Paget's disease (osteitis deformans, osteodystrophy) - a pathological condition in which disturbed normal synthesis or recovery of bone as a result of internal and external impacts.
Bone in Paget's disease become mosaic in structure, brittle and prone to fractures.
Overview
Bone - it is a dynamic system, in her stocking phosphates, calcium and magnesium, and if necessary, these elements are mobilized (washed to the needs of the body) of the bones of the skeleton.
Since the bones in the process of life are subjected to constant regeneration (cells are destroyed and replaced by new ones), Paget's disease violates the normal process of bone renewal.
As a result, the bones become defective, their structure is broken, so they are deformed, twisted, and cease to become brittle to fully perform its support functions.
Causes
Paget's disease occurs after 40-50 years, progressing as far as age. Up to 3-4% of the population of Europe and Russia are the initial manifestations of the disease. Men often get sick.
The exact cause remains unknown, nominated the following versions:
- Disturbances in calcium and phosphorus metabolism of the different states
- Effect of viral diseases, especially chronic carriers of the virus. Special role of measles virus
- Heredity. All members of the family of the sick recommended dynamic observation of blood examination at the level of a particular enzyme (alkaline phosphatase), and X-rays of bones
- Perhaps the combined impact of both factors. The result is a degenerative process - bone disorder and timely supply of its renewal, the bones become softened, thickened and can not withstand the usual loads.
Distinguished two forms of Paget's disease: monoossalnaya (affecting one of the bones) and poliossalnaya (affecting several bones).
There is no generalized lesions, that is, suffering only a few bones, this disease is different from most other similar diseases - chronic osteomyelitis, acromegaly, Metastases to bone.
Paget's disease is a condition predisposing to further the formation of bone sarcoma.
Changes in the bones
Paget's disease of bone change process is continuous, there is no connection of bones changes with the load.
Depending on the predominance of the processes of osteolysis (bone resorption) and bone formation (bone reconstruction) the disease is divided into three phases:
- Osteolytic or initial, there is resorption of bone sections to form a distinctive hollow pits
- Active with a combination of both processes, there is a simultaneous process for forming hollow holes and bone overgrowth. On the joints of old and new bone revealed the line "bonding." The bones at the same time have an uneven cellular structure
- Inactive in this stage is osteosclerosis - replacement of bone connective tissue.
- Bone changes characteristic - long bones are curved and spiral curve, surface roughness bone. Bone thicken, and their weight is reduced. If struck skull is its thickness of 3-5 cm, which leads to deformation of the face and head. When the lesion of the spine vertebrae are increased in volume or flattened, forming a curvature of the spinal column.
Symptoms of Paget's disease
Symptoms of Paget's disease will depend on the extent of damage and the type of the affected bone. First of all, there are
- bone lesions in areas close to the joint and joint involvement in the disease,
- bone pain continuous, dull or aching, they are worse after rest or at rest,
- stiffness in the joints and limited mobility in them,
- palpable thickened and irregular bone edges, fractures often occur from minor impacts,
- legs bent in the shape of the letter X or O, although there may be other options.
- back becomes stooped, concerned about pain in the back,
- changes the shape of the head and face,
- compressed nerve endings between the cervical vertebrae or other, with the typical picture of neuritis,
- may experience loss of vision and hearing loss due to problems with the bones of the skull.
Sometimes the process of osteolysis (bone destruction) goes into the tumor transformation.
Diagnostics
If you suspect that Paget's disease conducted laboratory testing blood levels opredelnie calcium, magnesium, phosphorus and especially alkaline phosphatase, accurately reflecting the degree of degenerative processes dice.
X-rays of affected bones is necessary to the establishment of the typical changes - cellularity, areas of bone resorption. It is conducted periodically in order to control the process.
appoint more bone scintigraphy - introduction of radio-isotopes that accumulate in the bones, and the study of pictures taken with the drug distribution level.
Treatment of Paget's disease
Treatment of Paget's disease are engaged in orthopedic doctors. They control the process of the disease and prevent its complications.
The mainstay of treatment are special drugs - bisphosphonates - ksidifon, ostak, skelid, osteotab and others. They can significantly slow down the process of remodeling (rebuilding) of bone, but the reception they will be long.
Paget's disease is not completely cured, but it slows down significantly during the many years. The course lasts about six months, repeated as necessary. Drugs have some side effects. Therefore, the dose and duration of application determined by the physician.
When pain symptoms are used NSAIDs - ibuprofen, diclofenac, acetaminophen, ketoprofen dosage determined by the physician. Needs calcium intake and vitamin D, as they are actively spent on the remodeling of bones.
In cases of permanent fractures may require prosthetic joints or the installation of special structures for maintaining bone. With the defeat of hearing is used hearing aids and prosthetics.
complications
The major complications of Paget's disease include:
- pathological fractures with the movement disorder,
- hearing loss with changes in the bones of the inner ear area,
- development of bone sarcomas at the site of the pathological process.
Therefore, you need timely treatment and observation at the orthopedist that will prevent or significantly slow down the formation of complication.
