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Flashcards in metabolic syndromes Deck (60):
1

what happens to bone in pagets disease

bone broken down and replaced faster than normal, where the new bone is weaker and less organized than normal bone

2

clinical features of pagets

multiple areas of the skeleton
enlargement of skull, jaws, facial bones, deformity of spine and long bones
increased pathologic fracture

3

you see pagets in pts over what age

40

4

what are you concerned about in pagets denture pts

enlargement of jaws and widening of the alv ridges so dentures wont fit, and there are spaces bt teeth

5

when do you see a cotton wool appearance on a radiograph

pagets

6

when do you see a decreased density of bone and hypercementosis on multiple teeth

pagets disease

7

when do you see osteoporosis circumscripta in a radiograph

pagets

8

howw do you verify pagets with lab values

increased serum alkaline phosphatase
normal calcium and phosphorus
increased urine
hydroxyproline levels

9

what can pagets cause

bone pain, deformity, and neurologic probs

10

tx for pagets

calcitonin and bisphosphonates (with increased incidence of osteosarcoma)

11

primary hyperparathyroidism

pth gland, hyperplasia, adenoma, carcimoma
= too much pth

12

secondary hyperparathyroidism

kidney disease, hypercalcemia

13

2 causes of hyperparathyroidism

primary (pth gland) and secondary (kidney disease)

14

hypercalcemia leads to

bone and joint pain
renal calculi
peptic ulcers
cognitive impairment
painful bones, renal stones, abdominal groans, psychotic moans

15

when do you see a ground glass appearance

fibrous dysplasia and hyperparathyroidism

16

unlike fibrous dysplasia, hyperparathyroidism affects

all of the bones with no expansion

17

radiographic features of hyperparathyroidism

ground glass
loss of trabeculation, lamina dura, cortical plates
brown tumors

18

lab features of hyperparathyroidism

primary: increased calcium and pth
secondary: decreased or normal serum ca, increased pth (the gland is working fine, but the body cant absorb it)

19

microscopic evidence of hyperpth

giant cell lesions identical to CGCG and cherubism

20

tx of prim pth-ism

excision of pth gland

21

tx of secondary pth-ism

renal dialysis or transplant

22

cause of osteogenesis imperfecta

genetics leading to defective cross linking of collagen and osteoblasts produce a defective osteoid

23

t/f congenital onset of osteogenesis imperfecta is more sever than later onset

true

24

state of bones in osteogenesis imperfecta

fragile as hell

25

skeletal features of osteogenesis imperfecta

multiple fractures, thin cortical plates, excessive callus formation, large skull with prominent frontal bones

26

orally, osteogenesis imperfecta looks like

dentinogenesis imperfecta (different genes)
opalescent teeth

27

clinical features of osteogenesis imperfecta

blue sclerae
hearing loss
ligament laxity

28

class III malocclusion is increased in pts with

osteogenesis imperfecta

29

tx for osteogenesis imperfecta

none

30

what happens in osteopetrosis

osteoclasts are unable to resorb bone

31

t/f in osteopetrosis, there is a marked increase in bone density but there they have a lot of bone fratures

true

32

what can pts with infantile osteopetrosis also present with

cranial nerve compressions, normocytic anemia, hepatoslenomegaly, frequent fractures,
marrow failure

33

adult osteopetrosis is different than infantile how

marrow failure is rare, 40% asymptomatic, rare bone fractures with common nerve compression OR common bone fractures with rare nerve compressions

34

2 types of ectodermal dysplasia

anhidrotic and hidrotic

35

anhidrotic ectodermal dysplasia

partial or complete absence of eccrine sweat glands, sebaceous glands, and hair follicles

36

face abnormalities in ectodermal dysplasia

midface hypoplasia

37

skin and appendage abnormalities of ectodermal dysplasia

fine, sparse hair, scanty eyelashes and eyebrows, heat intolerance and fever

38

whats happening with the teeth in pts with ectodermal dysplasia

cone shaped and less teeth

39

oral manifestations of ectodermal dysplasia

hypodontial or anodontia
hypoplasia of alv ridges
decreased vdo
protuberant lips
cone shaped teeth
unerupted teeth
xerostomia

40

cherubism

genetic disease affecting osteoblasts and clasts

41

clinical findings of cherubism

appears less than 10
progressive, nontender swelling of jaws
mand always involved
round, cherubic face

42

radiographic findings in cherubism

BILATERAL radiolucent lesions
multilocular
multiple unerupted teeth

43

tx for cherubism

lesions stabilize at puberty
surgical recontouring

44

gardner sydrome

genetic disease with GI polyps and multiple osteomas
associated with cancer and colon/rectum carcinomas

45

2 syndromes with GI polyps

gardners and peutz jeghers

46

t/f by age 60, 100% of the intestinal polyps are malignant

true

47

tx with garners syndrome

osteomas and epidermoid cysts removed
prophylactic colectomy recommended (what we're really worried about)

48

craniofacial dysostosis is also known as

crouzon disease (apert is also the same, but they have syndactyly - fingers and toes fuse together)

49

manefestation of crouzon disease

synostosis (early closures of the suture)

50

clinical features of crouzons

midface hypoplasia and exopthalamus
prominent nose

51

radiographic findings of crouzons

beaten metal appearance

52

tx of crouzons

craniectomy early in life to provide space for the brain

53

cleidocranial dysplasia

genetic disease with late closure of fontanels with wormian bones
brachycephalic skull

54

percent of cleidocranial dysplasia with out clavice

10%

55

whats happening with the shoulders of cleidocranial dysplasia pts

aplasia, hypoplasica or thinning of clavicles

56

what happening orally in cleidocranial dysplasia pts

high narrow palate, multiple, unerupted supernumerary teeth

57

treacher collins sydrome clinical findings

down sloping palpebral fissures
hypooplasia of mand and malar bones
coloboma
deformed pinna - conductive hearing loss

58

what syndromes that we know of have cafe au lait spots

neurofibromatosis, jaffe, and mccune albright

59

papillon-lefevre syndrome is also known as

juvenile periodontosis with palmar-plantar hyperkeratosis

60

what do you find clinically in pts with papillon leffevre syndrome

generalized aggressive periodontitis with hyperkeratotic palms and soles of feet