CH 12--DISEASES OF BONES--Bone 2/Metabolic Diseases Flashcards

1
Q

Metabolic Disorders X 5

Includes:

  1. Rickets & osteomalacia
  2. Osteoporosis
  3. Paget’s disease
  4. Fibrous dysplasia
  5. Hypertrophic (_____________) osteoarthropathy
A

pulmonary

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2
Q
  1. Rickets & Osteomalacia–1

Features:
1— Due to vitamin D deficiency mineralization of bone
2— _______ in children
3— ____________ in adults

A

Rickets

Osteomalacia

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3
Q
  1. Rickets & Osteomalacia–2

Sources of Vitamin D:

(1) Diet: Meat and dairy products absorbed in _________ with fats.
(2) Skin: 7-dehydrocholesterol vitamin D by UVR in _______
—Liver: Hydroxylated into 25-hydroxyvitamin D (inactive)
—Kidney: Hydroxylated in proximal tubules =
1-25 dihydroxyvitamin D (active form) = INCREASES absorption of calcium & phosphorus from intestine = ________________ of skeleton

A

intestine

sunlight.

Mineralization

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4
Q
  1. Rickets & Osteomalacia–3

CAUSES of vitamin D deficiency:

  1. Inadequate exposure to SUNLIGHT
  2. Dietary Deficiency of vitamin D
  3. Malabsorption (e.g. biliary, pancreatic or
    intestinal dysfunction)
  4. Renal failure or liver disease (reduced hydroxylation)
  5. _____________ depletion:
    - poor intestinal absorbtion (e.g. antiacids)
    - impaired reabsorbtion in renal tubules
A

Phosphate

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5
Q
  1. Rickets & Osteomalacia–4

Clinical Features:

(a) Rickets in growing children = Failure of deposition of calcium phosphate in zone of calcified cartilage
b. Growth of cartilage without cartilage degeneration:
(1) Wide irregular epiphyseal line
(2) EXCESS cartilage
c. FAILURE of deposition of _______ ________ in newly formed bone
d. INCREASED amount of _______ __________tissue =
SOFT EASILY DEFORMED BONE!!

A

calcium phosphate

uncalcified osteoid

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6
Q
  1. Rickets & Osteomalacia–5
VITAMIN D PROBLEMS:
Skull: 
1--Delayed closure of \_\_\_\_\_\_\_\_\_
2--Delayed eruption of teeth
3--Excess \_\_\_\_\_\_\_ tissue at frontal and parietal prominences (frontal bossing)
4--Flattening of occipital bones

Chest:
1–Rachitic rosary: beaded costochondral junctions
2–Harrison’s sulcus: groove opposite origin of diaphragm
3–______ chest: flattening of sides & protrusion of sternum

Vertebral column:
1— Dorsal kyphosis
2—Lumbar lordosis
3—Scoliosis

Long bones:
1— __________ of weight bearing bones
e.g. tibia (forward), femur (outward)

A

fontanelles

osteoid

Pigeon

bending

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7
Q
  1. Rickets & Osteomalacia–6

VITAMIN D PROBLEMS CONTINUED

Osteomalacia (SOFT bone): - occurs in adults (e.g. pregnancy & lactation) = Bones need ______ and _______ to remain healthy and strong, but the body also needs vitamin D to be able to absorb these two minerals. Without this necessary vitamin, bones can become SOFT and flexible. This softening is called osteomalacia, or rickets when diagnosed in children. The condition is called osteomalacia when the bone softening occurs AFTER the growth plates have closed.

Pathogenesis: defective mineralization = soft, deformed bones = bone pain

A

calcium

phosphorus

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8
Q
  1. Osteoporosis–1

Features:
a— DECREASED total mass of bones (_______ = bone
atrophy)
b— Simultaneous ______ of organic matrix (osteoid
tissue) & minerals
c— Most common metabolic abnormality in USA (more than ____ million are affected and 1 million Americans experience a significant fragility fracture every year)

A

osteopenia

LOSS

15

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9
Q
  1. Osteoporosis–2

Etiology:
1. _________ ostoeporosis (aging): attenuated osteoblasts
2. POST-MENOPAUSAL (estrogen deficiency):
a—- _________ normally DECREASES release of IL-1
(osteoclast activation factor) from macrophages
b—- decreased estrogen levels AFTER menopause
INCREASES ____ release = stimulates osteoclasts
3. Prolonged immobilization = decreased physical
activity (disuse atrophy)
4. Endocrine disease = e.g. hyperparathyroidism

A

Senile

estrogen

IL-1

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10
Q
2. Osteoporosis--3
Clinical Features:
- affects \_\_\_\_ bones
- symptoms are felt in weight-bearing ones:
EX.  Vertebral bodies:
- change in shape
- decreased height
- compression fractures
- kyphosis, kyphoscoliosis

ALL THIS LEADS TO BACK PAIN

  • -Long Bones: pathological fracture (femur neck)
  • – widespread radiolucency
A

all

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11
Q
  1. Osteoporosis–4

Management:

  • Drug therapy like “________’ to help bone build up
  • Hormone replacement therapy
  • Exercise, physiotherapy modalities
  • Adequate dietary intake of calcium before age_____
  • Adjustment (may or may not be contraindicated)

Supplementation:

  • Calcium
  • Vitamin D

Osteoporotic vertebral body (right) shortened by
compression fractures compared with a normal verbral
body. The lamellar bone exhibits discontinuous, thin trabeculae.—SEE SLIDE 22!! WOW!

