Chapter 20 Flashcards
What is Dysostosis
Developmental anomaly of bone
What gene is altered in dyostosis
Homeobox gene
What is defective ossification of fetal cartilage
Dysostosis
What are the three types of Dysostosis
Aplasia (absent or incomplete development)
Supernumerary digits
Abnormal fusion of bone
What is Dysplasia
Mutations that interfere with growth or homeostasis (Dwarfism)
What is Dysplasia in bone
Osteodysplasia
What is Dysplasia in Cartilage
Chondrodysplasia
What is another name for Osteogenesis imperfecta
Brittle bone disease
What is mutated in Osteogenesis imperfecta
Type 1 collagen
If osteogenesis imperfecta autosomal dominant or recessive
Autosomal dominant
How many types of osteogenesis imperfecta are there
2
What type of osteogenesis imperfecta is letal in utero
Type 2
Children with osteogenesis imperfecta are treated with what drug
Bisphosphonates
What is the appearance of bone in an X-ray of a person who has osteogenesis imperfecta that has been treated with bisphosphonates
Zebra or striped
What is the most common form of Dwarfism
Achondroplasia
What is the main problem in Achondroplasia
decreased cartilage synthesis = Decrease in growth plate expansion
What gene is mutated in Achondroplasia
Fibroblast growth factor receptor (FGFR3)
What percentage of Achondroplasia patients have a spontaneous mutation Vs. autosomal dominant
Spontaneous 75%
Autosomal Dominant 25%
What are some signs of Achondroplasia
Short proximal extremities
Lower extremity bowing
Frontal bossing
Midface hypoplasia (low nasal bridge)
What type of dwarfism is fatal
Thanatophoric dwarfism
why is thanatophoric dwarfism fatal
Extremely small thorax leads to perinatal respiratory failure
What type of appearance do the vertebrae have in patients with Achondroplasia
Bullet shaped
What are the pathological causes of death in Achondroplasia
Brain stem compression
Cardiovascular abnormalities
Rare genetic condition where there is a decrease in osteoclast-mediated bone resorption
Osteopetrosis
What are patients with Osteopetrosis prone to having
Pathological fractures
What are some problems associated with osteopetrosis
Foraminal stenosis
Hepatospelnomegaly
Recurrent infections
anemia
How do you treat osteopetrosis
Stem cell replacement
What is osteoporosis
Bone becomes more porous thus decreasing bone mass
What are the two categories of osteoporosis
Localized
Entire skeleton
What type of generalized osteoporosis is most common
Primary
What causes Primary generalized osteoporosis
Old age (decrease GF +osteoblast activity) Postmenopausal (decrease estrogen)
What are some causes of secondary generalized osteoporosis
Neoplasia Nutrient deficiency Drug exposure (corticosteroids, smoking, alcohol)
Is osteopetrosis congenital or acquired?
Congenital
Is osteoperosis congenital of acquired
Acquired
What type of bone is most impacted by osteoporosis
Spongy bone
in postmenopausal women what happens to bone when estrogen decreases
Acceleration of bone loss
How can osteoporosis in women be prevented
Diet and exercise at an early age to develop bone, and continue on into later years
Ca++ and Vit.D supplementation
What are the common fractures associated with osteoporosis
Vertebral compression (thoracolumbar) Femoral neck fracture
What is the curve/hump called due to vertebral compression from osteoporosis
Dowager’s hump
Having vertebral compression due to osteoporosis can lead to what infection
Pneumonia
What is another name for Paget Disease
Osteitis deformans
What is happening in Paget disease
Bone mass increases, but it is irregular and weak due to osteoclastic activity and bone formation
What is the appearance of pagets disease on an X-ray
Saggy appearance
What can someone do to prevent/slow the process of paget disease
Increase serum alkaline phosphatase (by product of osteoblast activity)
What are the two types of paget disease
Idiopathic (antigens on osteoclasts)
Asymptomatic (most common)
What are some features of paget disease
Bone pain
osseous deformation
Nerve compression
1% can transition to sarcoma
What are the most common sites for paget disesase
Femur
Pelvis
Sacrum
Skull
What age is paget disease most common
Adults at age 70
What sex is most at risk of developing paget disease
Males
What disease is associated with the Ivory vertebra sign
Paget disease
What causes Vitamin D deficiency
Poor diet
UV deficiency
Abnormal metabolism
What happens in Vitamin D deficiency
Hypocalcemia
Bone demineralization
Bones become soft
Vitamin D deficiency in children causes
Rickets
Vitamin D deficiency in Adults causes
