bone Flashcards
effect of corticosteroids on bone
direct
- reduction of osteoblast activity + lifespan
- suppression of replication of osteoblast precursors
- reduction in calcium absorption
indirect
- inhibition of gonadal + adrenal steroid production
which type of collagen is affected in osteogenesis imperfecta?
type 1
genetic mutation that affect maturation + organisation of type 1 collagen
osteogenesis imperfecta
brittle bones brone to fractures - due to mutations affecting matuaration + organisation of type 1 collagen
8 types depending on mutation - vary in severity, mild may present in adult
most autosomal dominant
osteogenesis imperfecta presentation
recurrent + inappropriate fractures
blue/grey sclera (whites of eyes)
dental problems deafness short stature triangle face hypermobility bone deformities - bowed legs, scoliosis
diagnosis of osteogenesis imperfecta
clinical
genetic testing can be done but not routine
xrays for fractures
management of osteogenesis imperfecta
no cure - fix + prevent further fractures
bisphosphonates - increase bone density
vit D supplementation
MDT - physio, occ, paeds, social workers
which bones are most affected in paget’s disease?
axial skeleton
pelxis
lumbar spine
skull
long bones
may be single site (monostotic) or multiple (polyostotic)
Paget’s disease
disorder of excessive bone turnover - overactivity of osteoblasts + osteoclats
uncoordinated, leads to patchy areas of sclerosis + lysis
increased risk of pathological fractures
Paget’s presentation
older patients
bone pain
bone deformity + fractures
hearing loss - bones of ear affected
Paget’s appearance on x-ray
bone enlargement + deformity - patchy
osteoporosis crcumscripta = well defined osteolytic lesions that appear less dense
cotton wool appearance of skull - patchy sclerosis + lysis
V-shaped defects in long bones
biochemistry in paget’s
raised alkaline phophatase - other LFTs normal
normal calcium
normal phosphate
Pagets investigations
X-ray - patchy, less dense bones
biochemistry - raised alkaline phosphatase
management of Paget’s
bisphosphonates
NSAIDs for pain
calcium + vit D - esp when on bisphosphonates
Paget’s complications
osteogenic sarcoma (osteosarcoma)
spinal stenosis + spinal cord compression
osteogenic sarcoma (osteosarcoma)
key complication of Paget’s
poor prognosis
increased focal pain, bone swelling, pathological fractures