The Latin technical term pubertas praecox translates as “premature puberty”. This refers to the onset of puberty before the age of eight (in girls), and in boys, the onset of puberty before the age of nine.
What is pubertas praecox?
Pubertas praecox is diagnosed when the development of secondary sexual characteristics, such as a breast set or pubic hair, begins long before the usual age. Increased length growth can also be observed in affected children. Since the reason for premature puberty may be a tumor on the liver, brain, or thyroid gland, the growth of which affects hormone regulation, or premature puberty may cause short stature in the affected person, premature puberty is considered pathological and should be treated.
Causes
In order to treat the condition, the pediatrician first investigates the causes of early pubertal development. For example, a tumor may stimulate an overproduction of sex hormones by simulating the endocrine gland so that puberty is initiated early. It is also conceivable that the regulatory hormones of the sex hormones are not secreted in sufficient numbers. This refers to androgenital syndrome, a metabolic disorder that must be treated with regular hormone administration. Premature puberty may also be genetic; in this case, treatment is still appropriate because a premature growth spurt puts affected individuals at risk of hardly growing at all later on, resulting in a very small body size.
Symptoms, complaints and signs
If children show the signs of physical and mental development that are characteristic of puberty at a very early age, they may have pubertas praecox. The cut-off date is considered to be the attainment of the eighth year of age in girls and the ninth year of age in boys – from this age onwards, one no longer speaks of premature onset of puberty. The progression of early puberty can be very rapid, but it can also be slow. Blemished skin or even acne, greasy hair, vaginal discharge or the onset of menstruation before the age of eight should alarm parents. A change in demeanor can also give clues to early onset puberty. Premature puberty occurs about five times more often in girls than in boys.
Diagnosis and course of the disease
As a rule, the pediatrician is the first point of contact in cases of premature puberty. By taking a family history, he or she determines whether the parents of the affected child have suffered from similar symptoms in the past; this provides clues to the genetic disposition under which pubertas praecox sometimes occurs. Also required are examinations to rule out the presence of a tumor. A disease is not always present when puberty begins unusually early. If a doctor can rule out the possibility that the symptoms are pathological – that is, that they are caused by a disease – treatment may still be necessary to stop the early onset of puberty. Even if such treatment does not seem necessary at first, the further course of puberty should be closely monitored. This is because early puberty has an influence on the development of body size. For this reason, one of the examinations that a doctor performs in the course of treating pubertas praecox is an X-ray examination of the bones in the hand. With the X-ray he determines the so-called skeletal age (also called bone age). The skeletal age may differ from the chronological age in that certain cartilages and adhesions of the skeleton are already more advanced than those in a representative comparison group. With the aid of these comparative data, the physician can thus make the finding that the skeletal age is a certain number of years ahead of the chronological age. This finding can then be used to make a prediction about the expected later height of the person in question. If necessary, early onset puberty can then be treated hormonally to ensure that the affected child will have an appropriate body size in adulthood. Affected individuals should consult specialists with this concern: In a pediatric endocrinologist, both the necessary examinations and treatment can be performed.Of course, before treating early onset puberty as such, it is necessary to check whether there is not another disease underlying pubertal development.
Complications
If pubertas praecox is caused by a tumor, possible complications result first from the underlying disease and its treatment. In benign growths, surgical removal is usually possible without serious complications. Brain tumors are an exception, however, and their surgical removal is almost always risky. If chemotherapy is indicated for malignant tumors, the complications usually result from the side effects. If a tumor can be ruled out as the cause of premature onset of puberty, serious complications are only to be expected if the disorder is accompanied by short stature or dwarfism (microsomia). If this risk exists, pubertas praecox must be treated hormonally, otherwise the affected person will no longer reach their normal height. If the premature onset of puberty is not pathological, complications can arise primarily when the affected children can no longer cope with their peer environment due to their physical and usually also mental changes. Because of this risk, treatment of pubertas praecox should be considered even if it is not absolutely necessary for medical reasons. Hormonal therapy may be associated with side effects, which must be discussed individually. Complications are not to be feared.
When should you go to the doctor?
The disease pubertas praecox should always be treated by a doctor. There is no self-healing in this process, and the disease cannot be treated by means of self-help either. Early treatment has a positive effect on the further course of pubertas praecox and can prevent further complications and complaints. A doctor should be consulted if the affected person suffers from severe acne accompanied by greasy hair or a heavy vaginal discharge. The symptoms usually appear very spontaneously and can reach different degrees of severity. However, if the symptoms persist for a longer period of time and do not disappear on their own, a doctor must be consulted in any case. Since pubertas praecox can also promote the formation of tumors, regular preventive examinations should be carried out in order to detect and treat cancer at an early stage. As a rule, the life expectancy of the affected person is not negatively affected by pubertas praecox. The condition can be treated by a pediatrician or by a general practitioner. However, seeing a specialist may also be necessary.
Treatment and therapy
Hormone administration can stop the onset of puberty again. This is advisable when there are no pathological reasons for the onset of puberty, as well as when there is a suspicion that early development strongly affects the height of the affected person. In this case, regular administration of the retarding drugs is stopped again only at a later stage, when adequate mental maturity of the affected child has been reached and the skeletal age is approximately aligned with the chronological age. The drugs leuprolerin or triptolerin are approved in Germany for the treatment of premature puberty. They are administered directly in the practice by injection into the subcutaneous fatty tissue. The hormone depot created in this way then blocks the release of the puberty hormones. Once a month, the hormone depot must be replenished by another injection. This type of treatment has no known side effects.
Prevention
Pubertas praecox occurs when hormone regulation of sex hormones is disturbed. Since there are no ways to influence this, prevention of early onset puberty is not possible.
Aftercare
For the aftercare of a successfully treated pubertas praecox, an individual therapy is very important. This should be made dependent on the underlying conditions. Taking medications that curb the formation of sex hormones (so-called GnRH analogues) may also be necessary after treatment of pubertas praecox, as patients will continue to produce too many sex hormones for life if left untreated.This is particularly important for the prevention of bone diseases and malformations. If puberty is to be initiated at an older age, close monitoring and therapy is necessary to achieve normal height growth. Hormone levels should be checked regularly by blood sampling at the doctor’s office. If a removed tumor of the liver was the cause of pubertas praecox, it is absolutely necessary to have regular check-ups with the doctor for early detection of a new tumor. In addition, a lifelong liver-sparing diet (avoidance of offal and a low-fat diet) may be necessary. If the pubertas praecox was caused by a genetic disease such as adrenogenital syndrome, it is important to continue treating this disease even after the successful treatment of pubertas praecox. Lifelong intake of cortisol and progesterone may be necessary. With intensive monitoring of hormone levels in the blood, long-term therapy should be discussed with the physician in charge.
What you can do yourself
When pubertas praecox is diagnosed, it must first be clarified whether premature puberty was triggered by a tumor. It should then be treated as an underlying disease. If the condition is not triggered by a tumor, a decision must be made as to whether the patient should be treated with hormones. This decision should be made early by the parents, since pubertas praecox can lead to short stature. Here, parents should follow the recommendations of their endocrinologist. If medications are prescribed, they must be taken regularly. It is certainly not easy for both patients and their parents to deal with premature puberty. The children feel like outsiders because they are not at the same stage of physical development as their classmates and may even be bullied. At the same time, they are often irritable, moody and unbalanced, which can drive parents to despair. Therefore, both the parents of the affected children and the children themselves should seek psychological care. There are no special support groups for this disorder, which may be due to the fact that it is rare and irrelevant after the completion of puberty. However, there are various forums on the Internet where those affected exchange information.
