metabolic syndromes Flashcards
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
clinical features of pagets
multiple areas of the skeleton
enlargement of skull, jaws, facial bones, deformity of spine and long bones
increased pathologic fracture
you see pagets in pts over what age
40
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
when do you see a cotton wool appearance on a radiograph
pagets
when do you see a decreased density of bone and hypercementosis on multiple teeth
pagets disease
when do you see osteoporosis circumscripta in a radiograph
pagets
howw do you verify pagets with lab values
increased serum alkaline phosphatase
normal calcium and phosphorus
increased urine
hydroxyproline levels
what can pagets cause
bone pain, deformity, and neurologic probs
tx for pagets
calcitonin and bisphosphonates (with increased incidence of osteosarcoma)
primary hyperparathyroidism
pth gland, hyperplasia, adenoma, carcimoma
= too much pth
secondary hyperparathyroidism
kidney disease, hypercalcemia
2 causes of hyperparathyroidism
primary (pth gland) and secondary (kidney disease)
hypercalcemia leads to
bone and joint pain renal calculi peptic ulcers cognitive impairment painful bones, renal stones, abdominal groans, psychotic moans
when do you see a ground glass appearance
fibrous dysplasia and hyperparathyroidism
unlike fibrous dysplasia, hyperparathyroidism affects
all of the bones with no expansion
radiographic features of hyperparathyroidism
ground glass
loss of trabeculation, lamina dura, cortical plates
brown tumors
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)
microscopic evidence of hyperpth
giant cell lesions identical to CGCG and cherubism
tx of prim pth-ism
excision of pth gland
tx of secondary pth-ism
renal dialysis or transplant
cause of osteogenesis imperfecta
genetics leading to defective cross linking of collagen and osteoblasts produce a defective osteoid
t/f congenital onset of osteogenesis imperfecta is more sever than later onset
true
state of bones in osteogenesis imperfecta
fragile as hell
skeletal features of osteogenesis imperfecta
multiple fractures, thin cortical plates, excessive callus formation, large skull with prominent frontal bones
orally, osteogenesis imperfecta looks like
dentinogenesis imperfecta (different genes) opalescent teeth
clinical features of osteogenesis imperfecta
blue sclerae
hearing loss
ligament laxity
class III malocclusion is increased in pts with
osteogenesis imperfecta
tx for osteogenesis imperfecta
none
what happens in osteopetrosis
osteoclasts are unable to resorb bone
t/f in osteopetrosis, there is a marked increase in bone density but there they have a lot of bone fratures
true
what can pts with infantile osteopetrosis also present with
cranial nerve compressions, normocytic anemia, hepatoslenomegaly, frequent fractures,
marrow failure
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
2 types of ectodermal dysplasia
anhidrotic and hidrotic
anhidrotic ectodermal dysplasia
partial or complete absence of eccrine sweat glands, sebaceous glands, and hair follicles
face abnormalities in ectodermal dysplasia
midface hypoplasia
skin and appendage abnormalities of ectodermal dysplasia
fine, sparse hair, scanty eyelashes and eyebrows, heat intolerance and fever
whats happening with the teeth in pts with ectodermal dysplasia
cone shaped and less teeth
oral manifestations of ectodermal dysplasia
hypodontial or anodontia hypoplasia of alv ridges decreased vdo protuberant lips cone shaped teeth unerupted teeth xerostomia
cherubism
genetic disease affecting osteoblasts and clasts
clinical findings of cherubism
appears less than 10
progressive, nontender swelling of jaws
mand always involved
round, cherubic face
radiographic findings in cherubism
BILATERAL radiolucent lesions
multilocular
multiple unerupted teeth
tx for cherubism
lesions stabilize at puberty
surgical recontouring
gardner sydrome
genetic disease with GI polyps and multiple osteomas
associated with cancer and colon/rectum carcinomas
2 syndromes with GI polyps
gardners and peutz jeghers
t/f by age 60, 100% of the intestinal polyps are malignant
true
tx with garners syndrome
osteomas and epidermoid cysts removed
prophylactic colectomy recommended (what we’re really worried about)
craniofacial dysostosis is also known as
crouzon disease (apert is also the same, but they have syndactyly - fingers and toes fuse together)
manefestation of crouzon disease
synostosis (early closures of the suture)
clinical features of crouzons
midface hypoplasia and exopthalamus
prominent nose
radiographic findings of crouzons
beaten metal appearance
tx of crouzons
craniectomy early in life to provide space for the brain
cleidocranial dysplasia
genetic disease with late closure of fontanels with wormian bones
brachycephalic skull
percent of cleidocranial dysplasia with out clavice
10%
whats happening with the shoulders of cleidocranial dysplasia pts
aplasia, hypoplasica or thinning of clavicles
what happening orally in cleidocranial dysplasia pts
high narrow palate, multiple, unerupted supernumerary teeth
treacher collins sydrome clinical findings
down sloping palpebral fissures
hypooplasia of mand and malar bones
coloboma
deformed pinna - conductive hearing loss
what syndromes that we know of have cafe au lait spots
neurofibromatosis, jaffe, and mccune albright
papillon-lefevre syndrome is also known as
juvenile periodontosis with palmar-plantar hyperkeratosis
what do you find clinically in pts with papillon leffevre syndrome
generalized aggressive periodontitis with hyperkeratotic palms and soles of feet
