Description of Paget’s disease
Paget’s disease is a bone disease that primarily affects the axial skeleton bones, it is mostly the skull, spine, pelvis, and also a large long bones elsewhere in the body. The disease is manifestedby changing the shape, sometimes deformities of bone, which often increase their diameter and volume (literally zduřují) and lose their strength. Therefore also often they cause fracture.Because the disease most often begins to manifest on the bones of the skull begins to swell and head circumference affected suddenly one day will be a little of their hat. Thus probably originated historic designation of the disease as “a disease of the small hat”.
Bone is not just a kind of scaffolding for the body, which once grew and now it causes no change.Vice versa. Although the bone mineral content through a very hard tissue, but it is very metabolically active (it occurs constantly in the metabolism), participates in maintaining constant internal environment and holds many important functions in the body. Constantly it also leads to redevelopment. And it just got us here are interested in. Special bone cells called osteoclastsdissolve bone tissue constantly and that, following the breakdown once again finishing touches and thus changes its microscopic structure. Such an ongoing bone remodeling is called “bone remodeling.” In Paget’s disease, however, is the rebuilding of excessive and disordered,osteoclast activity is increased. This leads to an excessive bone resorption that is displaced fiduciary bone mass, the nature of connective tissue containing a large amount of blood vessels.Response to increased degradation is also increased formation of bone, which is also messy and chaotic, bone and so changes its normal shape, the volume increases and its mechanical stability and strength decreases. The new bone is primitive, chaotic structure.
Simultaneously with these changes increases the blood flow to the bone, both because of the high demands of large bone activity on nutrient supply and by reason of abnormal couplings between arteries and veins, which produces in the bone (ie. Arteriovenous shunts). This can lead to high stress blood circulation, develops so-called. “Hyperkinetic circulation,” the heart is burdened excessively and can lead to its failure.
Swelling and deformity of bones, however, are not the main problems that afflict patients suffering from this disease. The disease is accompanied by severe pain that could handicap patients in normal life.
The bones of the skull normally associated holes through which passes many important nerves and sensory pathways in the brain and back. Now imagine that changing the shape and volume of the cranial bones is also important to close these “tunnels” through the skull, and it certainly does not bode well. Indeed, there is oppression nerves and other important structures, which has serious consequences. Hearing disorders arise, the oppression of cranial nerves arise neurological complications, the most common being dizziness (dizziness, feelings of instability) is threatened and vision. Furthermore, it can be numbness in the face, impaired mobility and weakness of the facial muscles. Oppression oculomotor nerve arise motility disorders eyes and double vision.
Spinal bone changes can cause the so-called. “Lumboischialgický syndrome”, which is a disease of the lumbar spine, which is manifested by pain and limited mobility in this area. Current radicular irritation (the nerves extending from the spinal cord leading to other parts of the body), so-called. “Radicular syndrome” causes radiating pain in the lower limb and can even lead to paralysis of muscles and limbs fault sensitivity.
There is also a spontaneous fracture of affected bones, bone break even at the minimum load due to the loss of its mechanical resistance.
The disease can in some cases lead to permanent disability.
Fortunately, despite these severe symptoms, the disease does not show steps in all cases.Subjective complaints (which he sees himself sick) occur in only about 15% of all cases of the disease. Other cases are asymptomatic (symptoms are not reflected) so often even undiagnosed and unaware of them or the patients concerned.
Risk factors for Paget’s disease
Since the cause of the disease is still unknown, we can not state with certainty the risk factors that are responsible for causing disease.
What is certain is that genetic predisposition (congenital conditions) play a role as a risk factor so we can mark the previous occurrence of the disease among blood relatives and ancestors. Then, the probability of disease is much higher.
Causes of Paget’s disease
The causes of the disease are not yet known.
It is considered a cause of viral diseases. Bone cells responsible for the rebuilding of the affected bone because they contain particles which resemble viruses of the Paramyxoviridae family, and thus is considered a paramyxovirus such as a trigger condition.
Undoubtedly, however, the genetic influence. If anyone in your family suffers or has in the past suffered from the disease, new outbreaks of the disease is much more likely.
The possible influence of genetic and external environment suggests different geographic prevalence of Paget’s disease.
Prevention of Paget’s disease
There is no known effective prevention.
Treatment of Paget’s disease
Paget’s disease is a chronic disease requiring long-term, often even lifelong treatment. Since the initial failure at the level of osteoclasts (bone-degrading cells) used a substance that inhibits the activity or reduce their number. Historically, the first drug was used salmon calcitonin, which was produced artificially.
Gradually they began to use drugs called bisphosphonates (used to treat osteoporosis), in the form of oral (oral) and intravenous in large doses, which are used today. Recently, a new drug with excellent results called zolendronate (zolendronová acid). It has been the recent medical procedure using zolendronate in a short infusion once a year. Patients treated with this new procedure are the best results and also show a significantly better quality of life than previous drugs.
Another treatment is symptomatic, which treats the symptoms of the disease. Involves administration of analgesics (pain relieving drug), and optionally can also be performed surgical procedures.
Complications of Paget’s disease
Complications of the disease are fractures, decreased hearing deafness, dizziness conditional oppression cranial nerves, neurological complications and excessive load on the heart, which can lead to heart failure. It can also occur when spinal lumboischialgický syndrome (see above – description of the disease, symptoms), manifested as low back pain radiating into the lower extremity. In about 1-2% of cases may develop malignant bone cancer – osteosarcoma.
Other names: Disease small hat, Paget’s disease, osteitis deformans, osteodystrophia Deformans
