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Flashcards in Exam 2- Bones Deck (51):
1

Which cells have the RANK receptor?

Osteoclasts and Osteoclast Precursors

2

What happens when RANKL binds the RANK receptor on Osteoclasts?

Binding stimulates survival and generation of Osteoclasts and increases NFKB (not for keeping bone)

3

What is Osteoprotegrin?

Osteoprotegrin is produced by Stromal Cells or Osteoblasts and inhibites RANKL-RANK receptor interaction

4

What is M-CSF?

M-CSF is a cytokine produced by osteoblasts or stromal cells that results in the generation and survival of osteoclasts --> increased bone resorption and increased plasma Ca levels

5

What is the function of the WNT/Beta-Catenin Pathway?

WNT is produced from stromal cells and binds Osteoblasts (through LRP5 and LRP6) to increase secretion of Osteoprotegrin, blocking RANK-RANKL interaction

**Note that a LOF mutation in LRP5/6 --> Osteoporosis and a GOF mutation in LRP5/6 --> Drastic increase in bone mass

6

What is the main component of the bone's organic matrix?

Type 1 Collagen

7

What is Woven Bone?

Random Collagen deposition, pathologic, seen in rapid bone growth (ex-healing a fracture)

8

What are the subtyes of Lamellar Bone? Describe its characteristics

1. Compact Bone
2. Spongy Bone
-Stronger bone with ordered collagen deposition; replaces woven bone

9

What will be increased with increased Osteoblast activity?

Osteopontin (osteocalcin) and Alkaline Phosphatase

10

RANK Ligand is located on which cells?

Osteoblasts and Marrow Stroma Cells

11

The end of a bone (distal to the growth plate) is the

Epiphysis

12

The area beneath the growth plate is call the

metaphysis

13

The central region of a long bone is the

Diaphysis

14

What is dystosis?

LOCAL problems in migration of mesenchyme and its condensation

Ex- bradydactyly because of HOXD13 defect

15

What is dysplasia?

GLOBAL defect in regulation of skeletal organogenesis

Ex- achondroplasia

16

What is the defect causing Cleidocranial Dysplasia and describe the symptoms

RUNX2 transcription factor defect

-Short stature, abnormal clavicles, wormian bones, too many teeth

17

What is the cause of Achondroplasia?

FGFR3 mutation --> GOF that inhibits cartilage growth because of decreased chondrocyte proliferation in growth plates

18

Continuation of appositional and intramembranous bone formation in Achondroplasia results in what?

Thick cortical bone

19

What are the physical characteristics of Achondroplasia?

Short proximal limbs, normal trunk length, big forehead and head, depressed root of nose

Normal life span, intelligence and reproductive status

20

What is the cause of Thanatophoric Dwarfism and describe the symptoms

FGFR3 GOF mutation (same as Achondroplasia)

-See short, bowed limbs, frontal bossing with macrocephaly, bell shaped chest (so respiratory failure) and CLOVERLEAF Skull

21

What is Osteoporosis Pseudoglioma Syndrome?

Disease caused by LOF mutation of LRP5 that causes skeletal fragility and loss of vision

22

What is the cause of Osteopetrosis (Marble Bone Disease)?

Disease of decreased osteoclast bone resorption caused by a defect in RANKL, LRP5 GOF mutation, or Carbonic Anhydrase II (CAII) deficiency (so cannot acidify pit)

AD- benign, AR- malignant

23

What are the clinical findings of Osteopetrosis?

1. Extramedullary hematopoiesis --> hepatosplenomegaly
2. Bulbous long bones (erlenmeyer flask deformity)
3. Narrow neural foramina (mild cranial nerve defects)
4. Brittle Bones, multiple fractures
5. Mild anemia
**TREAT- with Bone Marrow Transplant

24

What is the cause of Osteogenisis Imperfecta?

Mutation in the alpha 1 or alpha 2 chains of TYPE 1 COLLAGEN

25

What are the clinical characteristics of Osteogenisis Imperfecta?

1. Brittle bones and multiple fractures (may decrease after puberty)
2. Blue Sclerae
3. Hearing Loss
4. Loose joints

26

What are mucopolysaccharidoses?

Defects in enzymes degrading dermatan sulfate, heparan sulfate and keratan sulfate --> abnormal hyaline cartilage --> malformed bones

27

What are some potential causes of Osteoporosis?

1. Nutritional (lack of Ca)
2. Menopause (increased RANK/RANKL expression --> increased osteoclast activity)
3. Physical Activity (space flight)
4. Aging (decreased replication of progenitor cells and synthetic activity of osteoblasts)
5. Vitamin D Receptor Polymorphism
6. Drugs, diabetes, malignancy, liver/ GI disease

28

What is osteoporosis?

