Rheumatology II Flashcards
Name some inherited non-autoimmune bone disorders (3)
- Osteogenesis Imperfecta
- Cleidocranial Dysplasia
- Osteopetrosis
Name some acquired non-autoimmune bone disorders (6)
- Osteomalacia and Rickets
- Osteoporosis
- Osteomyelitis
- Paget’s disease of bone
- Osteoarthritis
- Fibrous dysplasia
What type of condition is osteogenesis imperfecta?
Autosomal dominant inherited condition
Osteogenesis imperfecta causes defective formation of which type of collagen?
Type 1 collagen
What are some clinical features of osteogenesis imperfecta? (3)
- Multiple fractures on minimal impact (less after puberty)
- Gross deformity and dwarfism due to bone distortion on healing
- Parietal bossing causing eversion of upper part of ear
What are some potential complications that can arise from osteogenesis imperfecta? (6)
- Cardiac complications
- Deafness
- Blue sclera
- Easy brushing
- Hernias
- Weak tendons/ligaments
How could you diagnose osteogenesis imperfecta? (5)
- Positive family history
- Clinical presentation
- Radiographs
- Skin biopsy
- DNA-based sequencing
How is osteogenesis imperfecta managed? (2)
- Supportive therapy to decrease fractures and deal with disability
- Bisphosphonates to prevent bone loss
What is the dental relevance of osteogenesis imperfecta? (5)
- Handle patients carefully due to bone fragility
- Not to be confused with physical abuse
- Minimal force, support jaws, ensure haemostasis
- Linked with dentinogenesis imperfecta
- Bisphosphonates used
What is Cleidocranial dysplasia?
Rare autosomal dominant trait on Chromosome 6 causing defect of membrane bone formation
What are some clinical features of Cleidocranial dysplasia? (4)
- Absent or defective clavicle
- Prognathic mandible due to maxillary hypoplasia
- Depressed nasal bridge
- Frontal, parietal and occipital bossing
How could you diagnose Cleidocranial dysplasia? (2)
- Clinical findings
- Radiographic imaging
What is the dental relevance of Cleidocranial dysplasia? (5)
- Facial abnormalities
- Deciduous teeth retention causing delayed permanent eruption
- Hyperdontia
- Twisted roots
- Dentigerous cyst
What is another name for Osteopetrosis?
Marble bone disease
What is Osteopetrosis?
What is it caused by?
- Rare genetic disorder characterised by excessive bone density
- Defective osteoclastic activity and remodelling
What are some clinical features of Osteopetrosis? (5)
- Bone pain and fractures
- Osteomyelitis
- Infections and anaemia
- Cranial neuropathies
- Epilepsy
What is the dental relevance of Osteopetrosis? (4)
- Frontal bossing
- Trigeminal/Facial neuropathies causes issue s
- Jaw fracture
- Delayed tooth eruption
What is Osteomalacia?
Failure of mineralization of replacement bone in normal bone turnover
What is rickets?
Inadequate mineralisation of bone framework