R/O - Pathology of bone disorders Flashcards
unmineralized bone is called:
osteoid
osteoblasts secrete what growth factors?
- IL-1
- RANKL (receptor activator of NFkB which combines with RANK receptor on osteoclasts
what cells live in howship lacunae?
osteoclasts
osteoclasts are derived from what lineage?
hematopoietic progenitor cells
what type of bone?
- normally present in fetal skeleton, growth plates, and processes where there is very rapid bone production
- almost always pathologic in adults
woven
what are the 4 types of lamellar bone?
- concentric
- interstitial
- inner circumferential
- outer circumferential
what are the 3 developmental disorders of bone pathology?
- achondroplasia
- osteogenesis imperfecta
- osteopetrosis
what is the pathology of achondroplasia?
- point mutation in gene encoding for FGFR3
- FGFR is persistently activated - inhibition of normal proliferation of chondrocytes at growth plates
what is the inheritance of achondroplasia?
AD
premature sealing of growth plates - diagnosis?
achondroplasia
what cell type is proliferating in the zone of proliferation?
chondrocytes
what is the pathology of osteogenesis imperfecta?
- abnormalities of type 1 collagen synthesis
- mutations in genes that code for a1 an a2 chains
what is the inheritance of osteogenesis imperfecta?
AD
what are the 4 main types of osteogenesis imperfecta?
- type I: increased fracture risk, not deforming
- type II: lethal, multiple fractures in utero
- type III / type IV: progressive deformity
what is the morphology seen in OI?
- decreased bone thickness because of sluggish periosteal bone formation
- reduced number of trabeculae
- abnormally thin trabeculae