Idiopathic juvenile osteoporosis is the occurrence of bone loss in children and adolescents. The cause of the condition is unknown.
What is idiopathic juvenile osteoporosis?
Idiopathic juvenile osteoporosis (IJO) is a form of bone loss that presents in childhood and adolescence. It is also called Dent-Friedman syndrome because it was named after the medical doctors Dent and Friedman, who first described it in 1965. Another name for the bone disease is osteoporosis of childhood and adolescence. Osteoporosis is a progressive disease characterized by the loss of bone density. It is also possible to lack bone formation, which weakens the bones, making them much more susceptible to fractures. Even minor falls that would not normally result in serious damage can result in a fracture of the affected bone. As a rule, seniors are particularly affected by osteoporosis. In rare cases, however, bone loss can also be seen in children or adolescents. Doctors then speak of juvenile or idiopathic juvenile osteoporosis. On average, children between the ages of 8 and 14 develop this form of osteoporosis. In younger children, bone loss sometimes occurs during growth spurts.
Causes
In many cases, no specific cause can be found for the development of bone loss in juvenile osteoporosis. For this reason, the term idiopathic juvenile osteoporosis is used. In some cases, however, the bone loss is the result of a genetic disorder, which includes osteogenesis imperfecta, for example, or an underlying triggering disease. Then it is a secondary juvenile osteoporosis. Diseases that can trigger secondary osteoporosis in childhood and adolescence include juvenile arthritis, hyperthyroidism, hyperparathyroidism, diabetes mellitus, Cushing’s syndrome, kidney diseases, anorexia nervosa, homocystinuria, and malabsorption syndromes. Sometimes the use of certain drugs also promotes the development of juvenile osteoporosis. These include primarily corticosteroids, anticonvulsants for seizures, and immunosuppressants. However, it is not uncommon for the lifestyle of the affected children to play a role in the onset of bone loss. For example, they are often unusually inactive or suffer from vitamin D and calcium deficiencies.
Symptoms, complaints and signs
Idiopathic juvenile osteoporosis becomes noticeable mostly during prepuberty, between the ages of 8 and 12, with pain in the lower back, hips, and feet. Often, affected children have problems walking. The joints and lower spine are also affected. In addition, growth often stops, so that the child’s height decreases. As the disease progresses, general bone loss increases and so-called fish vertebrae form. It is not uncommon for fractures to occur in the vertebrae as well as compression fractures in the long tubular bones. Other features include a shortened rib cage and an abnormal curvature of the upper spine, which is also known as kyphosis.
Diagnosis and course of the disease
In order to be able to take timely therapeutic measures against idiopathic juvenile osteoporosis, early diagnosis is extremely important. Bones can then be better protected from fractures. The typical symptoms are an important indication of the presence of idiopathic juvenile osteoporosis. In addition, the physician takes X-rays of the skeleton and measures the bone density. The typical spinal changes can usually be quickly identified on the X-rays. Bone density measurement is used to determine the demineralization of the bones. From a histological point of view, loosening of the cortical bone, rarefaction of the trabeculae and a reduced amount of osteoid can be determined. Differential diagnosis also plays an important role. Thus, juvenile polyarthritis, juvenile idiopathic arthritis, rickets, osteogenesis imperfecta, or eating disorders must be differentiated from IJO. The course of idiopathic juvenile osteoporosis is usually positive. Thus, after the onset of puberty, there is usually a spontaneous improvement.In severe cases, however, there is sometimes a threat of permanent disability due to deformities of the ribs or curvature of the spine.
Complications
Bone loss occurs in this disease. This usually has a very negative effect on the patient’s daily life and quality of life. Juvenile osteoporosis causes severe pain in the feet and hips. However, this pain does not occur before the age of eight, which is why it is not possible to diagnose this disease at an early stage. Furthermore, the patient experiences walking difficulties and, if necessary, limited mobility. The spine and various joints can also be affected by the disease. It is also not uncommon for growth to be halted, resulting in short stature. The risk of bone fractures is also increased by juvenile osteoporosis. In most cases, the pain leads to restrictions in everyday life and not infrequently to depressive moods. The patient’s ability to cope with stress decreases enormously and the affected person appears tired and fatigued. In some cases, the disease can regress spontaneously, so that no special treatment is necessary. In general, treatment can only be carried out with the help of medication or physiotherapy, although a cure cannot be guaranteed. Life expectancy is not limited by the disease.
When should you go to the doctor?