Osteomalacia
Vitamin D deficiency in adults is associated with what
Hyperparathyroidism
What hormone is secreted by the parathyroid glands
Parathyroid hormones
Excessive parathyroid hormone is associated with what
Renal failure
Adenoma (80% of cases)
What is the job of parathyroid hormone
Maintain serum Ca++
What is a brown tumor
hemorrhage, macrophages and fibrosis of bone, not an actual tumor
What causes brown tumors
Resorption or cortical trabecular bone from excessive osteoclast activity
What does an x-ray of a persons skull with hyperparathyroidism look like
Salt and Pepper skull
What are the categories of Fratures
Complete incomplete Closed Compound Comminuted Displaced
What type of fractures are most common in children
Incomplete
What is the fracture that occurs when skin is ruptured
Compound
What fractured is fragmented/splintered
Comminuted
What fracture has the distal segment misaligned
Displaced
What are pathological fractures
Fractures at the site of disease
What is a stress fracture
A fracture that develops slowly over time due to repetitive injury
Where are stress fractures most common
In the lower legs and feet
How long does it take for a fracture to heal (formation of bony callus)
6-8 weeks
What can cause delayed healing of a fracture
Nonunion Comminuted inadequate immobilization infection nutritional deficiencies Advanced age
What is avascular necrosis
Ischemia in bones leading to bony necrosis (infarction)
What causes avascular necrosis
Vascular disruption
how can an avascular necrosis present itself to a person
Asymptomatic or painful
Worsens with time, activity
What areas are most likely to have avascular necrosis
Subchondral area (hip, knee, shoulder, wrist, ankle)
What is the new bone growth that follows avascular necrosis called
Creeping substitution
What is Osteomyelitis
Bone marrow inflammation
What happens to the bone in osteomyelitis
Leukocytes destroy bone
How can bone marrow become infected
Hematogenus (sepsis, most common)
Adjacent infection
Traumatic implantation (compound fractures)
What are the symptoms of osteomyelitis
Acute fever
Malaise
Throbbing pain
What are the categories of osteomyelitis
Pyogenic bacteria
Mycobacterium tuberculosis
What is the most common bacterial cause of pyogenic osteomyelitis
Staphylococcus aureus
What are the features of pyogenic of osteomyelitis
Acute fever
Malaise
Throbbing pain
What bony feature associated with pyogenic osteomyelitis
Involcrum
Sequestrum
Draining sinus
What causes Tuberculous osteomyelitis
TB that has moved to the skeleton
What spinal abnormality is associated with tuberculous osteomyelitis
Pott disease
What type of granulomas are fromed in tuberculous osteomyelitis
Caseous granulomas
What does tuberculous osteomyelitis cause
Bony destruction
Severe deformation
What is the most common feature of a bone tumor
Bone pain that gradually increases
What are other signs of a bone tumor
age (50+ yr) History of cancer unexplained wt. loss no improvement from chiro care Pain doesn't go away when resting Bone pain more than 1 month
What are the types of primary bone cancer
Osteosarcoma
Shondrosarcoma
Ewin sarcoma
What are the benign bone tumors
Osteochondroma
Fibrous Cortical defects
What is the most common location and age for osteosacroma
Age 10-20
Location Knee
What is the most common location and age for osteoma
Age 40-50
Location Facial bones/skull
Where are the most common location for most bone tumors
Long bones of extremities
What are the main types of bone-forming tumors
Osteoma
Osteoid osteoma
Osteoblastoma
Osteosarcoma
How do you determine if there is a bone forming tumors
Biopsy
What type of bone tumor is a developmental anomaly and reactive growth
Osteoma
What are some characteristics of an Osteoma
Similar to normal bone
Slow growing
Benign
Where are Osteomas most common
Head
neck
What age do osteomas occur
40-50 years
What type of bone tumor is round-to-oval and is near the cortex of bone
Osteoid Osteoma and Osteoblastoma
What sex and age are Osteoid Osteoma and Osteoblastoma most common
Male
10-20
How do Osteoid Osteoma and Osteoblastoma look on an x-ray
They have a central nidus with a rim of sclerosis
What will a patient say about the pain related to an Osteoid Osteoma and Osteoblastoma
Bone pain at night
Features of an Osteoid Osteoma
Localized night pain
Relieved by aspirin
Near cortex of long bones
Small
Features of an Osteoblastoma
Large 2-6 cm
Poorly localized night pain
not relieved by asprin
Malignant transformation rare
What is the common location of an Osteoblastoma
Vertebral column