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PATHOLOGY..
Paget’s Disease
Maryam SharifianMSc student In Isfahan University Of medical Sciences
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INTRODUCTION
Paget’s disease is a chronic condition of bone
characterized by disorder of the normal bone
remodeling process.
Characterized by excessive breakdown of bone
tissue followed by abnormal bone formation.
Also known as Osteitis Deformans is a bone
disease unknown cause.
Affecting men twice as freaquently as women.
Named after the England Surgeons Sir James
Paget.
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DISEASE CLASSIFICATION
INFLAMMATORY
DISEASE
•Multifocal chronic
skeletal disease due to
Chronic paramyxoviral
Virus infection.
•As a result, the bone that
is formed is abnormal.
CONGENTIAL
•Certain of genes may
Been associated with
Paget’s disease, including
The Sequestrosome 1
Gene On Chromosome 5.
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ETIOLOGY
The cause of Paget’s disease is unknown
Disease may be caused by a virus: Paramyxo virus
Resulting in localized abnormal osteoclastic activity
typical of paget’s disease.
Also, a person’s genes may predispose them to
developing paget’s disease.
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Paget’s disease affects skeletal older bone of
adults
There is also an extremely rare form of paget’s
disease in children, referred to as Juvenile Paget’s
disease
In two of every three cases of Paget's disease,
more than one bone is affected
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Pathogenesis
Hyper vascular/
Osteolytic phase
Initial phase of
Disorder involves
Bone resorption by
osteoclasts
Intermediate
phase
Osteolytic activity
+
Osteoblastic activity
Exhaustive
(burn out)
stage
Abnormal matrix
Persists but cellular
Activity is nearly
Absent.Paget’s disease
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MANIFESTATIONS - SYMPTOMS
Paget’s disease is more commonly asymptomatic, but
may exhibit a variety of non-specific symptoms due to
increased bone turnover.
The bones that are move commonly affected include the
pelvis, spine, skull, and the long bone.
Individuals may experience any or a combination of the
following symptoms;
Bone pain
fractures
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symptoms …
Pain
Stiffness
Difficulty moving your affected joints
Nerve damage
Loss of balance
bone fractures
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Other symptoms …
Hearing loss
Headaches
Osteoarthritis
visual abnormalities
Cardiac abnormalities
Skeletal Deformities
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SIGNIFICANT LAB TESTS
Blood chemistry (blood patient who have paget’s
disease) results indicate very high alkaline
phosphates levels with normal serum calcium and
phosphorus.
There is no known cure for this disease.
Most cases are mild and asymptomatic no
treatment is necessary in symptomatic cases
medications.
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IMAGING CONSIDERATION
X-ray of the skull, spine, pelvis and long bone.
CT scan
MRI (Magnetic Resonance Imaging)
Radionuclide bone scan or RNI (Radionuclide
Imaging).
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RADIOLOGICAL APPEARANCE
Radionuclide bone scans readily detect Paget’s
disease even in its very early stage.
Radio graphically of the affected bone typically
demonstrate cortical thickening, with a coarse,
thickened trabecular pattern.
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Radiograph of the
fibula and tibia of the
patient in figure
demonstrating the
effect of advanced
proliferative Paget’s
disease on the fibula
and tibia.
Paget’s disease….
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Paget’s disease on pelvis…
Paget’s disease
involving the left hemi
pelvis and right, there
is severe osteoarthritis
of the left hip but a
relatively normal joint
space in the right hip.
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Paget’s disease….
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Treatment …
Not everyone with Paget's disease will
require treatment.
If treatment is needed, medication is most
often used.
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Maryam sharifian