Pedzhet's disease

Pedzhet's disease – the deforming disease of bones at which processes of remodeling and recovery of their fabric are broken. Synonyms of a disease are the deforming osteosis, the deforming osteopathy.

About Pedzhet case history

At the end of the 19th century James Pedzhet described several cases of diseases of bones of the inflammatory nature. As it appeared, to it similar cases met too, but were considered as casuistry. Changes of bones, characteristic of Pedzhet's disease, were found even at excavation in skeletons of ancient people.

Assumed Pedzhet that the chronic inflammatory reaction caused by viruses is the cornerstone of a disease. Years later this theory became one of leaders in the doctrine about Pedzhet's disease though scientists cannot still agree on a consensus about a disease origin.

At the microscopic level in a bone tissue at similar defeat the sites of a sclerosis which are replaced by osteoporosis, growth of blood vessels in the place of defeat are found. The lower extremities suffer and are deformed stronger as the affected bone is influenced also by weight loading of all body.

Now there are facts confirming inheritance of family predisposition to Pedzhet's disease. Men are ill more often than women, the age of patients at the time of detection of a disease is almost always more senior than 40 - 50 years, youthful forms meet seldom. The sick person is younger, the quicker develops and zlokachestvenny the disease progresses.

Most often Pedzhet's disease meets in Great Britain, and here in Japan and India any case is not registered.

Symptoms of a disease of Pedzhet

At this disease the bone tissue is surprised. Allocate forms of a disease of Pedzhet with damage of one or several bones. Initial manifestations are insignificant and are not specific. Pedzhet's disease affects bones:

• Lower extremities;
• Skulls;
• Shoulder girdle;
• Edges.

Bones become weak, pererastyagivatsya and bent. On degree of a caries during a disease allocate 3 stages.

The main symptoms of a disease of Pedzhet are:

• Disturbance of functions of the affected extremities, a pelvic and shoulder girdle, a backbone;
• The aching ostealgias;
• Bone deformations.

Pains begin to disturb the person on average for 5 years earlier, than it will be possible to find pathological process in bones by means of X-ray. The patient tests more expressed pain at defeat of pelvic bones, legs and a backbone.

At this disease the form of a brain part of a skull changes, it increases in sizes, legs get the X-or Au-shaped form. One of symptoms of a disease of Pedzhet is decrease in hearing. There is it because reorganization of a temporal bone causes a prelum of the acoustical nerves passing in it.

It is considered that for 1 year of a disease struck there is about 1 cm of a bone tissue.

Diagnosis of a disease of Pedzhet

Except the anamnesis and complaints of the patient the disease can be confirmed with objective methods.

Radiological symptoms of a disease of Pedzhet:

• Are surprised, generally neurocranium bones, vertebrae of chest department of a backbone, edges and extremities;
• The centers of osteoporosis (osteoporosis) alternate with the centers of consolidation of a bone;
• Thickening of bones of a skull twice;
• Outside table of the scull with indistinct outlines;
• The internal table of the scull is sharply condensed;
• Vertebrae look deformed, flattened, increase in a circle;
• Joint cracks of the lower extremities are narrowed.

Because on X-ray the area of defeat looks strongly changed, it is required to differentiate Pedzhet's disease with tumoral metastasises and a hemangioma. Stsintigrafiya of bones helps to establish the exact place of defeat.

From laboratory diagnostic methods there can be useful indicators testimonial of activation of mineral exchange – an alkaline phosphatase and osteocalcine, and also levels of calcium, phosphorus and magnesium.

Complications of a disease of Pedzhet

Because of non-regulated reorganization of a bone tissue the defeat centers at Pedzhet's disease can regenerate in malignant bone tumors. Most often against the background of a disease the fibrosarcoma, an osteosarcoma and a giant-cell tumor develop.

Such symptoms of a disease of Pedzhet as gradual strengthening of pains which become more long can suggest an idea of malignant regeneration and do not react to therapy by diphosphonates. Deformation of bones amplifies, in them the soft centers and pathological changes can appear.

Treatment of a disease of Pedzhet

Therapy shall take place under control of the traumatologist-orthopedist. As drug treatment of a disease of Pedzhet, with the purpose to influence mineral exchange in bones, apply means on the basis of thyrocalcitonin. The course of treatment these drugs on average lasts 28 – 30 days, within a year repetition its 2 – 3 times is required.

In the course of treatment by thyrocalcitonin control of indicators of mineral exchange is necessary (bone fraction of an alkaline phosphatase, calcium and phosphorus in blood and urine, urine hydroxyproline). At overdose of thyrocalcitonin there is a hypocalcemia (in blood calcium level decreases).

Drug ксидифон (group of biphosphonates) cannot be used at osteomalacy. It reduces calcium level in blood. The dose is selected individually, taking into account dynamics of process.

Fight against a pain syndrome of a disease of Pedzhet by means of non-steroidal anti-inflammatory drugs (for example, accepting paracetamol). Patients with such disease need enough vitamin D and calcium.

At fractures and arthritises the help of surgeons and traumatologists, endoprosthesis replacement can be necessary, at disturbance of ability to hear – special hearing aids.

The surgical help can become replacement of a joint (endoprosthesis replacement) necessary for elimination of fractures of bones or treatment of arthritis. At decrease in hearing use hearing aids.