metabolic bone disease osteolysis (i.e.—hyperparathyroid states) defective bone formation...
TRANSCRIPT
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Metabolic Bone Disease
Osteolysis(i.e.—hyperparathyroid states)
Defective Bone Formation
Inadequate mineralization of osteoid (RICKETS)
Defective osteoid production (aka Osteoporosis)
•Nutritional sec.•Renal sec.•Primary
•HAC•Osteogenesis imperfecta
•Renal sec.•Decr. Ca or P•VITAMIN D DEF.•Hepatic•anticonvulsant
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Brinker, M. R. (2000). Basic sciences. In M. D. Miller & M. R. Brinker (Eds.), Review of orthopaedics (pp. 1-40). Saunders, Philadelphia
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Brinker, M. R. (2000). Basic sciences. In M. D. Miller & M. R. Brinker (Eds.), Review of orthopaedics (pp. 1-40). Saunders, Philadelphia
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Vitamin D functionsVitamin D functions
Regulates calcium and phosphorus levels in the body (calcium homeostasis)
Promotes absorption of calcium and phosphorus from the intestine
Increases bone mineralization Increases reabsorption of calcium and
phosphorus by renal tubules Maintains healthy bones and teeth
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0 nM > 100 nM
Vitamin D and bone
RicketsSecondary
hyperparathyroidismImpaired intestinal calcium absorption
Reduced bone volume with normal mineralization
Osteopenia
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USUALLY HYPOVITAMINOSIS D --inadequate intake --disorders of vitamin D metabolism --renal failure (decreased synth. Of 1, 25-(OH)2-vitD—the most active) Decreased Calcium or phosphorus intake
(e.g.—inappropriate feeding of renal diets)
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Factors Effecting Vit. D Factors Effecting Vit. D StatusStatusFactors Effecting Vit. D Factors Effecting Vit. D StatusStatus
1)1) Geographic locationGeographic location
2)2) SeasonsSeasons
3)3) Skin pigmentationSkin pigmentation
4)4) Cultural practicesCultural practices
5)5) Living conditions/jobsLiving conditions/jobs
6)6) DietDiet
1)1) Geographic locationGeographic location
2)2) SeasonsSeasons
3)3) Skin pigmentationSkin pigmentation
4)4) Cultural practicesCultural practices
5)5) Living conditions/jobsLiving conditions/jobs
6)6) DietDiet
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•Dietary•Renal failure•Liver disease•Pancreas/small intestinal dz•Hypoparathyroidism
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METABOLIC CONTROLS of VIT. D ACTIONSMETABOLIC CONTROLS of VIT. D ACTIONS
25 (OH) D3
1, 25 (OH)1, 25 (OH)22 D3 D3
24, 25 (OH)2 D3KIDNEYKIDNEY ((--)) (+)(+)
BONEBONE OSTEOCLASTOSTEOCLAST activity
MATRIX BREAKDOWN
Ca++
OSTEOBLASTOSTEOBLAST differentiation
make more MATRIX
(+)(+)
(+)(+)
PTHPTH
(+)(+)
((--))(+)(+)
((--))
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•Etiology of Hypophosphatemia
•Internal redistribution•Re-feeding•Acute respiratory alkalosis•Hungry bone syndrome
•Decreased intestinal absorption•Inadequate intake (< 100 mg/day)•Chronic diarrhea, malabsorption•Vitamin D deficiency or resistance•Aluminum or magnesium ingestion
•Increased urinary excretion•Primary hyperparathyroidism•Secondary hyperparathyroidism•Proximal tubule dysfunction•Hereditary hypophosphatemic rickets•Onchogenic osteomalacia
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Clinical featuresClinical featuresRickets Soft bones Skeletal deformity
(bowed legs) Bone pain Increased tendency
of bone fractures Dental problems Muscle weakness Growth disturbance
Osteomalacia•Soft bones•Bone pain•Bone fractures•Compressed vertebrae•Muscle weakness
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Loosers zones
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Brinker, M. R. (2000). Basic sciences. In M. D. Miller & M. R. Brinker (Eds.), Review of orthopaedics (pp. 1-40). Saunders, Philadelphia
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Renal Renal osteodystroosteodystrophyphy
Osteopenia, subcortical resorption, lucent metaphyseal bands, widening of metaphysis
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"rugger jersey spine"
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Osteomalacia and RicketsOsteomalacia and Rickets
Diagnosis: Measuring serum levels of:
› 25-hydroxycholecalciferol› PTH› Calcium› Phosphate› Alkaline phosphatase
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Treatment principles for those with low vitamin D levels
Ergo- vs chole- calciferol
IM vs po administration
Daily vs intermittent dosing
Dose amount
Co-administration with Calcium
Cholecalciferol
Po
Intermittent
300,000 iu load
Separate
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Comparing vitamin D therapies
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Declarations
No pharmaceutical funding support for this talk
In last five years received honoraria, travel and subsistence expenses from: › Proctor and Gamble, Servier, Eli Lilly and
Novartis
Sunshine vitamin
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This 73 year old lady was referred from her GP to ENT with deafness.
