Overview
When remodeling is disrupted structure of live bone, which has the ability to recover. Thus, there is the formation of "weak bones" - twisted, stretched, prone to fractures and cracks.
Paget's disease is the second in the list of diseases of bone structures after osteoporosis. In most cases, the pathology observed in the elderly and most often affects people who live in Western Europe. Young people prone to this ailment more rarely, but there were cases when people have been able to 40 years to identify Paget's disease.
Any bone tissues in a human skeletal structure may be exposed to Paget's disease. But most of all recorded cases of lesions in the pelvic bones, the tibia and femur, as well as the spine, skull and humerus.
Abnormal process can be subject to several bone structures (poliossalnaya form), which unlike asymptomatic monoossalnoy more pronounced characteristic features.
Causes
About the causes of Paget's disease is little known. In most cases, doctors are inclined to believe that
the nature of the disease - viral, Wherein sluggish. A person can be a carrier of the virus for many years without knowing it, and only under the influence of symptoms in the full force of trigger factors. Measles and respiratory syncytial virus - two of the most frequently suspected infections that provoke this disease.Special attention is paid to the factor of heredity, illness often suffer from several members of one family. In view of this, when establishing the patient diagnosis "Paget's disease", all his close relatives, especially those living together, it is recommended regularly conduct research on blood biochemical control of the level of alkaline phosphatase, and X-rays to check for changes in bone tissues.
symptoms
Paget's disease symptomatology is diverse. This is due to the volume of bone lesions, focal location, stage of the disease and the presence of complications. In practice there are three stages of the disease.
dilution stage
Identify at an early stage of the disease is very difficult. Symptoms may even occur after several years of the date of possible infection. Distribution of lesions occurs slowly enough - can at one millimeter per year, and may not be distributed on the bone. In this process rarely involved joints, except the sacroiliac joints (they can be completely transformed into a structureless newly formed bone). It recorded as cases when Paget's disease moved directly to the spinal column of the body on its coupling discs.
Diagnose Paget's disease at an early stage in primary patients from the same family is possible only by X-ray, which is made for the study of an entirely different disease or by determining the reasons for the increased levels of alkaline phosphatase in body.
step seal
Asymptomatic ends during the emergence of pain, wearing a permanent nature. Complaints of patients straight to indicate it is on bone pain. Begin to show remarkable degenerative changes in skeletal structure:
- arcuate position of the lower limbs;
- expressed his torso bent with a clear shortening;
- appearance "monkey" gait;
- increase in the size of the cranium (the only symptom that allows to diagnose pathology quickly).
Among systemic signs characteristic of Paget's disease, heart failure can be identified. The appearance of this symptom is due to the large ejection systolic blood that the heart pumps in one minute work of the body. This process occurs under the influence of an altered vascularization in tissues.
stable stage
Chronic course of Paget's disease in addition to the core symptoms, which is expressed deformability by secondary processes in different parts of the skeleton, and acute painful crises, often manifest disturbances in the work of other bodies:
- From the nervous system (with the defeat of the spine and skull) there is hearing loss, increased intracranial pressure, hydrocephalus.
- When the bone tissue grows, there is squeezing root spinal canal narrows vertebral arteries that can cause ischemic processes.
- When vertebra protrusion toward the back is formed medullary canal myelopathy (Spinal cord).
- In the formation of foci of Paget's disease in sacral region observed cauda equina syndrome (compression compression of nerve endings).
- Bones become brittle patient prone to frequent fractures (called "banana") fractures, the formation of slot formations.
- Against the background of the disease can develop sarcoma, and pathology of proliferation of bone tissue structure of tumors of different etiology.
Diagnostics
To establish the diagnosis with Paget's disease is only possible with the help of X-ray examination. The pictures all the characteristics will be expressed quite clearly:
- scimitar-shaped or O-shaped curvature of the long bones;
- the central body is tubular bone extended around the perimeter or in part;
- thickened epiphyses (epididymis).
Treatment
Symptomatic therapy - is one of the main treatment for Paget's disease. Medications that are used for therapy, this group of bisphosphonates, calcitoninsPlikamitsin.
Their action is aimed at:
- reduction of disease manifestations;
- eliminating redness tissue over bone structures;
- slowing down proliferation rate of bone;
- stop remodeling processes.
The action of bisphosphonates is selective. They have a lasting effect on bone tissue and are able to maintain a stable effect. Their method is via intravenous injection at a dose calculated individually, based on the history of the disease and the level of biochemical parameters.
The disadvantages of these drugs include poor absorbability groups - 10% of the dose received. In addition, many foods and drinks adversely affect the action of the drug activity. At their reception can not eat foods high in iron and calciumAs well as dairy.
The drugs calcitonin similar to bisphosphonates. However, at first there is no effect of accumulation, so their purpose is very limited evidence. Basically it is a tendency to frequent fractures, with the threat of neurological disorders, severe pain - where the necessary short-term, but the immediate effect.
However, not all cases of this disease require treatment, indications are:
- poliossalny type of disease;
- the need to address the pain that arose during the course of the disease Paget, not when complications arise;
- poor prognosis propagation pathology;
- extensive damage bone structures;
- Warning multiple fractures.
Forecast
For small changes in bone structure, as well as the absence of pain expressed deformability processes and threats from the nervous system, the decision to expectant management. In the case of Paget's disease signs of activity assigned drug therapy, the results of which are favorable forecasts.
When you have any complications such as hearing loss, obvious degenerative changes in the bones, nerve compression, stop the process and restore lost functions impossible.
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