Rheumatology II Flashcards
(48 cards)
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
What is the main difference between osteomalacia and rickets?
Rickets affects children whereas osteomalacia affects adults
What are some risk factors associated with Osteomalacia and Rickets? (6)
- Vitamin D deficiency
- Pigmented skin
- Calcium malabsorption
- Renal diseases
- Drugs
- Pregnancy and lactation
What are some clinical features of Osteomalacia and Rickets? (6)
- Weak muscles
- Bone pain
- Fractures
- Bone deformity and impaired growth in children
- Hypocalcemia, tetany and seizures
- Swellings at costochondral junctions
What is Rachitic rosary?
Swellings at costochondral junctions
How could you diagnose Osteomalacia and Rickets?
Looking for pseudo fractures through radiology