They asked her to see the rheumatologist
Why?
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Paget’s Disease
Disease of bone remodeling
Accelerated bone resorption and formation
Disorganised mosaic pattern bone with increased vascularity and fibrosis
Cause unknown
› paramyxovirus, canine distemper
› Genetics- susceptibility loci More common in caucasian
M:F ratio 3:2 10% in over 70’s
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Paget’s Disease: clinical manifestations
Bone pain
Joint pain
Deformity
Spontaneous fractures
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Pagets Disease: complications
Fractures
Deafness
Nerve entrapment
Spinal stenosis
Cardiac failure
Osteogenic sarcoma
Hypercalcaemia (only if immobilized)
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Paget’s Disease: investigations
Raised serum alk phos
Urinary hydroxyproline, pyridinoline cross-links
Radiology
› cortical thickening
› osteolytic, osteosclerotic and mixed lesions
› osteoporosis circumscripta
› bone scan
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Normal
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Courtesy of Pierre Delmas, MD.
Early-Stage (Lytic) Paget’s Disease: Tibia
V-shaped “blade of grass” lesion characteristic of lytic phase of Paget’s disease
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Courtesy of Pierre Delmas, MD.
Advanced Paget’s Disease in the Tibia: Sclerotic and Lytic Lesions
Primarily sclerotic changes, with enlargement and thickening of long bones
Secondary osteolytic front
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1976
Paget’s Disease: Progression Over 15 Years in Untreated Patient
Courtesy of Pierre Delmas, MD.1991
Bowing
Cortical thickening
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Courtesy of Pierre Delmas, MD.
Early-Stage (Lytic) Paget’s Disease in the Skull: Known as “Osteoporosis Circumscripta”
Lytic border
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Advanced (Sclerotic) Paget’s Disease: “Cotton Wool” Skull
Lyticlesion
Diffuse sclerotic changes
Courtesy of Pierre Delmas, MD.
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Courtesy of Nuria Guañabens, MD.
Advanced Paget’s Disease in the Pelvis
Bony enlargement
Diffuse sclerotic changes
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Courtesy of Nuria Guañabens, MD.
Paget’s Disease in the Femur
Curved deformity of the femur Cortical
thickening
Accentuation of trabecular pattern
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Paget’s Disease: “Picture Frame” Vertebral Body
Courtesy of Pierre Delmas, MD.
Lytic lesion
Corticalthickening
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Complications of Paget’s Disease: Fissure Fracture in the Tibia
Fissurefracture
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Complications of Paget’s Disease: Complete (Chalk-Stick) Fracture in a Femur
Courtesy of Pierre Delmas, MD.
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Paget’s Treatment
Bisphosphonates› calcitonin
Indicated if › Complications› Pain› Deformity› AP 2-3X Upper limit› Skull disease