Abnormal Development And Bone Pathology

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Achondroplasia?

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mutation of fibroblast growth factor receptor 3

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What happens with the mutation of FGFR3?

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results in the reduction of the proliferation of chondrocytes on growth plate

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Morphology of Achondroplasia?

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zones of proliferation and hypertrophy are narrowed and disorganized and contain clusters of large chondrocytes instead of well-formed columns

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Osteogenesis Imperfecta?

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deficiency in synthesis of type 1 collagen

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deficiency in synthesis of type 1 collagen?

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incorporation of defective alpha2 chains that cause instability and degradation of the triple helix

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Osteogenesis Imperfecta Type II

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fatal in utero or during the perinatal period...fetus is still within the womb

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Findings for Osteogeness Imperfecta?

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extreme skeletal fragility..blue sclerae caused by a decrease in collagen content, exposing the coridal veins...hearing loss related to both a sensorineural deficit and impeded conduction owing to abnormalities in the bones of the middle and inner ear...dental imperfections due to a deficiency of dentin...

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Mucopolysaccharidoses

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caused by deficiencies in the enzymes

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Deficient enzymes in Mucopolysaccharidoses

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that degrade dermatan sulfate, heparan sulfate, and keratan sulfate

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Mucopolysaccharidoses

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abnormalities in hyaline cartilage

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Signs of Mucopolysaccharidoses

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patients are frequently of short stature and have chest wall abnormalities and malformed bones

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Hunter's syndrome

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associated with increased tissue stores and excretion of dermatan sulfate and heparan sulfate

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Senile Osteoporosis

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continuous loss of bone at both the trabecular and cortical layers, which become thinner by internal resorption

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Steroid-induced Osteoporosis

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catabolic effect of corticosteroids may affect trabecular bone, producing a decrease in bone formation

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Effects of Steroids on Caclium absorption

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steroids also decrease intestinal absorption and renal resorption of calcium, so that less calcium reaches the bone

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Post-menopausal osteoporosis

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most common after the fifth decade of life...decreased remodeling and decreased amount of bone deposited by osteoblasts...b/c lack of estrogen

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Immobilization osteoporosis

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confinement of bed results in a loss of 30% of initial bone volume...lack of exercise seems to be the cause...

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anorexia nervosa and osteoporosis

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anorexia nervosa leads to osteoporosis in women b/c of loss of menstrual period...
reduction in bone mass with normal bone mineralization

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Osteopetrosis

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Marble bone disease, Albers-Schonberg disease

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Osteopetrosis

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osteoclast dysfunction...stonelike quality of the bones; however, the bones are abnormally brittle and fracture like a piece of chalk

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Osteopetrosis xray findings

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Erlenmeyer flask

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Deficiency seen in Osteopetrosis

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carbonic anhydrase II deficiency

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Morphology of Osteopetrosis

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osteoclasts lack the usual ruffled borders and show decreased functioning

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Clinical features of Osteopetrosis

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fractures, anemia, hydrocephaly are often seen...

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Paget Disease (Osteitis Deformans)

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haphazard arrangement of cement lines

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Paget Disease (Osteitis Deformans)

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can often lead to bell's palsy if impinging on the CN VII

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Paget Disease (Osteitis Deformans)

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predilection for the skull, pelvis, tibia, and femur

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Paget Disease (Osteitis Deformans) Lab findings

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serum alkaline phosphatase level is markedly elevated/increased levels of urinary hydroxyproline...

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X ray findings ofr Pagets

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bone lysis and reformation are characteristic...

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Pathology of Pagets

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"mosaic" pattern of osteoclasts...so termed b/c of cement like material forms narrow boundaries

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Cancer associated with Pagets Disease

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often associated with Osteosarcoma

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Treatment for Pagets

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administration of calcitonin, which decreases the resorption

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Signs and Symptoms of Pagets

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bone pain, fractures, and deformities...deafness when skull is affected...height distortion due to vertebral compression

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Osteonecrosis (AvascularNecrosis)Differential Diagnosis

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1) mechanical vascular interruption (fracture)
2) corticosteroids
3)thrombosis and embolism (nitrogen bubbles in dysbarism)4) vessel injury (vasculitis, radiation therapy)5) increased intraosseous pressure with vascular compression
6) venous hypertension

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Osteonecrosis course

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chronic pain that is initially associated only with activity but then becomes progressively more constant until finally it is present at rest

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MCC bilateral segmental osteonecrosis or avascular necrosis (AVN) of the femoral head

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systemic steroid therapy

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Osteomyelitis

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inflammation of the medullary and cortical portions of bone, including the periosteum

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Osteomyelitis bones affected, child vs adult

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children, the long bones are most often affected; in adults, the vertebrae

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Osteomyelitis Pathogenesis

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1) Most commonly, the hematogenous spread of bacteria from a distant focus of sepsis
2) Invasion of bone from adjacent septic arthritis or soft tissue abscesses
3) Penetrating trauma
4) Complication of fractures
5) Complications of surgery

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Osteomyelitis increased risk

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pts with sickle cell disease, hemodialysis patients, patients with bone or joint prostheses, and intravenous drug abusers

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Pyogenic Osteomyelitis caused by

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Staphylococcus aureus is responsible for 80% to 90% of the cases