Miami Children's Hospital
Local: 305-666-6511
Toll Free: 800-432-6837
My Kids Patient Portal
Search
Advanced Search

Pediatric Center
In this section

Ear Nose And Throat

Paget’s disease of the bone

  PRINT    
     Bookmark and Share

Definition

Images

Paget's disease is a disorder that involves abnormal bone destruction and regrowth, which results in deformity.

Alternative Names

Osteitis deformans

Causes, incidence, and risk factors

The cause of Paget's disease is unknown, although it might have to do with genes or a viral infection early in life.

The disease occurs worldwide, but is more common in Europe, Australia, and New Zealand.

In people with Paget's disease, there is an abnormal breakdown of bone tissue, followed by abnormal bone formation. The new bone is bigger, but weaker and filled with new blood vessels.

The disease may only be in one or two areas of the skeleton, or throughout the body. It often involves bones of the arms, collarbones, leg, pelvis, spine, and skull.

Symptoms

Most patients have no symptoms. Paget's disease is often diagnosed when an x-ray is done for another reason.

If they do occur, symptoms may include:

  • Bone pain, joint pain or stiffness, and neck pain (the pain may be severe and present most of the time)
  • Bowing of the legs and other visible deformities
  • Enlarged head and skull deformities
  • Fracture
  • Headache
  • Hearing loss  
  • Reduced height
  • Warm skin over the affected bone

Signs and tests

Tests that may indicate Paget's disease include:

  • Bone scan
  • Bone x-ray
  • Elevated markers of bone breakdown (for instance, N-telopeptide)
  • Elevated serum alkaline phosphatase

This disease may also affect the results of the following tests:

  • ALP (alkaline phosphatase) isoenzyme
  • Serum calcium

Treatment

Patients who may not need treatment include those who:

  • Only have abnormal blood tests
  • Have no symptoms and no evidence of active disease

Patients with Paget's disease who are commonly treated include patients who have:

  • Certain bones, such as weight-bearing bones, involved, or bony changes that are getting worse quickly (treatment can reduce the risk of fractures)
  • Deformities
  • Pain or other symptoms
  • Problems with the skull, to prevent hearing loss

Drug therapy helps prevent further bone breakdown. Currently, there are several classes of medications used to treat Paget's disease. These include:

  • Bisphosphonates -- These drugs are the first treatment, and they help increase bone density. They may be taken by mouth or given through a vein (intravenously) less often. 
  • Calcitonin -- This hormone is involved in bone metabolism. It may be given as a nasal spray (Miacalcin), or as an injection under the skin (Calcimar or Mithracin)

Acetaminophen (Tylenol) or nonsteroidal anti-inflammatory medications (NSAIDs) may also be given for pain. Orthopedic surgery may be needed to correct a deformity in severe cases.

Support Groups

For additional support and resources, see the Paget Foundation.

Expectations (prognosis)

Disease activity and symptoms can generally be controlled with current medications. A small percentage of patients may develop a cancer of the bone called osteosarcoma. Some patients will need joint replacement surgery.

Complications

  • Bone fractures
  • Deafness
  • Deformities
  • Heart failure
  • Paraplegia
  • Spinal stenosis

Calling your health care provider

Call for an appointment with your health care provider if you develop symptoms of Paget's disease.

References

Lorenzo JA, Canalis E, Raisz LG. Metabolic bone disease. In: Kronenberg HM, Melmed S, Polonsky KS, Larsen PR, eds. Williams Textbook of Endocrinology. 12th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 29.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is an independent audit to verify that A.D.A.M. follows rigorous standards of quality and accountability. A.D.A.M. is among the first to achieve this important distinction for online health information and services. Learn more about A.D.A.M.'s editorial policy, editorial proces and privacy policy. A.D.A.M. is also a founding member of Hi-Ethics and subscribes to the principles of the Health on the Net Foundation (www.hon.ch).
The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. ©1997- 2010 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.