The Osteosclerosis describes a hardening of the bones due to different causes. This leads to an excessive increase in bone substance. However, the stability of the bones is impaired.
What is osteosclerosis?
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In the Osteosclerosis it is not a single disease. The term only describes the changes in the bones towards hardening and increasing bone mass. Despite the considerable bone density, the bone architecture is not stable. Frequent bone fractures occur and, depending on the underlying disease, further ossification.
There are both genetic causes of osteosclerosis and secondary bone hardening due to an underlying disease. In most cases, osteosclerosis is generalized. That is, the entire skeleton is affected by the disease process. But there are also cases with local osteosclerosis. A genetic defect can almost always be assumed to be the cause, especially in the primary form of hardening of the bones.
This form of the disease is very rare. Secondary osteosclerosis is more common and occurs especially with renal insufficiency. But there is also a form with increased intake of fluorides through food. This form also belongs to secondary osteosclerosis. Localized forms of osteosclerosis can result from malignant or benign bone tumors or from Paget's disease.
causes
There are several causes for developing osteosclerosis. Hardening of the bone is part of the outward appearance of the symptom, with the bone mass constantly increasing but the stability of the bone decreasing. The hardening of the bone may take place on the basis of the increase in the inorganic material, the organic component, or both components. In some cases, complex and uneven remodeling processes occur.
In other cases, only bone formation takes place without remodeling processes. In hereditary osteopetrosis (marble bone disease), for example, the bone is only built up without any breakdown processes occurring. Usually the precursors of bone cells consist of osteoblasts and osteoclasts. The osteoblasts are responsible for building bones, while the osteoclasts are supposed to break down excess bone material.
A constant remodeling process has to take place within the bones in order to continuously remodel the bones. But this is not the case with osteopetrosis. Another hereditary osteosclerosis is Engelmann syndrome. This leads to irregular bone growth with increasing bone hardening and loss of stability of the bones.
In melorheostosis, the bone metabolism is undisturbed, but irregular growth in some areas, especially on the extremities, leads to thickening of the bones. It looks like the bones are flowing. This disease is also caused by a genetic defect. Secondary osteosclerosis often develops in the context of renal insufficiency.
The condition known as renal osteodystrophy is a very complex disorder of bone growth. Renal insufficiency causes the vitamin D hormone metabolism to be disturbed. Calcium and phosphate are no longer retained and excreted to a greater extent. The resulting hypocalcemia ensures a higher concentration of the parathyroid hormone, which in turn leads to demineralization of the bone. To compensate for the demineralization, connective tissue-like bone material is increasingly formed. The bone mass increases. At the same time, however, there is a loss of stability.
Symptoms, ailments & signs
The course forms of the individual diseases are different. However, a common symptom is a constant increase in bone mass and at the same time a decrease in the stability of the bones. The accompanying symptoms depend on the underlying disease. Osteopetrosis is characterized, for example, by increased fragility of bones, enlarged liver and spleen, immune deficiency, cramps and damage to the cranial nerves.
There are also diseases that, in addition to osteosclerosis, are characterized by multiple dysplasias such as the genetic Lenz-Majewski syndrome. Secondary renal osteodystrophy is characterized by bone and joint pain and swelling. The bone fragility is increased. At the same time, muscle atrophy also occurs. External calcium deposits can be found on the joints in the area of the elbows, shoulder joints, knees, toes or finger joints.
These are deposited again on the edge of the bones through intensified calcium dissolution processes. This causes pain that severely restricts mobility. Calcium deposits can also form in the arteries, which can lead to arterial occlusive disease with all its consequences.
Diagnosis & course of disease
The diagnosis of osteosclerosis is heavily aligned with the underlying disease or disorder. If severe kidney disease is evident, the overall character of the disease is examined diagnostically. If a hereditary disease is suspected, human genetic tests can be carried out. For this, however, an intensive anamnesis of the family medical history is necessary beforehand. If there is local osteosclerosis, the tumor should also be examined.
Complications
Due to osteosclerosis, patients suffer from reduced bone stability.Even minor injuries often result in fractures or other injuries to the bones. For this reason, patients with osteosclerosis must definitely avoid accidents and bumps as the healing of the bones is also impaired.
Furthermore, it is not uncommon for those affected to have a significantly weakened immune system and also to cramps in the muscles. The liver and spleen are significantly enlarged so that pain can occur in these regions. Furthermore, the weakened immune system leads to infections and inflammation more often. Without treatment, brain damage can occur, which is usually irreversible and should definitely be avoided.
