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Rheumatology - Rutvi > Congential Acquired Dz > Flashcards

Congential Acquired Dz Flashcards

1
Q

What is the composition of bone?

A

65% inorganic - Ca hydroxyapatite – strength/hardness

35% - organic - Collagen = structure

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2
Q

What is osteoid?

A

nonmineralized bone, takes 12-15 days to mineralize

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3
Q

What are the various bone cell types?

A
  1. osteoprogenitor cells- become osteoblasts w/ CFBA1
  2. osteoblast - mononuclear
  3. Osteoclast - multinucleated
  4. osteocytes - mechanical stress, regulate serum Ca and phosphorous
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4
Q

What do osteoblasts do?

A

make osteoid; they are eventually surrounded by matrix, have PTH receptors (squamous cell carcinoma), feedback mechanisms for osteoclasts

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5
Q

Where are osteocytes found/

A

encased in bone, communicate via canaliculi

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6
Q

What do osteoclasts do?

A
  1. remodel, require cytokines for differentiation

2. resides in resorption pits

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7
Q

What cytokines do osteoclasts need for differentiation?

A

IL 1, 3, 6, 11, GMCSF, and MCSF

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8
Q

Interplay btw osteoblasts and clasts?

A
  1. osteoblasts and stromal cells have RANKL which binds to clasts RANKL-receptor w/ MCSF and makes functional osteoclasts.
  2. stromal cells also secrete OPG
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9
Q

What is OPG

A

decoy receptor for RANKL = prevents bone resorption, inhibits clast differentation

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10
Q

What happens in mature bone?

A
  1. epiphyseal plate ceased to grow
  2. metaphyseal vessels penetrate cartilage plate
  3. transverse bars of bone separate plate from metaphysis
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11
Q

What is osteogenesis Imperfecta?

A
  1. brittle bone dz, type 1 collagen mutations, teeth deformed, mostly AD
  2. different types exists - know that type 2 and 3 are fatal to kids
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12
Q

What are the 4 major clincal criteria for OI?

A
  1. osteoporosis
  2. blue sclera ( due to thinning of scerla allows underlying choroid to become visible)
  3. dentingenesis imperfecta
  4. premature otosclerosis
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13
Q

What is achondroplasia?

A

impaired formation of long bone, locus = FGF3 receptor, most are due to new mutations that are AD. Features = large forehead, prominent supraoribtal ridges, deep set root of nose

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14
Q

What is osteopetrosis?

A
  1. marble bone dz; osteoclast dysfunction = CA II deficiency??
  2. long bones w/ out medullary cavity, bullous ends, irregular trabeculae, woven bond = more prone to fractures
  3. most severe form = ARO
  4. Rx w/ osteoclast precursor via BMT
  5. pts are neutropenic and anemia
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15
Q

What is the pathogensis for osteopetrosis?

A

osteoclasts can’t resorb bone so bone marrow is occulded by weak, woven bone. the number of osteoclasts are normal. You can have a LOF mutation of RANKL.

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16
Q

What is osteoporosis?

A

systemic dz w/ low bone mass and microarchitectural deterioration of bone tissue (tin trabuclae w/ microfractures)

17
Q

How is the peak bone mass affected in osteoporosis?

A
  1. Menopause - decreases in estrogen, increases in RANKL, and increases in IL1/6/TNF
  2. Aging - decrease in osteoprogenitor replication, decrease in osteoblasts, decreased biologic activity of matrix bound GFs, decreased physical activity
18
Q

What is Paget’s dz of bone?

A
  1. unbalance and excessive osteoclast/blast function = increased bone turnover
  2. northern europeans
  3. paramyoviruses or measles
  4. mosaic pattern of lamellar bone
  5. predisposed to sarcoma
  6. irregular thick coarse cortex of bone
19
Q

What features are present in pagets dz?

A
  1. thick skull
  2. deafness
  3. kyphosis
  4. pain
  5. bowed legs
20
Q

What is osteomalacia?

A
  1. bone doesn’t mineralize due to not enough Vit D and/or Ca
  2. rickets in kids
  3. surface of bony trabeculae covered by thicker than normal layer of osteoid
21
Q

What are some causes of osteomalacia?

A
  1. Renal osteodystrophy - can’t make 1,25(OH) vit d due to tubular injury = hypocalcemia.
  2. HyperPTHism - increased PTH, Ca and increased Ca release fro bone, phosphate retention. Brown Tumor!!!
  3. decreased total bone mass
  4. increased bone formation in spine/long bones
22
Q

What is osteitis fibrosa cystica

A

increased bone activity and peritrabeular fibrosis and cystic brown tumors

23
Q

What are brown tumors caused by

A

Macs react to microfracturees and hemorrhage - hemosiderin deposits and fibrosis ensues

Rheumatology - Rutvi (7 decks)

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