullary cavity, hematopoieses (hepatosplenomegaly or recurrent infections/anemia)
- stem cell replacement
Osteopetrosis has no ___ with ___ deformity
medulla, erlenmeyer flask
Osteoporosis -
- Most important form of ___
- decreased ___ equals increased ___
- What are the two categories?
- Generalized has both primary and secondary subtypes, explain each
- Basically the disease attacks more on the ___ and less on the __
- osteopenia
- bone mass, porosity (bony fagility/fractures)
- Localized (disuse) and Generalized to entire skeleton
- primary = is the MC: senile, postmenopausal
- secondary = various causes: neoplasia, hyperparathyroidism, nutrient deficiency, drug exposure (corticosteroids, alcohol, smoking)
- inside, outside
Osteoporosis -
- MC impacts ____
- What does it cause?
- Locations?
- trabecular bone (cancellous, spongy)
- thinned cortex
- VB, femoral neck, calcaneus
Generalized Osteoporosis - Primary Osteoporosis:
SENILE
1. ___ related, normal phenomenon
2. decreased __/___activity
3. ___ retain normal activity causing an imbalance
4. % bone loss per year, beginning at what age?
POSTMENOPAUSAL
1. decrease ___ accelerates bone loss
2. % or P.M females, % of age matched males
- age
- GFs/osteoblast
- osteoclasts
- 0.5%, mid 20s
- estrogens
- 50%, 3%
Osteoporosis -
- Maximize bone density in ___
- What helps keep bone density?
- What is MC decreased in females?
- young adulthood
- diet and regular exercise (resistance, athletics)
- Ca++ and Vit. D supplementation
Osteoporosis -
- loss of ___ homeostasis
- Risks (6)
- asymptomatic until?
- Where are two common fracture sites?
- Explain each
- osteoclast/blast
- increased age, females, sedentary, family history, eating disorders, malnutrition/malabsorption
- skeletal fragility
- -Vertebral compression fracture (thoracolumbar): dowager’s hump, kyphoscoliosis
- Femoral Neck fracture: disabling, risk for pulmonary emboli
What machine is used to measure osteoporosis?
DEXA - dual energy xray absorptiometry
Osteoporosis -
- ___ are not sensitive enough, explain
- List three ways on how to prevent
- X-rays, non diagnostic for osteoporosis and require 30-40% decreased bone mass
- Physical activity/ regular exercise (best prior to age 30)
- dietary calcium and vitamin D
- antiresorptive pharmacologic agents (bisphosphonates)
Paget Disease -
- otherwise known as?
- __ phase leads to ___phase leads to exhaustion of cellular activity known as ___phase
- basically means increased ___, weak, with a ___ appearance
- regional ___ activity
- Excessive ___ consisting of disorganized ___ bone making bone?
- caused by?
- osteitis deformans
- lytic, mixed lytic and blastic, sclerotic
- bone mass, shaggy
- osteoclastic
- bone formation, lamellar, enlarged and fragile
- increased serum alkaline phosphatase (byproduct of osteoblast activity), idiopathic, paramyxoviridae antigens on osteoclasts
Paget Disease -
- MC associated with symptoms or assymptomatic (70-90%)
- becoming less ___ and less ___
- features depend on (2), but may include (4)
- asymptomatic (incidental x-ray findings, relatively benign)
- common, severe
- site and severity, bone pain (MC in neck and back), osseous deformation/fracture/increase vascularity), nerve compression (headache, visual/auditory), and 1% transition into sarcoma (poor prognosis)
Paget Disease -
- Lesions: solitary % and multiple %
- % of cases involve the axial skeleton or femur including (3)
- pathological ___ fractures or ___
- Adults: MC Dx at age ___
- Risks: males or females, parts of the world
- treatment?
- 15%, 85%
- 80%, pelvis, sacrum, skull
- “chalkstick”, bowing
- 70
- males (2x), europe, new sealand, australia (U.S. only 1-3%)
- bisphosphonates - intended to slow lytic phase
What disease is related to mosaic pattern/jigsaw puzzle?
Paget Disease
What three diseases can refer to “ivory vertebra sign”?
- paget disease
- metastatic cancer (prostate)
- lymphoma
Vit D Deficiency -
- defective ___
- hypocalcemia leads to ___ which demineralizes bone
- Name when associated with children, explain
- name when associated with adults, explain
- mineralization (dietary/UV deficiency or abnormal metabolism)
- PTH (unmineralized bony matrix: soft/brittle)
- Rickets, poor growth plate mineralization, more severe
- osteomalacia, remodeled bone is undermineralized, mild, similar to osteoporosis fractures, associated with hyperparathyroidism
Hyperparathyroidism -
- parathyroid glands secrete ___, maintains ___
- Explain primary hyperparathyroidism
- explain secondary hyperparathyroidism
- Excessive PTH: MC from ___ %, __ osteoclast activity, increased ___
- Dx : (2), MC nonmalignant cause of ___
- PTH, serum Ca++
- autonomous PTH production
- renal failure, mild
- adenoma 80%, increased, renal tubule resorption of Ca++ and Vit. D synthesis
- hypercalcemia, parathyroid immunoassay, hypercalcemia
Hyperparathyroidism -
- > 50% are ___
- MC people who get this are ___
- excessive ___ activity impacts ___
- resorption of (2) bone
- replaced by ___
- Possible ___
- asymptomatic
- post-menopausal women
- osteoclast, skeletal homeostasis
- cortical and trabecular
- loose connective tissue
- “brown tumor”
Hyperparathyroidism -
- What are four results?
- Which is the MC?
- Characterized by a “___”, which are?
- X-rays of this are termed ___
- renal stones, peptic ulcers, pathologic fractures, bowing of long bones
- renal stones
- salt and pepper skull, small erosions
- Rugger-Jersey spine
Brown Tumor -
- very ___ lead to ___ leads to ___ leads to ___
- Not a true ___
- brittle/fracture, hemorrhage, macrophages, fibrosis
2. neoplasm/tumor
Hyperparathyroidism -
- Features are ___
- list 8
- treatment depends on ___
- list 3
- reversible with ?
- widespread
- bone pain, fractures, deformation, kidney stones, nausea, anorexia, fatigue, decreased cognition
- cause
- drinking water, physical activity, avoidance of diuretics (water pills)
- normalization of PTH levels
Fractures -
- ___ osseous pathology
- What are the five categories?
- commone
- -complete vs incomplete
- closed
- compound (open)
- comminuted
- displaced