Pathoma: Skeletal System Flashcards Preview

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Flashcards in Pathoma: Skeletal System Deck (55):
1

What is achondroplasia?

impaired proliferation on the growth plate...dwarfism

2

Genetic mutation cause of achondopliasia?

Activating mutation in fibroblast growth factor receptor 3 (FGFR3)

3

Process of endochondrial bone formation?

Formation of cartilage matrix which is then replaced by bone

4

Process of intramembranous bone formation?

Bone formation without preexisting cartilage matrix

5

What are the clinical features of someone with achondroplasia?

Short extremities with normal sized head and chest

Normal mental function, lifespan and fertility

6

What is osteogenesis imperfecta?

Congenital defect of bone formation resulting in structurally weak bone

7

What is the mutation found in osteogenesis imperfecta?

Collagen type I mutation

8

Is the OI mutation usually AR, AD, or x linked?

AD

9

Clinical features of OI?

Multiple fractures of bone

Blue sclera

Hearing loss

10

What is osteopetrosis?

Inherited defect of bone resorption...thick, heavy bone that fractures easily

11

Which type of cells are poorly functioning in osteopetrosis?

Osteoclasts

12

Clinical features of osteopetrosis?

Bone fractures, bone marrow under production, extramedullary hematopoiesis, vision and hearing impairment, hydrocephalus, renal tubular acidosis

13

Why is there renal tubular acidosis in osteopetrosis?

because it is associated with a CA II mutation (which decreases reabsorption of bicarb and results in a metabolic acidosis)

14

What is the defect in rickets or osteomalacia?

Defective mineralization of osteoid

15

Which cells produce osteoid?

osteoblasts

16

What is the main cause of rickets or osteomalacia?

low vit D--> low Ca and PO4

17

What are the 3 main actions of vitamin D?

Increase intestinal absorption of Ca and PO4

Increase renal reabsorption of Ca and PO4

Increase bone resporption of Ca and PO4

18

What are the lab findings in osteomalacia?

(Ca, PO4, PTH, alkaline phosphate)

decreased Ca

decreased PO4

increased PTH

increased AP

19

When does someone have their peak bone mass?

30 yo

20

What is osteoporosis?

reduction in trabecular bone mass leading to porous bones with an increased risk of fracture

21

What is Paget disease of the Bone?

Imbalance between osteoblast and osteoclast function

THICK, SCLEROTIC BONE that fractures easily

22

What are the 3 clinical stages of paget disease of the bone?

osteoclastic

Mixed osteoblastic- osteclastic

osteoblastic

23

What is the histology pattern in Paget disease on biopsy?

Mosaic pattern of lamellar bone

24

What are the clinical features of Paget disease?

Bone pain, increasing hat size, hearing loss, lion-like facies, isolated elevated alkaline phosphatase

25

What is the treatment for pagets?

Calcitonin and bisphosphonates

26

What is osteomyelitis?

Infection of marrow and bone

27

What is the main organism that causes osteomyelitis?

S. aureus

28

Osteomeylitis in sexually active young adults?

N. gornorrhoea

29

Osteomyelitis in sickle cell disease?

Salmonella

30

Osteomyelitis in diabetic or IV drug users?

Pseudomonas

31

Osteomyelitis in dog/ cat bites?

Pasturella

32

Name a benign tumor of bone associated with facial bones and Gardener Syndrome?

Osteoma

33

Name a benign tumor of osteoblasts that are surrounded by reactive bone in the cortex of long bones with paint hat resolves with aspirin

Osteoid osteoma

34

Benign tumor of osteoblasts, surrounded by reactive bone that arises in vertebrae and is not responsive to aspirin?

Osteoblastoma

35

What is a tumor of bone with an overlying cartilage cap?

Osteochondroma

36

What osteosarcoma?

malignant proliferation of osteoblasts

37

Risk factors of osteosarcoma?

retinoblastoma, paget disease, radiation exposure

38

Where in the bone does osteosarcoma usually form?

metaphysis of long bones ( in the knee region usually)

39

Imaging for osteosarcoma reveals...

destructive mas with a sunburst appearance

Codman triangle

40

What is a tumor comprised of multinucleated giant cells and stroma cells that arise in epiphysis of long bones?

giant cell tumor

41

Appearance of giant cell tumors on imaging?

soap bubble

42

What is a malignant prolifeation of porly differentiated cells derved from neuroectoderm that is in the diaphysis of long bones

Ewing Sarcoma

43

What age is Ewing sarcoma usually seen in?

<15 yo

44

Which cancer has a t(11;22) translocation?

Ewing sarcoma

45

Appearance of Ewing sarcoma on xray?

"onion skin"

46

What is a benign tumor of cartilage that arises in the medullary of small bones of HANDS and FEET?

Chondroma

47

What is a malignant cartilage forming tumor that arise in the medulla of pelvis or central skeleton?

Chondrosarcoma

48

What is the most common tumor in bone/ cartilage?

Metastatic tumors....you see osteolytic lesions

49

What is a benign tumor or adipose tissue?

lipoma

50

What is a malignant tumor of adipose tissue?

Liposarcoma

51

Lipoblast is the characteristic cell of...

liposarcoma

52

What is a benign tumor of skeletal muscle?

Rhabdomyoma

53

What is a malignant tumor of skeletal muscle?

rhabdomyoscaroma

54

Rhabdoblast is the characteristic cell of...

rhabdomyosarcoma

55

Which tumors are desmin (+)?

Rhabdomyosarcoma