Paget's disease of bone

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Paget's disease of bone
Synonyms	Osteitis deformans
Pronounce	N/A
Specialty	N/A
Symptoms	Bone pain, deformities, fractures
Complications	Osteosarcoma, heart failure, arthritis
Onset	Usually after age 50
Duration	Long-term
Types	N/A
Causes	Unknown, possibly genetic and environmental factors
Risks	Family history, age, European descent
Diagnosis	X-ray, bone scan, blood test for alkaline phosphatase
Differential diagnosis	Osteoporosis, bone metastases, fibrous dysplasia
Prevention	N/A
Treatment	Bisphosphonates, calcitonin, pain management
Medication	N/A
Prognosis	Generally good with treatment
Frequency	1-2% of people over age 55
Deaths	N/A

Paget's disease is a disorder occurring in the middle-aged and elderly in which bone does not form properly, causing bone weakening, thickening, and deformity.

Paget's disease of Right Hip Bone

Diagnosis

Diagnosis is by X-rays and alkalinephosphatase blood test, and a bone scan.

Paget's disease

Pathophysiology

Paget's disease of the bone represents an imbalance of bone formation and resorption.

Paget's disease in vertebra on CT

Excess bone resorption

• It typically begins with excessive bone resorption followed by excessive bone formation. There is an exaggeration of osteoclastic bone resorption, initially producing a localized bone loss.
• The excessive resorption and formation culminates at the tissue level in an abnormal mosaic pattern of lamellar bone associated with extensive vascularity and increased fibrous tissue deposition in adjacent marrow spaces.
• Early in the disease process, osteolysis is accompanied by some level of repair.
• Paget's Disease can affect any bone in the body such as the spine, skull, pelvis, femora, and tibia are most commonly involved.
• Fractures as a result of this condition are common

Clinical presentation

• Paget's Disease may occur in only one bone when it is called monostotic or in multiple bones when it is called polyostotic Paget's Disease.
• The most common bones involved include spine, pelvis, skull, femur and tibia.
• It usually presents with pain
• Involvement of the skull and facial bones can result in leonine facies and sometimes loss of bone conduction hearing.
• The disorder can also lead to gross deformity, compression of roots or spinal cord, fracture of an involved bone, or alteration of joint structure and function leading to osteoarthritis
• The overlying skin over the involved long bones may be warm and hyperemic, possibly due to increased blood flow to the entire region.
• Paget's Disease affecting the spine may produce pain directly or as a result of nerve root irritation, compression or both.

Nerve compression

• Compression of nerve fibers may also occur as well as involvement at the base of the skull may lead to basilar invagination or platybasia with consequent compression of structures in the posterior fossa, spinal cord or, rarely, cerebellar tonsillar herniation leading to ataxia, weakness, or respiratory compromise.

Lack of strength in bone

• The x-ray appearance of Pagetic bone reflects the underlying process
• Pagetoid bone lacks the strength of normal bone and hence it deforms and fractures easily.

Risk of cancer

Patients with Paget's Disease may develop primary bone malignancies in the bone, including osteogenic carcinomas that can spread rapidly and have an extremely poor prognosis.

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