Paget Disease

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PAGET BONE DISEASE

INTRODUCTION

Metabolic disease of the bone known as “deforming


osteocitis” or “Paget bone disease”, this pathology
involves an alteration of the bone remodeling the
process of destruction and regrowth of the bone, which
occurs at an accelerated rate, undermining the
structural integrity of the skeleton making the bone
weaker and predisposing to fractures and bone
malformations
Remodelling takes place by two cell:

Osteoblast: Osteoclasts:
Responsible for the bone Responsible for the
construction, through the demolition of the bone tissue
“deposition” process trough the “resorption”
process

The balance between the two processes is altered, the demolition of the
bone tissue occurs rapidly due to a notable resorption by the osteoclasts and
an excess of abnormal neoformation and an increase in connective tissue
and blood vessels follows.
LOCATION

Paget’s disease is localized and affects one or a few


bones such as tibia, femur, humerus, spine, unlike
osteoporosis which is a generalized bone disease
The risk of onset of the disease increases with age.
Men are more affected !
DRUGS AND CARE

There isn’t cure but some drugs can control the


disease and reduce pain , such as biphosphonates.
Early diagnosis and drug treatment are effective in
controlling the disease
AETIOLOGY

The causes is unknown but the etiological hypotheses are


two:

1. Predisposition provided by hereditary genetic factors


like the RANK (receptor activation of nuclear factor
KB) which activates the osteoclasts, and the
sequestosome gene, which encodes the p62 protein.

2. Infection by a paramyxovirus
SINGS AND SYMPTOMS

The most common is bone pain. Others are: high


temperature, bone deformity (as enlarged jaw),
weakness, hearing loss, headache, dizziness, spinal
stenosis
POSSIBLE MEDICAL CONDITIONS

Fractures, osteoarthritis, osteosarcoma, deafness,


cardiovascular diseases, cranial nerve paralysis,
paraplegia
DIAGNOSIS

It can be established using the following instrumental


investigations:

X-RAY of the skeletal segment concerned (first choice


investigation)

Bone scintigraphy, to determine the extent of the disease

CAT to monitor the course of the disease

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