Sie sind auf Seite 1von 37

Metabolic Bone

Disease

Outline
Introduction
Clinical Evaluation of Metabolic Bone

Disease
Rickets
Osteomalacia
Hyperparathyroid Bone Disease
Pagets Disease
Other Sclerosing Bone Disorders

Introduction

Calcium-Regulating
Hormones

Calcium-Regulating
Hormones
Parathyroid Hormone
stimulate resorption

Vitamin D
stimulate resorption

Calcitonin
inhibits bone resorption

Clinical Evaluation
Metabolic Bone Disease
Bone Densitometry
Biochemical Measurements
Markers of Bone Formation
Markers of Bone Resorption
Bone Biopsy
Skeletal Imaging

Bone Densitometry
Most widely procedure
Measuring bone mass
Reported in terms of T-scores / Z-scores
T-scores : SD from the young adult norm for

that instrument
Z-scores : SD from the expected value for
individuals of the same sex, age, and body
size
Different for men and women and for

racial and ethnic groups

Bone Densitometry
Dual-energy x-ray absorptiometry (DEXA)
Quantitative Computed Tomography
Peripheral Densitometry

Dual-Energy X-Ray
Absorptiometry
DEXA
bone mineral content (BMC)
bone mineral density (BMD)

The lumbar spine, the proximal femur, the

distal radius, and the whole body


Radiation exposure is minimal (<10 mrem)
Scanning time is short (5 to 20 minutes)
Variability of repeated readings is less than
1% for phantom standards; less than 2% for
lumbar spine, total body, and radius; and
less than 3% for proximal femur

Dual-Energy X-Ray
Absorptiometry
Disadvantages
Moderately expensive
Anteroposterior measurements of the

lumbar spine in older patients : aortic


calcifcation and osteoarthritic changes

Bone Densitometry
Quantitative Computed Tomography
Used to assess true bone density
Separate cancellous and cortical bone in the vertebral
body
Measure trabecular BMD in the presence of osteoarthritis
Radiation exposure(100 to 300 mrem) is larger than for
DEXA
Peripheral Densitometry
Radiography , x-ray absorptiometry, U/S ,CT
Predict fracture risk
rapid and portable
screening programs

Indications for bone


Densitometry

Markers of Bone Formation


Alkaline Phosphatase
Osteocalcin
Procollagen Peptides

Markers of Bone Resorption


Calcium
Hydroxyproline
Collagen Cross-Links
Other Assays
Tartrate-resistant acid phosphatase 5b
cathepsin K

Bone Biopsy
Transiliac bone biopsy
Direct information about cancellous bone volume
Trabecular plates (connectivity)and the function of
bone cells
Bone resorption is more diffcult to assess by bone
biopsy
Large needle with a 7- to 9-mm internal bore
Special stains : mast cells in mastocytosis aluminum
in renal osteodystrophy
Assessment of therapeutic responses in clinical
trials
unusual skeletal lesions or for young men or women
who have fragility fractures

Skeletal
Radiographs
CT
Bone scans
MRI

Imaging

Metabolic Bone Disease

Metabolic Bone Disease

RICKETS AND
OSTEOMALACIA

Disorders of the mineralization of newly

synthesized organic matrix


OSTEOMALACIA:Adults: involves only bone
RICKETS : children : growth plate and in
the mineralization of cartilage
Metabolic defects in the vitaminD hormone
system
Occurs before closure of the epiphyses

VitaminD

OSTEOMALACIA
Oncogenic Osteomalacia : fibrous dysplasia,

neurofibromatosis, osteobalstoma
,hemangioperiocytoma
Production phosphatonins : FGF23
Fibrous and mesenchymal tumors
Hypophosphatasia
rare autosomal recessive disorder

Drug-Induced Osteomalacia
High doses of sodium fuoride
Frist-generation bisphosphonates (e.g.etidronate)
Anticonvulsant : phenobabitol

RICKETS
Nutritional rickets
Vitamin D-resistant rickets
Vitamin D-dependent-type I
Vitamin D-dependent-type II
Renal osteodytrophy

Nutritional rickets
Inadequate vitamin D intake
Reduced UVB exposure
widening of the cartilaginous ends of the

long bones
Onset : 6 mo.-3 yr
Enlargement of cartilage at the growth
plate : rachitic rosary
genu valgus/varus , coxa vara , stress
fracture, kyphoscoliosis, loosers zone

kyphoscoliosis

Widening endplate

loosers zone

Pseudofracture of Lt. tibia

Nutritional rickets
Treatment
1,25-dihydroxy (calcitriol): 6-10 WK
Radiographic change improve in 2-4 WK

Vitamin D-resistant rickets


Familial hypophosphatemic rickets
x-link dominant /autosomal dominant
renal tubular acidosis
Albrights syndrome, Fanconis syndrome
Family history
Treatment : Vitamin D + Phosphate +/-

alkalinized urine

Vitamin D-dependent
Vitamin D-dependent-type I
25-hydroxy VitaminD -/->1,25-dihydroxy

VitaminD
Treatment : 1,25-dihydroxy (calcitriol)
Vitamin D-dependent-type II
Autosomal recessive
Rare
Difficalt to treat

Renal osteodytrophy

Renal osteodytrophy
Slipped capital femoral epiphysis
Salt and pepper appearance
Resorption of terminal tuft
Subperiosteal resorption
Rugger jersy spine
Brown tumor

Resorption of terminal tuft


Subperiosteal resorption

Rugger jersy spine

Salt and pepper appearance

Renal osteodytrophy
Treatment
Rx underlying disease : Dialysis / KT
1,25-dihydroxy (calcitriol)

LAB