Premature puberty

Sexual maturation is a period in the life of every human being during which hormonal and somatic changes take place in the body, leading to sexual maturity and fertility. Sexual maturation is one stage in the lifelong evolution of the hypothalamic-pituitary-gonadal system.
The period of sexual maturation is characterized by two independent processes. The first is adrenarche, during which there is an increase in the activity of the adrenal glands in terms of secreting steroid hormones, and the second is gonadarche characterized by an increase in the activity of the gonads.
In the pathology of this period, we distinguish two groups of disorders - premature puberty and delayed puberty.

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Premature puberty - what is it?

Maturation is a very complex process influenced by a number of genetic, environmental and socio-economic factors (including nutritional status). The onset of sexual maturation in the hypothalamic-pituitary-gonadal endocrine system is considered to be the increase in the amplitude and frequency of pulsatile secretion of gonadoliberin (GnRH) after childhood rest. Activation of pulsatile GnRH secretion leads to an increase in the secretion of gonadotrophins by the pituitary gland, which stimulate the gonads to produce sex steroids and gametes. The term premature puberty in our latitude describes the appearance of puberty (secondary sexual characteristics) before the age of 8 in girls and 9 years in boys.
Premature puberty may be of central (gonadotropin-dependent) origin associated with the early activation of the hypothalamic-pituitary axis or peripheral (pseudo) without activation of this axis.
Central precocious puberty (true, GnRH-weighted) is when a child of a younger than normal age for the onset of puberty presents with the same symptoms and in the same sequence as during true puberty. Breast and pubic hair development and growth acceleration occur in a similar sequence to normal puberty, except that they occur earlier in life. It is related to the earlier activation of areas in the brain responsible for the control of the ovaries and testes (activation of the gonadal axis).
Peripheral premature puberty (pseudo-GnRH-independent) is when the body begins to produce sex hormones without stimulating the gonadal axis described above. Typically, pubic and axillary hair develops with epidermal acne symptoms, behavioral difficulties, and growth acceleration with bone maturation. Such changes may appear in connection with congenital disorders of the adrenal glands or even neoplastic processes within the adrenal glands or gonads.

In addition to the above-mentioned forms of premature puberty, there are also mild variants of it. These are:
A) adrenarche praecox- is associated with the activity of the adrenal glands. It can occur in both girls and boys and is associated with slightly increased body hair, acne or a change in the smell of sweat;
B) thelarche praecox - isolated breast enlargement, that is, one that is not accompanied by other symptoms of puberty or increased hormone secretion;
C) menarche praecox - premature first menstruation that occurs as a result of the increase in the sensitivity of the endometrium to estrogen levels after activation of the hypothalamic-pituitary-ovary axis, where estrogen levels are too low to cause other pubertal symptoms.

In all cases of premature puberty, an appropriate and, above all, prompt intervention of an endocrinologist is required to establish the causes and initiate possible timely treatment.

Premature puberty - symptoms

The symptoms of premature puberty are:
- premature development of secondary sexual characteristics (in girls, the first symptom is usually enlargement of the nipples),
- acceleration of the growth rate inadequate to the chronological age,
- acceleration of skeletal growth and maturation,
- low final height,
- incorrect body proportions,
- psychosexual development in line with chronological age,
- additionally, there may be acne, oily hair and "puberty" smell of sweat.

Premature puberty - causes

There are many causes of premature puberty, and they should be divided into gonadotropin-dependent and non-gonadotropin-dependent causes.

Premature puberty GnRH-dependent (central):
- intracranial tumors,
- malformations of the central nervous system (neurofibromatosis),
- irradiation of the skull,
- increased intracranial pressure (idiopathic, post-traumatic, post-inflammatory),
- organic changes in the central nervous system as a result of compression, disruption or other damage by a tumor to the nerve pathway that inhibits GnRH secretory neurons.

Premature puberty GnRH-independent (peripheral, pseudo):
- ovarian and / or adrenal gland tumors secreting estrogens,
- exposure to exogenous estrogens (iatrogenic form),
- primary hypothyroidism,
- McCune-Albright syndrome,
- congenital adrenal hyperplasia,
- adrenal gland tumors,
- adenomatous bilateral adrenal hyperplasia,
- ovarian tumors,
- exposure to exogenous androgens (iatrogenic form).

In mild types of premature puberty (adrenarche, thelarche, menarche), medicine does not yet provide a clear answer to the question of their cause.

Premature puberty - diagnosis

In the first stage of the diagnosis of premature puberty, the methods used in auxology (the science of development) are used, which use the following methods of assessing the course of puberty:
1) growth rate (percentile grids),
2) bone age,
3) evaluation of the pubertal stage according to the Tanner scale:
a) for pubic hair (pubarche) - 5 stages,
b) for axillary hair - 4 stages,
c) for the assessment of the development of the mammary glands (thelarche) - 5 stages.

