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What is the main constituent of the inorganic mineral component of bone matrix?
What is the main constituent of the inorganic mineral component of bone matrix?
Which cells are responsible for bone formation?
Which cells are responsible for bone formation?
What is the function of the periosteum?
What is the function of the periosteum?
What is the composition of the organic component of bone matrix?
What is the composition of the organic component of bone matrix?
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Which factor reflects exuberant bone turnover in Paget disease?
Which factor reflects exuberant bone turnover in Paget disease?
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In which percentage of patients can Paget's disease lead to the development of osteogenic sarcoma?
In which percentage of patients can Paget's disease lead to the development of osteogenic sarcoma?
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What can hypervascularity in extensive polyostotic Paget disease lead to?
What can hypervascularity in extensive polyostotic Paget disease lead to?
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Which symptoms are associated with Paget disease?
Which symptoms are associated with Paget disease?
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What is the process that involves the formation of an organizing hematoma and a pro-inflammatory state at the fracture site?
What is the process that involves the formation of an organizing hematoma and a pro-inflammatory state at the fracture site?
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What factors are released by platelets, inflammatory cells, and fibroblasts during fracture healing?
What factors are released by platelets, inflammatory cells, and fibroblasts during fracture healing?
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What is the transformation of the soft callus into during fracture healing?
What is the transformation of the soft callus into during fracture healing?
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What frequently results in deformity in fractures?
What frequently results in deformity in fractures?
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What can inadequate immobilization lead to in fracture healing?
What can inadequate immobilization lead to in fracture healing?
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What delays healing and may never completely normalize in fracture cases?
What delays healing and may never completely normalize in fracture cases?
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Which type of bone marrow begins to appear in the distal bones of the extremities around age 5-7?
Which type of bone marrow begins to appear in the distal bones of the extremities around age 5-7?
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In adult life, which of the following bones contain red bone marrow?
In adult life, which of the following bones contain red bone marrow?
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Which cells of bone include osteoblasts, osteoclasts, and osteocytes, each with specific functions?
Which cells of bone include osteoblasts, osteoclasts, and osteocytes, each with specific functions?
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Which process is involved in the development of long bones from a cartilage mold?
Which process is involved in the development of long bones from a cartilage mold?
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What is involved in the growing process of bone radially?
What is involved in the growing process of bone radially?
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What is involved in the formation of epiphyseal plate?
What is involved in the formation of epiphyseal plate?
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What is involved in the tightly regulated process of adult skeleton remodeling?
What is involved in the tightly regulated process of adult skeleton remodeling?
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At what stage is peak bone mass achieved?
At what stage is peak bone mass achieved?
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Which cells regulate osteoclast differentiation and bone resorption?
Which cells regulate osteoclast differentiation and bone resorption?
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What is the process under the control of locally produced substances and hormonal effects?
What is the process under the control of locally produced substances and hormonal effects?
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In what two forms is the bone matrix synthesized?
In what two forms is the bone matrix synthesized?
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What process involves the resorption of bone exceeding formation, resulting in a decline in skeletal mass?
What process involves the resorption of bone exceeding formation, resulting in a decline in skeletal mass?
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Which receptor triggers OPG production on osteoblasts?
Which receptor triggers OPG production on osteoblasts?
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What is the cause of achondroplasia?
What is the cause of achondroplasia?
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Which condition is caused by FGFR3 mutations and is a lethal variant of dwarfism?
Which condition is caused by FGFR3 mutations and is a lethal variant of dwarfism?
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What is the cause of osteogenesis imperfecta?
What is the cause of osteogenesis imperfecta?
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What characterizes osteopetrosis?
What characterizes osteopetrosis?
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What does osteopenia refer to?
What does osteopenia refer to?
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How is osteoporosis characterized?
How is osteoporosis characterized?
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What is primary osteoporosis commonly associated with?
What is primary osteoporosis commonly associated with?
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What is the characteristic feature of dysostosis?
What is the characteristic feature of dysostosis?
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What are the consequences of osteopetrosis?
What are the consequences of osteopetrosis?
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What do LRP5/6 receptors trigger on osteoblasts?
What do LRP5/6 receptors trigger on osteoblasts?
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What is the result of defective type I collagen synthesis?
What is the result of defective type I collagen synthesis?
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Which of the following factors contribute to osteoporosis pathogenesis?
Which of the following factors contribute to osteoporosis pathogenesis?
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What is the impact of thoracic and lumbar vertebral fractures in osteoporosis?
