ToB S7 - Ossification and Bone Disease Flashcards

1
Q

Outline endochondral ossification

A

Long bone

  • Bone formation in cartilage model, which is reabsorbed and replaced by long bone
  • Begins as primary centre of ossification in the shaft (diaphysis) and later the secondary centres form at each end (epiphysis)
  • Growth in diameter is by deposition of bone at the periphery of the shaft
  • Growth in length occurs at the cartilaginous epiphyseal growth plate
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2
Q

Outline intramembranous ossification

A

Flat bone

  • Bone development starts in highly vascularised connective tissue (Eg flat bones of skull)
  • This focus of activity is the primary centre of ossification
  • Mesenchymal cells differentiates into osteoblasts which are surrounded by collagen fibres and ground substances
  • Osteoblasts secrete uncalcified matrix (osteoid), which later becomes calcified
  • Osteoblasts become osteocytes (embedded in osteoid)
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3
Q

Describe osteogenesis imperfecta

A
  • Characterised by bone fragility
  • Predisposes the sufferer to fractures and other deformities (other connective tissue abnormalities of eyes, tooth, joints, teeth and skin)
  • Multiple fractures are present at, or before birth, these often fatal
  • Defect in Type I collagen
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4
Q

Explain the importance of Vitamin D in normal bone development

A
  • Both dietary and synthesised vitamin D (in skin under influence of sunlight) is essential for normal ossification as it is involved in the absorption of calcium and phosphate by the small intestine
  • In it’s absence a poorly mineralised, pliable matrix known as osteoid (the uncalcified matrix secreted by osteoblasts) is formed
  • Affected bones are unable support the body weight and bend
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5
Q

Describe the histological changes occurring in osteoporosis

A
  • Bone density is reduced to a point where there is risk of fractures
  • The collagen framework and deposited minerals are broken down much faster than they are formed Osteoclast activity > osteoblast activity
  • Medullary canals in the centre of the bone become enlarged and gaps develop in the lamellae, making the bone fragile
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6
Q

List the most common risk factors for osteoporosis

A
  • Age
  • Women are more susceptible because of rapidly falling levels of oestrogen after the menopause - Poor diet
  • Insufficient exercise
  • Smoking
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7
Q

Outline achondroplasia

A
  • Achondroplasia is a congenital and often hereditary skeletal disorder, caused by failure of proliferation and column formation of epiphyseal cartilage cells
  • The defect is in endochondral bone formation
  • Impairs longitudinal growth of tubular bones
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8
Q

Describe the histology of a achondroplasia sufferer

A
  • Epiphyseal growth plates are thin
  • There are few cells in the proliferating zone
  • Hypertrophic cartilage cells form irregular columns
  • The zone of provisionally calcified cartilage is small and does not provide adequate scaffolding for bone matrix deposition by metaphyseal osteoblasts
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9
Q

What does excessive growth hormone cause (bone)?

A

Gigantism

In the adult, increase in the length does not occur because of the lack of epiphyseal growth plate cartilage, however bone increase in width by periosteal growth Acromegaly - long bones become very thick

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10
Q

What does lack of growth hormone cause (bone)?

A

Pituitary dwarfism

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11
Q

What cells provide the starting point for intramembranous ossification?

A

Mesenchymal cells

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12
Q

What is the genetic basis of osteogenesis imperfecta?

A

Autosomal dominant

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13
Q

What is the potential medicolegal importance of osteogenesis imperfecta?

A

Mutation in gene for type I collagen means that a person is more susceptible to fracture, multiple fractures could lead to concerns regarding deliberate injury

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14
Q

What are the two types of osteoporosis?

A

Type I - affects post-menopausal women only due to increase in osteoclast number due to decrease in oestrogen

Type II - occurs in elderly (above 70) of both sexes due to attenuated osteoblast function

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15
Q

Why is osteoporosis an important consideration in elderly patients?

A

Elderly people are more likely to fall and so osteoporosis will increase the likelihood of fractures when falls happen which can have severe clinical consequences

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16
Q

What is the genetic cause of achondroplasia?

A

Autosomal dominant point mutation

17
Q

Describe the appearance of an achondroplasia sufferer

A
  • Short limbs
  • Vault of skull enlarged
  • Small face
  • Flattened bridge of nose
  • Normal trunk length
18
Q

What is acromegaly?

A

Excessive growth hormone in adults leads to increase in bone diameter due to promoting periosteal growth

19
Q

What is the effect of excess growth hormone?

A

Before puberty - Gigantism

Adults - Acromegaly

20
Q

What results as a result of thymine deficiency?

A

Cretinism

21
Q

What is the consequence of precocious sexual maturity?

A

Epiphyseal growth plates fuse prematurely which retards bone growth

22
Q

What is the role of sex hormones on bone?

A

Influence appearance and development of ossification centres