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Paget's disease- type 1

Overview

A chronic, slowly progressing bone disorder where the bone is destroyed rapidly and replaced by abnormal bone which is dense and fragile. Type 1 is caused by a mutation on chromosome 6p21.3.

Symptoms - Paget's disease- type 1

  • Bone pain
  • Joint pain
  • Back pain
  • Hip pain
  • Knee pain
  • Headache
  • Enlarged thighs
  • Bowed thighs
  • Enlarged lower legs
  • Bowed lower legs
  • Protruding forehead
  • Enlarged skull
  • Waddling gait
  • Muscle disturbance
  • Sensory disturbance
  • Hearing loss
  • Asymptomatic

Causes - Paget's disease- type 1

The disease occurs worldwide, but is more common in Europe, Australia, and New Zealand, where it’s seen in up to 5% of the elderly population. Although its exact cause is unknown, one theory holds that early viral infection causes a dormant skeletal infection that erupts many years later as Paget’s disease. Genetic factors are also suspected.

Prevention - Paget's disease- type 1

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Diagnosis - Paget's disease- type 1

Signs and symptoms of Paget's disease, type 1 may vary on an individual basis for each patient. Only your doctor can provide adequate diagnosis of any signs or symptoms and whether they are indeed Paget's disease, type 1 symptoms.

Prognosis - Paget's disease- type 1

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Treatment - Paget's disease- type 1

Primary treatment consists of drug therapy and includes one of the following:

  • Calcitonin (subcutaneously or intranasally) is used to retard bone resorption (which relieves bone lesions) and reduce levels of serum alkaline phosphate and urinary hydroxyproline secretion. Although calcitonin therapy requires long-term maintenance, improvement is noticeable after the first few weeks of treatment.
  • Bisphosphonates, such as etidronate, alendronate, pamidronate, tiludronate, and risedronate, produce rapid reduction in bone turnover and relieve pain. They also reduce serum alkaline phosphate and urinary hydroxyproline secretion. Therapy produces noticeable improvement after 1 to 3 months.
  • Plicamycin, a cytotoxic antibiotic, is used to decrease calcium, urinary hydroxyproline, and serum alkaline phosphatase. It produces remission of symptoms within 2 weeks and biochemical improvement in 1 to 2 months.
  • Plicamycin is used to control the disease and is reserved for severe cases with neurologic compromise and for those resistant to other therapies. However, it may destroy platelets or compromise renal function. Orthopedic surgery is used to correct specific deformities in severe cases, reduce or prevent pathologic fractures, correct secondary deformities, or relieve neurologic impairment. Joint replacement is difficult because bonding material (methyl methacrylate) doesn’t set properly on pagetic bone. Other treatment varies according to symptoms. Analgesics or nonsteroidal anti-inflammatory drugs may be given to control pain.

Resources - Paget's disease- type 1

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