Furthermore, there are discomforts in the joints and an arterial blockage can occur. Usually a causal treatment of the osteosclerosis takes place. There are no special compilations, but a tumor may have to be removed. The patients also need chemotherapy, which usually has side effects.
When should you go to the doctor?
If there is pain in the bone area or problems moving the hands and feet, a doctor should be consulted. The symptoms indicate osteosclerosis, which must be clarified quickly in order to avoid an increase in symptoms and long-term consequences. Affected persons should consult a doctor if the symptoms mentioned do not recede by themselves or increase in intensity within a short time. Medical advice is required at the latest when the typical joint noises are added.
The doctor can quickly determine the osteosclerosis with the help of imaging tests and an anamnesis and, if necessary, initiate treatment directly or refer the patient to a specialist. People who have already had a bone infection or an inflammation of the bone belong to the risk groups. You should confer with the responsible doctor if the symptoms described occur and do not go away on their own. Close medical control is also necessary after bone injuries, contact with toxic substances and bone cancer. In addition to the family doctor, the orthopedist or a specialist in internal medicine can be consulted. In cooperation with nutritionists and genetic counseling centers, the cause of the osteosclerosis can be determined. Physiotherapists, sports physicians and other specialists are involved in the treatment during therapy.
Therapy & Treatment
The therapy of an existing osteosclerosis depends on the cause. The hereditary diseases cannot be treated causally. Only symptomatic treatments are possible here. This includes constant monitoring and treatment of any fractures that may have occurred. Surgical measures are of course necessary for a diagnosed tumor.
If the tumor is malignant, chemotherapy and radiation therapy follow. Renal osteodystrophy can only be treated as part of the overall therapy for kidney disease. If it turns out that fluorosis is present, it is sufficient to discontinue the high fluoride intake.
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➔ Medicines for painOutlook & forecast
The prognosis of osteosclerosis is based on the stage at which the disease is recognized and treated and what form of osteosclerosis it is. If the condition is left untreated, the ossification can intensify and cause various health problems in the course of life. First, the resilience of the bones is reduced, which means that the patient has to accept various restrictions. There is no prospect of a full recovery. Existing hardening of the bone tissue can only be reduced by complex procedures. The orthopedic specialist makes the prognosis. Depending on the form and severity of the disease, he may involve other medical professionals.
Since the osteosclerosis develops in attacks and usually the cause cannot be treated, the prognosis must be regularly adjusted to the current state of health of the patient. Thanks to modern medicine, the prospect of a symptom-free life is relatively high. Life expectancy is usually not restricted by osteosclerosis. However, there is an increased risk of obesity, cardiovascular problems and falls. In the secondary form of osteosclerosis, there are also serious underlying diseases such as cancer or kidney disease, which can reduce quality of life and life expectancy.
prevention
A general recommendation for the prevention of osteosclerosis cannot be given. The causes are very diverse and only rarely depend on the way of life. A normal fluoride intake should be maintained only to prevent fluorosis.
Aftercare
In most cases of osteosclerosis, those affected have very few and usually only limited direct follow-up measures available. For this reason, those affected with this disease should consult a doctor very early to prevent further complications and complaints. It cannot heal on its own, so those affected should always consult a doctor.
Most of those affected are usually reliant on an operation that can remove the tumor. The earlier this procedure takes place, the better the further course is usually. After such an operation, the person concerned should rest and take care of himself in any case, avoiding exertion or stressful physical activities.
Most patients also depend on family support and help during treatment. Loving and intensive conversations also have a positive effect on the further course of osteosclerosis and thus also prevent psychological upsets or depression. In some cases, osteosclerosis also reduces the life expectancy of those affected.
You can do that yourself
People diagnosed with osteosclerosis can take some steps to promote recovery and make everyday life with the condition easier.
First of all, however, it is important to adhere to the doctor's specifications regarding diet and physical activity. Patients should work with a nutritionist on a diet and follow it consistently. The bone disease cannot be cured in this way, but an individually adapted diet reduces the pain and increases the well-being of those affected. The same applies to sport and a regular daily routine with enough sleep and little stress. Any restrictions on movement must be compensated for using aids such as walking aids or prostheses. Patients should consult a specialist at an early stage and take the necessary steps. In addition, constant monitoring and treatment of any fractures is necessary in any case.
If the osteosclerosis is caused by a malignant tumor, chemotherapy or radiation therapy is indicated. This can be supported by those affected by rest and physiotherapy. If fluorosis is the cause, it is sufficient to stop the food or medication that triggered it and to go easy on the body for a few weeks.
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