Bone Disorders (3) Flashcards
(41 cards)
What are the four inherited bone disorders?
- Osteogenesis imperfecta
- OSteopetrosis
- Cleidocranial dysplasia
- Cherubism
What are the six acquired bone disorders?
- Osteitis deformans (Paget disease)
- Fibro-Osseous dysplasia
- Cemento-Osseous dysplasia
- OSteoporotic bone marrow defect
- Idiopathic osteosclerosis
- Simple bone cyst
__________ leads to abnormal bone mineralization and low bone density (osteopenia) in osteogenesis imperfecta.
Defective collagen
T/F: 90% of osteogenesis imperfecta is autosomal dominant.
True
What are some clinical features of OI?
Fragile bones, blue sclera, hearing loss
CL III occlusion, triangular faces
T/F: Osteogenesis imperfecta and dentinogenesis imperfecta are the same disease.
False
Similar features but different mutations
____________ involves failure in osteoclast function which leads to increased bone density.
Osteopetrosis
Why are patients with osteopetrosis more susceptible to infections and osteomyelitis?
Loss of hematopoietic precursor cells (pancytopenia)
T/F: Patients with the autosomal dominant form of osteopetrosis typically die before 20 y.o.
False
Autosomal recessive is worse prognosis
Can live long with autosomal dominant
What are the clinical features of Cleidocranial dysplasia?
Prominent forehead, long neck, narrow and drooping shoulders
T/F: Patients with cleidocranial dysplasia are often missing several teeth.
False
Teeth are their but they do not erupt properly
What is the prognosis for a patient with cleidocranial dysplasia?
Good;
Live long life, often need extractions and orthodontics
___________ is a disorder often seen in children that causes painless bilateral expansion of the jaws, especially the mandible.
Cherubism
T/F: Surgical intervention is the best treatment for cherubism.
False
Can accelerate growth of lesions
T/F: Osteitis deformans (Paget disease) will cause weakening and thinning of bones.
False
Weakening and thickening
What are some clinical features of Paget’s disease?
40% have pain
“Simian” stance due to bowing of femurs
Elevated alkaline phosphatase
What is the radiographically appearance of bone in patients with osteitis deformans?
Cotton wool appearance
Hypercementosis
What is the treatment for osteitis deformans?
No treatment if asymptomatic
Bisphosphonates
What is the prognosis of osteitis deformans?
Not life-threatening
Watch for osteosarcoma
What are some dental complications with patients with osteitis deformans?
Difficult to extract teeth, hemorrhage, and poor wound healing
Poor prognosis for implants
May need several dentures remade
T/F: Fibrous dysplasia will present earlier in life than paget disease.
True
T/F: The majority of fibrous dysplasia cases are polyostotic.
False
Monostotic (only involve one bone)
What are the radiographic features of fibrous dysplasia?
Early stages radiolucent/mottled
Become opaque as they grow
“Ground glass” pattern
What are the two types of polyostotic fibrous dysplasia?
Jaffe - two or more bones affected and cafe-au-lait spots with jagged border
McCune-Albright - 2 or more bones affected, cafe-au-lait spots and endocrine disturbances