In the history differentiating premature puberty, the period when the first symptoms of premature puberty appeared and how quickly they were building should be taken into account in particular. Additionally, interviews should be conducted:
family, taking into account the age of puberty of parents, siblings,
concerning the intake of exogenous hormones,
concerning health condition (past diseases, injuries),
concerning the neurological symptoms (changes in behavior, changes in appetite, headache, visual disturbances, seizures, fainting).

In the next stage of searching for the causes of premature puberty, the following are also important:
- Laboratory tests:
- hormonal tests, which include, among others determination of the level of gonadotropins, sex steroids, adrenal androgens, thyroid hormones, prolactin, progesterone;
- dynamic hormone tests used to detect enzyme deficiencies: GnRH stimulation test, ACTH test (adrenogenital syndrome), dexamethasone test (adrenal tumors);

- and imaging diagnostics:

- pelvic ultrasound to assess internal genitalia,
- ultrasound of the adrenal glands,
- X-ray of the skull,
- magnetic resonance imaging of the skull,
- computed tomography of the above-mentioned organs,
- in some cases also EEG and other tests ordered by a doctor.

Premature puberty - treatment

There are many causes of premature puberty, which are best categorized as gonadotropin dependent and independent. Such classification helps in understanding the etiology of the disorder and in making decisions about the choice of treatment.
The general goals of treating premature puberty include:
• remission or inhibition of the progression of physical symptoms and hormonal changes,
• inhibition of the rapid progression of skeletal maturation,
• achieving normal final height and correct body proportions,
• future fertility,
• reduction of the increased risk of breast cancer (in case of early menarche),
• preventing the emotional effects of premature puberty.

In the case of central premature puberty (GnRH-dependent), treatment consists of administering drugs (GnRH analogues) aimed at suppressing the activity of the gonadal axis, which stops the maturation process until the child reaches such an age and such emotional maturity, that maturation can take place.
In alleged precocious puberty (GnRH-independent), management depends on the underlying cause. Here it becomes important:
- treatment of the underlying disease of the central nervous system (central nervous system malformations),
- treatment of primary hypothyroidism,
- surgical treatment of the ovaries, adrenal glands (adrenal tumors, ovarian tumors)
- the use of antigonadotrophins, i.e. progestogen-like drugs that inhibit the development of gonads.

In mild types of premature puberty (adrenarche, thelarche, menarche), medicine does not yet provide a clear answer to the question of their cause. Therefore, these types of disorders of the maturation process are only subject to periodic medical observation, do not require treatment and do not affect the future life and development of girls.

Source:
1) Izabela Rogozińska, Przyczyny przedwczesnego dojrzewania – co nowego?, Postępy Nauk Medycznych, t. XXVII, nr 10B, 2014
2) Barbara Garanty-Bogacka, Teresa Adamczyk, Małgorzata Syrenicz, Maria Ginalska-Malinowska, Ewa Małunowicz, Mieczysław Walczak, Adrenarche praecox u 5-letniej dziewczynki z rodzinnie występującym niedoborem dehydrogenazy 3β-hydroksysteroidowej, Endokrynologia Pediatryczna, Vol. 2/2003 Nr 3(4)
3) Jarząbek-Bielecka Grażyna, Warchoł-Biedermann Katarzyna, Sowińska Elżbieta, Wachowiak-Ochmańska Katarzyna, Przedwczesne dojrzewanie płciowe, Ginekologia Polska, 2011, 82, 281-286
4) https://www.juniorowo.pl/przedwczesne-dojrzewanie/
5) Grażyna Jarząbek, Michał Pawlaczyk, Zbigniew Friebe, Przedwczesne pokwitanie dziewcząt, Seksuologia Polska 2005, 3, 1, 29.31

FAQ

1. What features of puberty in a girl might suggest precocious puberty?

In girls, the first sign of puberty is an enlargement of the mammary glands, which may initially affect only one of them. Additionally, the hair on the pubic mound or in the armpits that appears a little later is noteworthy. It is often accompanied by growth acceleration and a change in the smell of sweat, and sometimes with the appearance of seborrheic changes on the face.
When these features appear in a girl before the age of 8, they may suggest premature puberty.

2. Is it possible to cure premature puberty completely?
Treatment of premature puberty of hypothalamic origin ends when the child reaches the physiological age of puberty. Lifetime treatment is required for premature puberty associated with adrenal disease. Nodular gonadal lesions require surgical treatment. Small nodules in the hypothalamus can only be monitored with MRI every 6–12 months.