metabolic syndromes

Question 1

what happens to bone in pagets disease

Answer 1

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

Question 2

clinical features of pagets

Answer 2

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

Question 3

you see pagets in pts over what age

Answer 3

40

Question 4

what are you concerned about in pagets denture pts

Answer 4

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

Question 5

when do you see a cotton wool appearance on a radiograph

Answer 5

pagets

Question 6

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

Answer 6

pagets disease

Question 7

when do you see osteoporosis circumscripta in a radiograph

Answer 7

pagets

Question 8

howw do you verify pagets with lab values

Answer 8

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

Question 9

what can pagets cause

Answer 9

bone pain, deformity, and neurologic probs

Question 10

tx for pagets

Answer 10

calcitonin and bisphosphonates (with increased incidence of osteosarcoma)

Question 11

primary hyperparathyroidism

Answer 11

pth gland, hyperplasia, adenoma, carcimoma

= too much pth

Question 12

secondary hyperparathyroidism

Answer 12

kidney disease, hypercalcemia

Question 13

2 causes of hyperparathyroidism

Answer 13

primary (pth gland) and secondary (kidney disease)

Question 14

hypercalcemia leads to

Answer 14

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

Question 15

when do you see a ground glass appearance

Answer 15

fibrous dysplasia and hyperparathyroidism

Question 16

unlike fibrous dysplasia, hyperparathyroidism affects

Answer 16

all of the bones with no expansion

Question 17

radiographic features of hyperparathyroidism

Answer 17

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

Question 18

lab features of hyperparathyroidism

Answer 18

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

Question 19

microscopic evidence of hyperpth

Answer 19

giant cell lesions identical to CGCG and cherubism

Question 20

tx of prim pth-ism

Answer 20

excision of pth gland

Question 21

tx of secondary pth-ism

Answer 21

renal dialysis or transplant

Question 22

cause of osteogenesis imperfecta

Answer 22

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

Question 23

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

Answer 23

true

Question 24

state of bones in osteogenesis imperfecta

Answer 24

fragile as hell

Question 25

skeletal features of osteogenesis imperfecta

Answer 25

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

Question 26

orally, osteogenesis imperfecta looks like

Answer 26

``` dentinogenesis imperfecta (different genes) opalescent teeth ```

Question 27

clinical features of osteogenesis imperfecta

Answer 27

blue sclerae hearing loss ligament laxity

Question 28

class III malocclusion is increased in pts with

Answer 28

osteogenesis imperfecta

Question 29

tx for osteogenesis imperfecta

Answer 29

none

Question 30

what happens in osteopetrosis

Answer 30

osteoclasts are unable to resorb bone

Question 31

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

Answer 31

true

Question 32

what can pts with infantile osteopetrosis also present with

Answer 32

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

Question 33

adult osteopetrosis is different than infantile how

Answer 33

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

Question 34

2 types of ectodermal dysplasia

Answer 34

anhidrotic and hidrotic

Question 35

anhidrotic ectodermal dysplasia

Answer 35

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

Question 36

face abnormalities in ectodermal dysplasia

Answer 36

midface hypoplasia

Question 37

skin and appendage abnormalities of ectodermal dysplasia

Answer 37

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

Question 38

whats happening with the teeth in pts with ectodermal dysplasia

Answer 38

cone shaped and less teeth

Question 39

oral manifestations of ectodermal dysplasia

Answer 39

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

Question 40

cherubism

Answer 40

genetic disease affecting osteoblasts and clasts

Question 41

clinical findings of cherubism

Answer 41

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

Question 42

radiographic findings in cherubism

Answer 42

BILATERAL radiolucent lesions multilocular multiple unerupted teeth

Question 43

tx for cherubism

Answer 43

lesions stabilize at puberty | surgical recontouring

Question 44

gardner sydrome

Answer 44

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

Question 45

2 syndromes with GI polyps

Answer 45

gardners and peutz jeghers

Question 46

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

Answer 46

true

Question 47

tx with garners syndrome

Answer 47

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

Question 48

craniofacial dysostosis is also known as

Answer 48

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

Question 49

manefestation of crouzon disease

Answer 49

synostosis (early closures of the suture)

Question 50

clinical features of crouzons

Answer 50

midface hypoplasia and exopthalamus | prominent nose

Question 51

radiographic findings of crouzons

Answer 51

beaten metal appearance

Question 52

tx of crouzons

Answer 52

craniectomy early in life to provide space for the brain

Question 53

cleidocranial dysplasia

Answer 53

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

Question 54

percent of cleidocranial dysplasia with out clavice

Answer 54

10%

Question 55

whats happening with the shoulders of cleidocranial dysplasia pts

Answer 55

aplasia, hypoplasica or thinning of clavicles

Question 56

what happening orally in cleidocranial dysplasia pts

Answer 56

high narrow palate, multiple, unerupted supernumerary teeth

Question 57

treacher collins sydrome clinical findings

Answer 57

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

Question 58

what syndromes that we know of have cafe au lait spots

Answer 58

neurofibromatosis, jaffe, and mccune albright

Question 59

papillon-lefevre syndrome is also known as

Answer 59

juvenile periodontosis with palmar-plantar hyperkeratosis

Question 60

what do you find clinically in pts with papillon leffevre syndrome

Answer 60

generalized aggressive periodontitis with hyperkeratotic palms and soles of feet