Question

Paget's disease -

Can you one of these and describe the symptoms, how it is diagnosed and treated?

Answer 1

Paget's disease

Paget's disease which isalso called osteitis deformans is a condition is a chronic bone disorder. It involves cellular remodeling and deformity of one or more bones.

It can affect any one or multiple bones of the body most commonly affected ones being pelvis, femur, tibia, skull and lumbar vertebrae. It does not spread from bone to bone.

Symptoms

Early cases of Pagets disease are often asymptomatic. Therefore most of the cases are diagnosed incidentally during the checkup for other diseases.

If at all there is symptoms, most common one is bone pain. There will be joint pain also.

Sometimes Pagets can be confused with arthritis. So diagnosis can be late.

It can also be presented as bone deformities.

If it affects skull bones, it will result in frontal bossing and headaches.

Patient may have hearing loss which can be due to narrowing of auditory foramen and compression of nerves in the inner ear.  

Rarely vision loss can occur due to compression of the nerves supplying the eye

Diagnosis

First clinical finding is an elevated alkaline phosphatase in the blood. So elevated alkaline phosphatase with normal aminotransferase, calcium and phosphate in aelderly patient can be paget's disease.

X-rays- Typical appearance of pagetic bone

Presence of markers of bone turnover in the urine: pyridinoline.

Hydroxyproline levels elevated in serum and urine

Bone scans can be taken to determine the extent and activity of the condition.

Treatment

In order to treat paget's there are some medications which are indicated in symptomatic patients and those with elevated levels of alkaline phosphatases and those with chance of bone fracture. Surgery is recommended for some complications.

1)Medication

Aspirin, other anti-inflammatory medications, pain medications are used for severe bone pain and joint pain.

Bone deformity may require supports like heel lifts or specialized footwear.

bisphosphonates orally or IV given

calcitonin (Miacalcin)

2) Surgery

It is to manage complications of Paget's disease.

• Fractures — Surgery allows fractures to heal
• Severe degenerative arthritis — joint replacement of the hips and knees may be useful.
• Bone deformity — Cutting and realignment of pagetic bone may be used in painful weight bearing joints.

3)Diet and exercise

They should have 1000-1500 mg of calcium

Adequate sun exposure

Atleast 400 units of vitamin D daily

Exercise regularly.