Precocious puberty
"Premature pubertal development" is defined as the rapid pre-term growth of a subject with respect to the population in question; the individual's pubertal development, however, suddenly stops, causing serious anatomical, structural and statural consequences in adulthood.
Transition period
It would be useless to describe precocious puberty without first defining the very concept of puberty: it is a very delicate period of change, which expresses the passage from "childhood to" adolescence, the moment in which the future woman and the future man mature from the sexual point of view, acquiring the ability to reproduce.
The increase in the production of hypothalamic GnRH delineates the onset of puberty: FSH (follicle stimulating hormone) and LH (luteinizing hormone) are the gonadotropins produced by the pituitary gland which, when secreted in large quantities, favor a significant increase in gonadal steroid levels [tract from Clinical Andrology, Wolf-Bernhard Schill, Frank H. Comhaire, Timothy B. Hargreave].
The acquisition of reproductive capacity, the appearance of secondary sexual characteristics and the radical psychological changes in the subject, represent the first stage that leads to full sexual maturation, as a consequence of the strengthening of the synthesis of gonadal hormones and the stimulation of gonadotropic cells.
It is clear that, when pubertal development occurs before the physiological age (8-10 years), in the organism the growth mechanisms are accelerated prematurely, causing considerable upheaval, physical and psychological, in the affected subject.
Males and females
Pubertal development has slight differences between male and female: more precisely, the upper limit of early pubertal development corresponds to the age of 8 years for the female and 9 years for the male, an age that varies according to the population. , nutritional status and genetic factors.
The causes that favor early pubertal development are also variable: if in the female the problem is hardly related to precise causes (we speak of origin idiopathic), early pubertal development is associated with diseases of a nature in most affected males organic (compared to the reference population).
Basically, for the female, the early pubertal development consists in the "growth of the breast and in the anticipation of the first menstruation (menarche), while in the male it corresponds to a notable development of the testicular volume; in both cases, the final outcome is a deficit of stature in adulthood, which expresses the result of the premature welding of the growth plates induced by sex hormones (in particular by estrogens).
Peripheral and central precocious puberty
For "peripheral early pubertal development"s" means a condition caused by the use of sex hormones or by diseases affecting the adrenal gland or ovary, regardless of gonadotropin production: peripheral early pubertal development is also defined pseudo precocious puberty, a condition in which secondary sexual characteristics are favored by tumors secreting.
When it early pubertal development it defines itself "of central origin", the activation of the" hypothalamus-pituitary-gonadal axis occurs precociously, despite the fact that the regulation mechanism is almost identical to that of physiological puberty: it is precocious puberty true, which therefore favors the pre-term activation of hypothalamic GnRH. Consequently, there is an increase in the production of LH and FSH (precocious puberty that depends from gonadotropins). Central precocious puberty is characterized by the premature development of pubic and breast hair, which promotes rapid growth and, at the same time, accelerates bone maturation.
Central precocious puberty
In central early pubertal development, two types are distinguished:
- True idiopathic precocious puberty: As previously mentioned, the idiopathic form is present in 75% of girls with this disorder. The idiopathic form manifests itself with a clear female prevalence (incidence index: 1 male for every 10 females "affected" by precocious puberty); probably, this happens because the female sensitivity to GnRH is decidedly lower than the male, so the Idiopathic precocious puberty in girls is favored by a lower dose of gonadotropins. In idiopathic precocious puberty, an etiological research, however detailed, fails to identify any organic pathology in progress; the precocity of development seems to be favored, in these cases, by a modulation of uterine neuronal maturation to GnRH (hypothalamic hamartoma).
- True precocious puberty induced by known lesions / trauma: early pubertal development of the central type can also be induced by known neural trauma, including hypothalamic tumors (glioma, ependymoma), optic glioma, cerebellar tumors, tumors of the epiphysis ( germinoma, meningioma, pinealoma), head trauma, inflammatory processes (abscesses, granuloma) and malformative diseases (Williams Beuren syndrome, tuberous sclerosis).
Diagnosis
The early signs of ovarian development, in the female, can be highlighted by pelvic ultrasound, of absolute diagnostic utility; in addition, also magnetic resonance, the saddle examination (radiological analysis of the development of the skull) and radiography (which estimates the maturation skeletal bone), represent possible diagnostic investigations.
The male and female who register an early pubertal development can undergo special tests to assess the amount of gonadotropins produced by the pituitary: the LHRH test (acronym for LH Releasing Hormone) estimates the pituitary gonadotropin reserve. The LHRH test consists of the intravenous administration of LHRH synthetic, in order to evaluate the alterations in the levels of the hormones LH and FSH after some time intervals.
High values of testosterone in males and estradiol in females were calculated both in idiopathic premature pubertal development and in precocious puberty caused by CNS lesions.
Therapies and solutions
Therapies aimed at treating early pubertal development must be aimed at healing the cause which induced the problem, if this is recognized. It is clear that when precocious puberty is caused by a tumor, the most suitable therapy is chemotherapy, radiotherapy or surgical excision.
The purpose of the treatment, in addition to the eradication of the tumor (when present), is aimed at preventing the further (possible) development of secondary sexual characteristics that appeared prematurely. We have seen that an almost inevitable consequence of precocious puberty is represented by the final low height of the subject in adulthood; in this regard, therapies must also be aimed at slowing down the processes of accelerated bone growth.
In the case of a failure to identify the triggering factor, the therapy generally adopted in cases of idiopathic precocious puberty uses a synthetic peptide, known as LHRH, which is distinguished from the natural analogue peptide only for an amino acid. Synthetic LHRH appears to be able to inhibit excessive and premature production of gonadotropins.
In the past, patients suffering from early pubertal development were treated with progestogens, but the effects were not satisfactory, especially for the missed (and hoped for) outcomes on adult stature; in precocious pseudo-puberty testolactone and ketoconazole are still used.
Currently, the therapy dedicated to young patients suffering from premature pubertal development consists in the use of substances analogous to GnRH.
Summary
Definition
Premature pubertal development: rapid pre-term growth of a subject compared to the population under examination
Puberty early: secondary sexual characteristics appear before the age of 8
Puberty anticipated: secondary sexual characters appear between 8 and 10 years of age
Puberty
A very delicate period of change, which expresses the passage between childhood and adolescence, in which sexual maturation and the ability to reproduce is acquired.
- Incidence: Puberty is anticipated before the age of 8
- Prevailing causal factors: idiopathic origin of the disorder
- Features: breast augmentation and anticipation of the first menstruation (menarche)
- Incidence: precocious puberty is anticipated before the age of 9-10
- Prevailing causal factors: organic diseases
- Characteristics: remarkable development of the testicular volume
Consequences in adulthood
Deficit related to stature
Classification
- Peripheral precocious pubertal development: precocious pseudo-puberty
- Central precocious pubertal development: true precocious puberty
- Early idiopathic central puberty appears to be favored by hypothalamic hamartoma; it affects 75% of girls with early pubertal development
- Induced early central puberty could be favored by hypothalamic tumors, optic glioma, tumors of the epiphysis, head trauma, inflammation or malformative diseases
Diagnosis
Pelvic ultrasound, MRI, saddle exam, X-ray, LHRH test, testosterone and estradiol assessment
Therapies
- Cancer-induced early pubertal development: chemotherapy, radiotherapy, or surgical excision (to heal tumors)
- Idiopathic precocious puberty: use of the synthetic amino acid LHRH, testolactone and ketoconazole.
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