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Paget's disease is a bone disease in which the process of forming bone tissue is disturbed. As a consequence, the bones become weak, and thus - very susceptible to distortions and fractures. What are the causes and symptoms of Paget's disease of the bone? What is the treatment?

Paget's diseaseis a disease ofbone , the essence of which is the disruption of the functioning of osteoclasts and osteoblasts - two cells that make up the bone. Osteoclasts are osteoclasts that work to break down old bone tissue. Then, in its place, new bone tissue is formed, produced by bone-forming cells - osteoblasts. The balance between the activity of these two cells is necessary to maintain the proper bone structure.

In the course of Paget's disease, osteoclasts become overactive, and hence, excessive bone destruction. Consequently, in order to even out the resulting losses, the activity of osteoclasts and excessive bone reconstruction is increased. However, then bones with a chaotic structure are formed, much weaker, and thus - very prone to fractures and deformations (the so-called Paget bones).

The lesions can be localized to any bone, but the most common bones are in the pelvis, spine, skull and femurs. Moreover, they do not cover the entire bone and the entire skeleton, but usually a fragment of it.

Paget's disease of the bone - causes and risk factors

The causes of Paget's disease are not fully known. It is supposed to be:

  • genetically determined - there are families where the disease was inherited in an autosomal dominant manner; At least 4 gene mutations predisposing to the development of this disease have been identified;
  • caused by viruses;

Age is the most important risk factor - the later it is, the greater the likelihood of disease occurrence. Research shows that in people over 85 years of age, the incidence of the disease is almost 5 times higher than in the group of people under 60 years of age. Gender is the second risk factor - men suffer more often. In addition, it is known that Paget's disease of the bone is particularly common in northern European countries.

Paget's disease of the bone - symptoms

  • pain in the bone affected by the disease process;
  • warming up of the affected area (because blood flow is increased in the affected area);

Nearly 70 percent cases, the disease is asymptomatic, which makes it difficult to make a proper diagnosis

  • bone deformities, e.g. thickening of the bones of the skull;
  • bone fractures, most often in the long bones (usually the femur, tibia, humerus, radial, ulna). These fractures may be asymptomatic;
  • joint pains related to their degeneration (pain usually affects the joints in the vicinity of the bones affected by the disease);

If the skull bone is affected, the so-called external carotid blood-stealing syndrome. Then headaches, visual disturbances and hearing impairment appear. In extreme cases, an ischemic stroke may occur.

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Paget's disease of the bone - complications

Paget's disease is a serious condition that can lead to deafness (as a result of damage to the auditory nerve), secondary hyperparathyroidism, and even bone cancer (e.g. osteosarcoma), or heart failure or stroke (usually in patients with previous current heart disease or atherosclerosis). Some patients may struggle with kidney stones.

Paget's disease of the bone - diagnosis

If Paget's disease is suspected, an X-ray of the long bones, joints, spine and skull is performed. In addition, bone scintigraphy and blood tests are helpful (then an increase in serum alkaline phosphatase - an enzyme produced by osteoblasts) is found.

If the test results are inconclusive, the doctor may decide to perform a bone biopsy for histopathological examination.

It is worth knowing that lymphoma and primary bone cancer may have a similar course as Paget's disease. In addition, the doctor should rule out neoplastic metastases.

Paget's disease of the bone - treatment

In the treatment of Paget's disease, bisphosphonates are used to reduce the excessive activity of osteoclast cells, and thus - prevent the further development of the disease.

At the same time, the patient should supplement with calcium and vitamin D. In addition, painkillers are administered.

In some cases, surgery may be necessary - when the affected bone is fractured (then it is necessary to surgically fix it) or when the treatment of joint degeneration does not bring the expected effect (then the joints - hip or knee are replaced).

A person struggling with Paget's disease is usually under the care of several doctors - an endocrinologist, rheumatologist, orthopedist, neurologist and otolaryngologist.

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