If adolescents repeatedly complain of aching bones, increased attention is required. If the complaints persist unabated for several days or increase in intensity, a doctor is needed. Initially, the signs may be mistaken for growth symptoms. A visit to the doctor is necessary if the pain spreads throughout the body or the child exhibits particularly noticeable behavior. Discomfort in the back, hips or feet must be examined and treated as soon as it persists. If falls or accidents can be ruled out as the cause, medical examinations must be initiated. If there are restrictions in the range of movement, if the joints can no longer be loaded as usual or if the child’s physical performance decreases, a visit to the doctor must be made. In case of sleep disturbances, headaches, attention and concentration deficits or a general feeling of malaise, a doctor should be consulted. If visual changes in the skeletal system occur, a doctor should be informed immediately of the observations. There is a threat of further damage, which must be prevented in time. In case of mental problems, emotional conspicuousness and a refusal attitude of the child, a doctor is needed. If growth stops at a very early age between 8 and 12 years, it is advisable to have this development clarified by a doctor.
Treatment and therapy
To date, it has not been possible to develop a uniform form of treatment for idiopathic juvenile osteoporosis. Thus, therapy is determined by the physician based on certain factors. These include the extent of bone loss, the child’s age, general health, medical history, and how the child responds to certain treatments and medications. In some cases, no therapy is needed at all because the osteoporosis will regress. If treatment must be given, the focus is on protecting the bones and vertebrae from fractures. The most important therapeutic measures include physical training, physiotherapy and other supportive measures. The additional administration of vitamin D, calcium, calcitonin and fluoride is also considered promising. In persistent cases, bisphosphonates are also administered. These agents usually have a positive effect. A balanced, healthy diet for the child is also important. If there is an underlying disease that triggers secondary juvenile osteoporosis, it must be treated accordingly.
Outlook and prognosis
Idiopathic juvenile osteoporosis is a particularly insidious form of osteoporosis because it is often not even suspected because of the patient’s age. This means the patient may have been living with it for quite some time without it being diagnosed and treated. Since there is no known cause for the early onset of osteoporosis, its progression cannot be stopped, so symptoms and damage will occur. However, modern medicine can slow the progression once osteoporosis is recognized as such.As a result, the relatively young patient remains physically fit for a long time and, with a bit of luck, even almost symptom-free. At worst, however, osteoporosis causes lasting damage at an age when most people are still far from developing such degenerative diseases. The earlier such damage to the bones occurs, the more time it has to worsen later in life. This can also affect lifestyle, as a person who is still young has to be increasingly careful not to put too much strain on the bones, as they could otherwise break. Sports and exercise are much more difficult in this way. However, the less a person can exercise, the more they tend to lead a generally unhealthy lifestyle, which in turn can bring new problems.
Prevention
Because the causes of idiopathic juvenile osteoporosis are unknown, it is difficult to prevent bone loss. In general, it is recommended that the child maintain his or her body weight and get plenty of exercise. In addition, it should always be provided with sufficient calcium.
Follow-up
Idiopathic juvenile osteoporosis requires comprehensive preoperative and follow-up care. The early onset of damage to the skeleton increases the risk that the disease will worsen. This risk can be reduced with a healthy lifestyle. It is important to find the right amount of exercise to avoid putting too much strain on the bones. Those affected therefore have more gentle sports to choose from. They should not do without exercise, because otherwise further problems such as obesity can follow. In order to counteract bone loss, patients should ensure that they get enough exercise and maintain a reasonable body weight from an early age. A diet with sufficient nutrients and calcium provides the body with good support. Dairy products in particular contain a lot of calcium. Vitamin D improves the body’s absorption of calcium. Furthermore, nuts, seeds and green vegetables are recommended. Sports activities should have the lowest possible risk of injury. Among others, gentle gymnastics, swimming or dancing are suitable. However, team sports increase the risk of breaking bones, so soccer or basketball are not recommended. Physiotherapy can further stabilize the skeleton as a supporting apparatus. Adolescent patients should be careful in everyday life and not lift heavy loads.
What you can do yourself
Children and adolescents suffering from idiopathic juvenile osteoporosis should exercise a lot to strengthen bones and muscles. All sports that promote strength and endurance and are associated with a low risk of injury, such as jogging, swimming, or dancing, are suitable. Contact and team sports carry the risk of bone fracture and are therefore less recommended. Targeted gymnastic exercises under the guidance of a physiotherapist additionally stabilize the body’s supporting apparatus. Sick children and adolescents should not lift or carry anything heavy in order to avoid permanent damage to the spine. A balanced diet with a high proportion of calcium-rich foods is important: Dairy products are very good sources of calcium, but green vegetables such as kale, fennel and broccoli, as well as seeds and nuts, also contain a lot of calcium. In order for calcium to be absorbed by the body in sufficient quantities, vitamin D is necessary. The body can produce this itself under the influence of sunlight: Affected children and adolescents should therefore expose themselves to sunlight for at least half an hour every day in order to promote the production of vitamin D. Oxalic acid and phosphates inhibit the absorption of calcium: the consumption of spinach, rhubarb, red beets, meat, sausages, cocoa and Coca Cola is therefore only advisable in small quantities.