A disease of decreased bone mass and increased porosity --> increased risk of fractures

**May be described as osteopenia (decreased bone mass) to the point of risk for fracture

29

What determines most hereditary osteoporosis?

Vit D receptor, Collagen 1A1 and the Estrogen Receptor

30

How does decreased estrogen result in Osteoporosis in post-menopausal women?

Decreased Estrogen --> increased inflammatory cytokines that increase RANKL and decrease OPG

**Estrogen replacement is effective treatment, but increases risk of breast cancer

31

What are common causes of secondary osteoporosis?

1. Hyperparathyroidism (most commonly caused by parathyroid adenoma)
2. Neoplasia
3. Multiple Myeloma
4. Vit. D Defficiency (ricketts and osteomylasia)
5. Anticoagulants
6. Corticosteroids
7. Immobilization

32

What is Dual-energy absorptiometry used for?

Detection of Osteoporosis

33

Describe the clinical features of Hyperparathyroidism

1. Increased osteoclast fxn --> subperiosteal resorption that thins the cortices
2. Loss of lamina dura around teeth
3. Bone loss on radial aspect of middle phalanges of index and middle finger
4. General Osteopenia
5. Brown Tumor- bone that is replaced by fibrovascular tissue (fractures will result in hemorrhage and you see granulation tissue and hemosiderin)

34

What is renal osteodystrophy?

-Altered OC/OB activity --> osteomalacia (delayed mineralization), osteosclerosis and osteoporosis
-Hyperparathyroidism
-Decreased activation of Vit D
-Metabolic Acidosis

35

What are the stages of Paget's Disease?

1. Osteolytic Stage- loss of bone mass due to osteoclasts (see very thick cortex with "V shaped blade of grass" lesion
2. Mixed Stage- Prominent Osteoblasts and Osteoclasts
3. Osteosclerotic Stage- mosaic pattern of lamellar bone- jigsaw puzzle like cement lines

36

What is the important histological finding of Paget's Disease?

Bone mosaic with disorganized lamellae (look like swirls)

37

What are the clinical findings of Paget's Disease?

1. Pain from microfractures
2. Increased Alkaline Phosphatase
3. Normal Ca and PO4
4. Bone Overgrowth causing
-Cranial Nerve Palsy
-Heavy skull that is hard to hold up
-Severe secondary osteoarthritis
-Chalk stick-type fracture

38

Paget's Disease can cause what clinical problems?

1. High-output cardiac failure
2. Tumors (benign and malignant)

39

What is used to treat Paget's Disease?

Calcitonin and Biphosphonates

40

Describe the order of healing after a fracture

Soft Tissue Callus (procallus) --> Boney Callus

Procallus- hematoma/fibrin framework that activates osteoprogenitor cells; is not rigid and can be easily disrupted
Boney Callus- made of woven bone +/- cartilage for enchondral ossification

41

What is avascular necrosis (osteonecrosis) and what are the common causes?

-Infarction of bone and marrow due to vessel injury; the dead bone/fat is replaced by Calcium Soaps

-Causes: *Corticosteroids, infection, dysbarsim, pregnancy, sickle cell disease

42

What is a medullary infarct?

Subtype of avascular necrosis; can be small or large; large are painful (ex- dysbarism, sickle cell)

43

What is a Subchondral Infarct?

Subtype of avascular necrosis that results in a WEDGE SHAPED subchondral bone crack; secondary collapse may --> osteoarthritis

44

How would osteomyelitis appear on an X-Ray?

As a lytic bone lesion with surrounding sclerosis

45

What are the bacterial causes of osteomyelitis?

1. S aureus
2. E coli, Pseudomonas, Klebsiella (infections of IV drug users)
3. H flue and Group B Strep (infants)
4. Salmonella (Sickle Cell Patients)

46

Describe the common locations for osteomyelitis for different age groups

1. Neonate- Metaphysis or Epiphysis
2. Children- Metaphysis
Adult- Epiphyses and subchondral bone

47

What is a Brodie Abscess?

A small intraosseous abscess often in the cortex walled off by reactive bone

48

What is Sclerosing Osteomyelitis of Garre

Osteomyelitis in the jaw with extensive new bone formation

49

A draining sinus of pyogenic osteomyelitis can develop into what?

Squamous Cell Carcinoma

50

What is the clinical characteristics of TB Osteomyelitis?

1. Occurs in immunosuppressed
2. Pott's Disease: Lumbar and Thoracic Spine --> breaks through discs to other vertebrae --> scoliosis and kyphosis

51

What are the features of syphilis of the bone?

Saddle Nose and Saber Shin (tibia)