A

Evista

30

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12
Q
  1. Paget’s disease–Osteitis Deformans–1

Features:
- Common disease (3-4% of population over 60 years)

  • Etiology:
    1. Genetics
  • familial incidence
  • associated with certain HLA
  • high prevalence among British
    2. SLOW VIRUS INFECTION of ___________
A

osteoclasts

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13
Q
  1. Paget’s disease–Osteitis Deformans–2 = a chronic disease characterized by episodic accelerated bone resorption and growth of abnormal replacement bone, causing bone pain, deformation, fractures, and osteosarcoma; osteitis deformans.

Pathological lesions:
a—– Paget’s may involve one bone (monostotic) or many
(polystotic)
b—- Three phases: REMEMBER “L….C….B”
1— OsteoLYTIC stage:
- Focal areas of bone resorption
- Bone ____________ by vascular connective tissue
- Large osteoclasts with 12 or more hyperchromatic nuclei & virus-like inclusion bodies.
2—. OsteoCLASTIC-osteoblastic stage:
—NEW bone with _______ mineralization at margins
(tile or mosaic appearance)
3— OsteoBLASTIC stage:
- Excess osteoblastic activity → thickening of cortex & trabeculae
- SOFT, porous, poorly mineralized NEW bones
- ending in __________

A

replacement

decreased

osteosclerosis

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14
Q
  1. Paget’s disease–Osteitis Deformans–3
Complications:
- Malignant change → osteosarcoma (2-5%)
- Bone deformity or fracture
- Arterio-venous fistula → a state of hyperdynamic 
circulation → \_\_\_\_\_\_\_\_  \_\_\_\_\_\_\_\_\_\_
A

heart failure

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15
Q
  1. Paget’s disease–Osteitis Deformans–4

A radiograph of the spine shows vertebrae affected by paget disease. The vertebrae are ____ and _____ than normal, and display the characteristic “picture frame” appearance.

A

shorter

wider

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16
Q
  1. Paget’s disease–Osteitis Deformans–5
Clinical Manifestiations:
A---- Enlargement of \_\_\_\_\_\_\_bones:
i. Leontiasis ossea (Lion face)
ii. Nerve compression (Hearing loss)
iii. Kyphosis

B—- Vertebrae: collapse = __________
= Back pain
= Nerve compression
= Kyphosis

C—- Long bones = __________ of femur, tibia

A

skull

microfractures

bowing

17
Q
  1. Fibrous dysplasia—1

Features:
A— Focal replacement of bone by fibrous tissue.
B— Lesion starts at _____ ________ & extends into
cortex
C— Bone is replaced by:
i. fibroblasts
ii. collagen fibers
iii. scattered trabeculae of irregular bones
D— Affects: long bones, ribs, skull, & facial bones
E—- X-ray: well circumscribed radiolucent area = Pain,
Deformity, Pathological fracture.
F—- Either ________ = (single bone), in 70% or
_________ (many bones), in 25%

A

medullary cavity

monostotic

polyostotic

18
Q
  1. Fibrous dysplasia—2

__________syndrome: in 5% of cases Fibrous dysplasia associated with ENDOCRINE dysfunction

  1. multiple unilateral bone lesions
  2. unilateral pigmented skin (café-au-lait spots) over the buttocks, back, & sacrum
A

Albright

19
Q
  1. Fibrous dysplasia—3

EX. proximal femur shows a “______ _________” deformity, caused by fractures sustained over the years
.

A

shepherd’s crook

20
Q
  1. Hypertrophic (pulmonary) osteoarthropathy–1

Hypertrophic osteoarthropathy (HOA) is a syndrome of clubbing of the digits, periostitis of the long (tubular) bones, and arthritis.

Etiology:

  1. Lung:
    - bronchiectasis
    - abscess
    - carcinoma
  2. Heart:
    - bacterial endocarditis
    - congenital cyanotic heart disease
  3. Crohn’s disease
  4. Carcinoma of _________
A

thyroid

21
Q
  1. Hypertrophic (pulmonary) osteoarthropathy–2

Clinical Manifestations:
A— _______ of fingers
B— Periostitis = new bone formation
C— Arthralgia ( Arthralgia is the medical term for pain in the joints) & arthritis

A

Clubbing