Paget's disease - deforming bone disease in which the infringed remodeling and restoration of tissue. Synonyms of the disease are deforming ostoz deformed osteopathy.
On the history of Paget's disease
At the end of the 19th century, James Paget described several cases of bone inflammatory nature. As it turned out, before it, too, encountered similar cases, but I think casuistry. Characteristic for Paget's disease of bone changes were found during excavations in even the skeletons of ancient people.
Paget suggested that the basis of the disease is a chronic inflammatory reaction caused by viruses. Over the years, the theory has become one of the leading doctrine of Paget's disease, although scientists still can not agree on a common opinion about the origin of the disease.
On a microscopic level in bone tissue at sites of these lesions are detected sclerosis alternating with osteoporosis, proliferation of blood vessels in the lesion. The lower limbs are affected more and deformed, because the bone disease affects the entire body weight load.
Now there is evidence inherit family history of Paget's disease. Men get sick more often than women, the age of patients at the time of detection of the disease is almost always over 40 - 50 years old, junior forms are rare. The younger the sick person, the fast-growing malignant disease progresses.
Paget's disease most often occurs in the UK, and here in Japan, and India is not a single case.
Symptoms of Paget's disease
When this disease affects the bone. There are forms of Paget's disease with lesions of one or more bones. Initial symptoms are minor and non-specific. Paget's disease affects the bones:
- The lower extremities;
- The shoulder belt;
The bones become weak and distorted pererastyagivayutsya. According to the degree of destruction of bone tissue during stage 3 of the disease isolated.
The main symptoms of Paget's disease are:
- Violation of the functions of the affected extremity, pelvic and shoulder girdle, the spine;
- Aching in the bones;
- Bone deformation.
The pains begin to disturb the person an average of 5 years before it will be possible to detect the pathological process in the bones with the help of X-rays. Patients experiencing a severe pain in the defeat of the pelvis, legs and spine.
In this disease brain changes shape of the skull, it increases in size, the feet become X- and O-shaped. One of the symptoms of Paget's disease is a hearing loss. This is because that the restructuring of the temporal bone causes compression of passing it the auditory nerves.
It is believed that one year infected disease is about 1 cm of bone tissue.
Diagnosis of Paget's disease
In addition to the patient's medical history and complaints disease can be confirmed by objective methods.
Radiographic signs of Paget's disease:
- It affects mainly the bones of the cranium, vertebrae of the spine, ribs and limbs;
- Outbreaks of osteoporosis (bone loss) alternate with areas of bone compression;
- Thickening of the skull bones in 2 times;
- The outer plate of the skull bones with fuzzy contours;
- The inner plate of the skull bones sharply sealed;
- Vertebrae look deformed, flattened, increasing in circumference;
- Narrows the joint spaces of the lower extremities.
Due to the fact that X-rays affected area looks much changes need to differentiate Paget's disease with tumor metastasis and hemangioma. Bone scintigraphy helps identify the exact location of the lesion.
From laboratory diagnostic methods may be useful indicators that show the revitalization of mineral metabolism - alkaline phosphatase and osteocalcin, and the levels of calcium, phosphorus and magnesium.
Complications of Paget's disease
Due to the irregular adjustment of bone lesions in the Paget's disease may degenerate into malignant bone tumors. Most often in the background of the disease develop fibrosarcoma, osteosarcoma and giant cell tumor.
Suggest a malignant degeneration may be symptoms of Paget's disease, a gradual strengthening of pains, which become longer and do not respond to therapy with diphosphonates. There is a growing deformation of bones, they may appear soft pockets and pathological fractures.
Treatment of Paget's disease
Therapy must necessarily take place under the supervision of an orthopedic traumatologist. As medical treatment of Paget's disease, to influence bone mineral metabolism, is used by means of calcitonin. The course of treatment with these drugs lasts on average 28 - 30 days within a year requires the repetition of his 2 - 3 times.
During treatment requires monitoring of calcitonin indicators of mineral metabolism (bone alkaline phosphatase, calcium and phosphorus in the blood and urine, urine hydroxyproline). With an overdose of calcitonin is hypocalcemia (decreased blood levels of calcium).
The drug ksidifon (group of bisphosphonates) can not be used in osteomalacia. It lowers the level of calcium in the blood. Dose picked individually, taking into account the dynamics of the process.
With the pain of Paget's disease fighting through non-steroidal anti-inflammatory drugs (for example, by taking paracetamol). Patients with this disease require a sufficient amount of vitamin D and calcium.
For fractures and arthritis may need help surgeons and trauma, joint replacement, in violation of the ability to hear - special hearing aids.
Surgical assistance may be necessary to remove bone fractures or treatment of arthritis joint replacement (endoprosthesis). When hearing loss use hearing aids.