· defect in type II collagen synthesis
· abnormal accumulation of layers in rER of chondrocytes
· defective processing of proteoglycans
· inborn error of metabolism
· accumulation of mucopolysaccharides chondroitin sulphate and heparitin sulphate in chondrocytes
· inborn error of metabolism
· accumulation of mucopolysaccharide keratosulphate in swollen chondrocytes (foam cells)
· thyroid deficiency
· abnormal mucopolysaccharide metabloism
· chondroitin sulphate accumulation in chondrocyte
· no specific abnormality
· abnormalities may affect
· chondrocyte proliferation
· matrix production
· germ plasm defect
· enchondral bone formation inhibited
· membranous bone formation normal
· defect in proliferative zone
· chondrocyte proliferation decreased
· columns poorly formed
· subsequent narrowing of hypertrophic zone
· scanty metaphyseal bone formation
· periosteal strip of fibrous tissue from perichondrium may seal growth plate
· leads to decreased longitudinal growth and short bones
· shaft diameter normal
· subperiosteal membranous bone formation normal
· metaphysis flared
· due to continued latitudinal growth
· decreases chondrocyte production in proliferative zone
· chondrogenesis slowed
· bone formation in metaphysis continues until metaphyseal seal foms
· resulting dense line called growth arrest line or Harris line
· affects proliferative zone primarily
· decreases chondrocyte proliferation
· does not affect latitudinal bone growth
· similar bones to achondroplasia
Sex hormones
· oestrogen excess depresses chondroblastic proliferation
· testosterone excess increases chondroblastic proliferation
· complex interaction as may cause premature plate closure
Glucocorticoid excess
· depresses chondroblastic proliferation
Growth hormone excess
· increases chondroblastic proliferation
· also stimulates membranous ossification
· causes gigantism
Growth hormone deficiency
· opposite effect
· produces proportionate dwarf
· cartilage may be immature hypertrophic cells
· may also originate from cambium layer
· calcifiable matrix formed
· matrix does not calcify
· primary spongiosa not formed
· hypertrophic zone greatly widened and viable
· no calcified cartilage bars
· primary spongiosa not formed
· metaphysis consists of large osteoid seams surrounding thin bony trabeculae
· tongues of hypertrophic cells extend into metaphysis
· inborn error of ALP metabolism
· matrix fails to calcify
· hypertrophic zone widened
· similar appearance to rickets
· can involve
· vascular invasion
· ossification
· remodelling
· abscess starts here
· may result in partial or total destruction of physis
· may result in stimulation of growth
· fracture usually through hypertrophic zone
Salter-Harris I & II
· blood supply to plate disrupted
· increase in width of hypertrophic zone
· with healing, vascular supply reestablished
· plate becomes normal
Salter-Harris III, IV & V
· growth stops if healing of epiphysis to metaphysis occurs
· two effects
1. defect in chondroitin sulphate production in proliferative zone
2. deficiency in collagen formation
· primarily affects metaphysis
· calcified cartilage accumulates at bone-cartilage junction
· bony trabeculae sparse and thin
· collagen does not mature beyond reticulin fibre stage
· is disease of osteoblasts
· primarily affects metaphysis
· formation of secondary spongiosa deficient
· internal remodelling deficient
· calcified cartilage bars of primary spongiosa not resorbed
· primitive bone laid down
· no medullary cavity produced