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21 Cards in this Set
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Musculoskeletal System Lecture 10,11 Disease of the Bone
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Musculoskeletal System Lecture 10,11 Disease of the Bone
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10,11Q:
What the heck is achondroplasia? |
It's the most common form of inherited dwarfism.
autosomal dominant |
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10,11Q:
Whats the problem with achondroplasia? |
Mutation in fibroblast growth factor receptor 3 (FGFR3)
Activation of FGFR3 inhibits cartilage synthesis at the epiphyseal growth plate, resulting in decreased enchondral bone formation and premature ossification of the growth plates. The long bones become short and thick, leading to short extremities, and dwarfism. but don't you worry, cranial and vertebral bones are spared, and intelligence, lifespan, and reproductivity is normal. |
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10,11Q:
Whats the hereditary defect in Osteogenesis Imperfecta? |
abnormal synthesis of type I collagen.
OGI is also known as Brittle Bone Disease. |
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10,11Q:
What do you see with those with OGI? |
osteopenia (brittle bones), recurrent fractures, bone deformity, thin sclera with a blue hue, hypermobility, deafness, dentin deficiency
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10,11Q:
Type I Osteogenesis Imperfecta |
Autosomal dominant
Fractures Blue sclerae Hearing Loss Little progression after puberty |
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10,11Q:
Type II Osteogenesis Imperfecta |
Autosomal recessive
Stillborn infant or death after birth with generalized crumpled bones. *most serious type |
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10,11Q:
Type III Osteogenesis Imperfecta |
Autosomal dominant or recessive
Progressive Multiple fractures Severe skeletal deformity Dentinogenesis imperfecta Hearing loss Blue to white sclerae |
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10,11Q:
Type IV Osteogenesis Imperfecta |
Autosomal dominant
Variable severity Fractures Skeletal deformity Normal sclerae Sometimes dentinogenesis imperfecta |
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10,11Q:
Osteopetrosis hereditary defect |
decreased osteoclast function, leading to decreased resorption and thick sclerotic bones
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10,11Q:
Paget Disease (Osteitis Deformans) 3 Stages |
1. Osteolytic: osteoclastic activity predominates
2. Mixed osteolytic – osteoblastic 3. Osteosclerotic: osteoblastic activity predominates “burnout stage” |
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10,11Q:
Osteopetrosis Hereditary defect, pathology |
decreased osteoclast function, leading to decreased resorption and thick sclerotic bones. thick bones still break easily. Myelophthisic process may lead to pancytopenia due to narrowing and fibrosis of medullar cavities. Cranial Nerve Compression!
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10,11Q:
Clinical Forms of Osteopetrosis |
Autosomal recessive (malignant type)
Affects infants and children Multiple fractures Autosomal dominant (benign type) Affects adults Fractures Mild anemia Cranial nerve impingement Carbonic anhydrase II deficiency Autosomal recessive Renal tubular acidosis and cerebral calcification Early death due to anemia, infection or hemorrhage |
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10,11Q:
Most Common Bone Disorder in the United States of America. |
Osteoporosis
-most commonly occurs with postmenopausal and elderly women -decreased bone mass (osteopenia), resulting in thin, fragile bones that are susceptible to fracture -estrogen deficiency -genetic factors -lack of exercise, old age, nutrition |
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10,11Q:
Definition of Padget Disease? |
localized disorder of bone remodeling, resulting in excessive bone resorption followed by disorganized bone replacement, producing thickened but weak bone that is susceptible to deformity and fracture
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10,11Q:
How do the vertebral bodies change during Osteoporosis |
Osteoporotic vertebral body is shortened by compression fractures, compared with a normal vertebral body. Note that the osteoporotic vertebra has a characteristic loss of horizontal trabeculae and thickened vertical trabeculae.
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10,11Q:
common feature of Osteomalacia and Rickets |
both diseases are characterized by decreased mineralization of newly formed bone, usually caused by deficiency or abnormal metabolism of vitamin D
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10,11Q:
Osteomyelitis |
1.Pyogenic osteomyelitis
-Hematogenous spread the most commonn route of infection -Seeding of bone after bacteremia -Commonly affects the metaphysis 2.Tuberculous osteomyelitis -1% of TB cases -lumbar and vertabrae destruction |
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10,11Q:
Ewing Sarcoma |
Malignant neoplasm of undifferentiated cells arising within the marrow cavity
-classic translocation t(11;22), which produces the EWS-FLI1 fusion protein -concentric “onion skin” layering of new periosteal bone |
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10,11Q:
Most common primary malignant tumor of bone |
Osteosarcoma (osteoginic sarcoma)
Codman’s triangle (periosteal elevation) “Sunburst” pattern Bone destruction Often involves the metaphyses of long bones Usually around the knee (distal femur and proximal tibia) Large, firm, white-tan mass with necrosis and hemorrhage |
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10,11Q:
Whats more common, metastasis of bone or primary bone tumors? |
metastasis of bone is. It's the most common form of bone tumor.
Common primary sites are: Prostate (often osteoblastic) Breast Lung Thyroid Kidney |