What is the impact of thoracic and lumbar vertebral fractures in osteoporosis?
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Which specialized radiographic techniques are used to assess bone mineral density?
Which specialized radiographic techniques are used to assess bone mineral density?
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What are the manifestations of vitamin D deficiency?
What are the manifestations of vitamin D deficiency?
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What is the result of hyperparathyroidism?
What is the result of hyperparathyroidism?
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Where is Paget disease of bone relatively common?
Where is Paget disease of bone relatively common?
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What is the primary treatment for osteoporosis prevention and management?
What is the primary treatment for osteoporosis prevention and management?
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What is the characteristic feature of rickets?
What is the characteristic feature of rickets?
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What can result from fractures of the femoral neck, pelvis, or spine?
What can result from fractures of the femoral neck, pelvis, or spine?
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What is the impact of osteomalacia in adults?
What is the impact of osteomalacia in adults?
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What is the impact of decreased calcium nutritional state on osteoporosis pathogenesis?
What is the impact of decreased calcium nutritional state on osteoporosis pathogenesis?
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What is the result of Paget disease of bone?
What is the result of Paget disease of bone?
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Study Notes
Congenital Bone Diseases
- LRP5/ and 6 receptors on osteoblasts trigger OPG production
- Dysostosis includes abnormalities in mesenchyme migration and ossification
- Achondroplasia, caused by activating FGFR3 mutation, results in severe stunted long bones
- Thanatophoric dysplasia, a lethal variant of dwarfism, is caused by FGFR3 mutations
- Osteogenesis imperfecta is caused by defective type I collagen synthesis
- Osteogenesis imperfecta results in extreme skeletal fragility and extraskeletal manifestations
- Osteopetrosis is characterized by defective osteoclast-mediated bone resorption
- Osteopetrosis can lead to cranial nerve palsies, recurrent infections, and hepatosplenomegaly
- Nutritional, endocrine, and systemic disorders affect skeletal system development
- Osteopenia refers to decreased bone mass, while osteoporosis is severe osteopenia
- Osteoporosis is characterized by reduced bone mass, leading to fragility and fractures
- Primary osteoporosis is common and may be associated with aging or postmenopausal state
Osteoporosis: Pathogenesis and Clinical Course
- Osteoporosis leads to a significant increase in the risk of fractures in the vertebrae and hips.
- Age-related changes, reduced physical activity, genetic factors (e.g. LRP5 mutations), decreased calcium nutritional state, and hormonal influences contribute to osteoporosis pathogenesis.
- Thoracic and lumbar vertebral fractures are common in osteoporosis, leading to kyphoscoliosis and compromising respiratory function.
- Fractures of the femoral neck, pelvis, or spine can result in complications such as pulmonary embolism and pneumonia, leading to deaths.
- Osteoporosis is difficult to diagnose as it is asymptomatic until a fracture occurs and cannot be reliably detected in plain radiographs until 40% of bone mass is lost.
- Specialized radiographic techniques like dual-energy absorptiometry and quantitative computed tomography are used to assess bone mineral density.
- Osteoporosis prevention and treatment involve dietary calcium intake, vitamin D supplementation, regular exercise, and pharmacologic treatments including antiresorptive and osteoanabolic agents.
- Rickets and osteomalacia are manifestations of vitamin D deficiency, leading to impaired mineralization and unmineralized bone matrix accumulation.
- Rickets affects children, interfering with bone deposition in growth plates, while osteomalacia affects adults, resulting in under-mineralized bone formed during remodeling.
- Hyperparathyroidism results in increased osteoclastic activity, bone resorption, and osteopenia, affecting the entire skeleton.
- Excessive or inappropriate levels of parathyroid hormone can result from autonomous parathyroid secretion (primary hyperparathyroidism/parathyroid adenoma) or in the setting of underlying renal disease (secondary hyperparathyroidism).
- Paget disease of bone is a condition of increased, disordered, and structurally unsound bone, rare in Scandinavia, China, and Africa, but relatively common in much of Europe, Australia, New Zealand, and the United States, affecting up to 2.5% of the adult populations.
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Description
Test your knowledge of congenital bone diseases and osteoporosis with this quiz. Explore the pathogenesis, clinical course, and diagnostic and treatment strategies for these conditions affecting the skeletal system. Keywords: osteogenesis imperfecta, achondroplasia, osteopetrosis, osteoporosis, fractures, bone mineral density, osteoporosis prevention, rickets, hyperparathyroidism, Paget disease of bone.