What should I do if my child has precocious puberty, what is true precocious puberty and pseudo precocious puberty, what tests should I do and how should I treat it?
Recently, a good friend came to me for advice on whether her daughter should have an MRI of her skull, and upon closer questioning, she learned that her child had been diagnosed with precocious puberty. The parents were very nervous, and after I answered them one by one, I found that many parents actually lacked a basic understanding of this disease, or had poor communication with the attending doctor during the consultation, so many doubts were left unanswered.
Classification
Precocious puberty refers to boys before the age of 9 and girls before the age of 8 presenting secondary sexual characteristics, girls more than boys, according to its nature into 2 types.
1, true precocious puberty (CPP), also known as gonadotropin-releasing hormone (GnRH)-dependent precocious puberty, central precocious puberty, complete precocious puberty, with the hypothalamus-pituitary-gonadal axis (HPGA) similar to normal pubertal development, the programmatic process of initiation and maturation, until the maturation of the germline; that is, by the hypothalamus in advance of the secretion and release of gonadotropin-releasing hormone (GnRH), activating the pituitary gland The secretion of gonadotropins causes the gonads to develop and secrete sex hormones, resulting in the development of internal and external genitalia and the appearance of secondary sexual characteristics. In addition to gender-appropriate secondary sexual characteristics (breast development, external genitalia development, body hair, throat nodes and other changes), the child also has accelerated growth, increased bone age and the ability to have children.
2, pseudosexual precocious puberty, also known as non-GnRH-dependent precocious puberty, peripheral precocious puberty, incomplete precocious puberty is hypothalamic-pituitary-gonadal axis is not mature, due to various reasons caused by the body sex steroid hormone elevation to puberty level, so only the early appearance of secondary sexual characteristics, does not have the integrity of the developmental process.
In addition, according to the characteristics of the second sex characteristics, when the second sex characteristics appear the same as the original sex of the child, it is called homosexual precocious puberty, and when it is opposite to the original sex, it is called heterosexual precocious puberty. The so-called heterosexual precocious puberty is characterized by male breast development and female clitoral enlargement.
Performance]
Parents who find precocious puberty in children often see abnormal development of the child’s body, and the first thing they notice is mostly breast changes. But in fact, the clinical manifestations are not only this.
For true precocious puberty, there are 5 points as follows.
1, the second sex characteristics appear early (boys before the age of 9, girls before the age of 8), and according to the normal development process progress, the process of girls are: breast development, height growth rate increased suddenly, pubic hair development, generally in the breast development began 2 years after the first tide presented. For boys, the process is: testicular and penile enlargement, height growth rate, pubic hair development, and generally 2 years after the testicular enlargement, voice change and seminal emission.
2, there is a basis for gonadal development, girls are judged by B-ultrasound images, and boys have testicular volume ≥ 4 ml.
3.The development process presents a sudden increase in height growth.
4.Gonadotropins are elevated to pubertal level.
5. The bone age may be advanced, but not all bone age advancement is necessarily precocious puberty.
Pseudosexual precocious puberty has the following 4 points.
1. Early appearance of secondary sexual characteristics (before the age of 9 for boys and 8 for girls).
2. The development of sexual characteristics does not progress according to the normal developmental procedure.
3. The size of the gonads is at prepubertal levels.
4. The gonadotropins are at prepubertal levels.
This kind of precocious puberty only has partial early development of sexual characteristics but no maturation of sexual function.
Etiology]
This is when parents want to know the causes of precocious puberty.
The causes of true precocious puberty are the following 4 points.
1, organic lesions of the central nervous system, such as hypothalamus, pituitary tumors or other central nervous system lesions.
2. Transformation from peripheral precocious puberty.
3.Idiopathic central precocious puberty (idiopathic CPP, ICPP) is called if no organic lesion is found.
4.Incomplete central precocious puberty is a special type of CPP, which refers to the early appearance of secondary sexual characteristics in children, and its control mechanism also lies in the activation of hypothalamic-pituitary-gonadal axis, but its sexual characteristics development is self-limiting; the most common type is simple premature breast development, if it occurs in girls within 2 years of age, it may be due to the hypothalamic-gonadal axis being in a physiologically active state, also known as “small puberty”. The most common type is simple premature breast development.
In girls, ICPP is more common, accounting for more than 80%-90% of CPP, while in boys, on the contrary, more than 80% is organic.
Etiology of pseudoprecocious puberty
In case of girls.
1. Homosexual precocious puberty (secondary sex characteristics in girls): seen in genetic ovarian function abnormalities such as McCune-Albright syndrome, benign ovarian occupational lesions such as autonomic ovarian cysts, estrogen-secreting adrenocortical tumors or ovarian tumors, ectopic human chorionic gonadotropin (HCG)-secreting tumors, and exogenous estrogen intake.
2. Heterosexual precocious puberty (male secondary sexual characteristics): seen in congenital adrenocortical hyperplasia, androgen-secreting adrenocortical tumors or ovarian tumors, as well as exogenous androgen intake, etc.
In case of boys
1. Homosexual precocious puberty (male secondary sexual characteristics): seen in congenital adrenocortical hyperplasia (more common), adrenocortical tumors or testicular mesenchymal cell tumors, ectopic HCG-secreting tumors, and exogenous androgen intake, etc.
2. Heterosexual precocious puberty (female secondary sexual characteristics): seen in estrogen-producing adrenocortical tumors or testicular tumors, ectopic HCG-secreting tumors, and exogenous estrogen intake, etc.
Seeing the etiology also explains well that it is not the case that pseudo-precocious puberty is less severe than true precocious puberty. This is why doctors prefer not to simply refer to both diseases as true precocious puberty and pseudoprecocious puberty, but prefer to use the more scientific names of gonadotropin-releasing hormone (GnRH)-dependent precocious puberty and non-gonadotropin-releasing hormone (GnRH)-dependent precocious puberty.
Test]
In order to accurately distinguish the type of precocious puberty and arrange reasonable treatment as early as possible. Some auxiliary examinations are needed to confirm the diagnosis.
1. Basal sex hormone measurement. Basic luteinizing hormone (LH) has screening significance, such as LH <0.1 IU/L indicates that there is no central puberty initiation, LH>3.0-5.0 IU/L can be sure that there is central puberty initiation. β-HCG and AFP should be included in the basic screening test as important clues for the diagnosis of HCG-secreting germ cell tumors. Elevated levels of estrogen and testosterone are diagnostic aids.
2.Gonadotropin-releasing hormone (GnRH) stimulation test.
Method: A certain amount of GnRH is injected subcutaneously or intravenously, and serum LH and follicle stimulating hormone (FSH) levels are measured at 0, 30, 60 and 90 min after the injection. The true developmental threshold is determined by: peak LH >3.3-5.0 IU/L, and a central precocious puberty is diagnosed when the LH/FSH ratio is >0.6. Currently, it is considered that a single excitation value of 30-60 min after excitation, meeting the above criteria, can also be diagnosed.
If the peak of excitation is mainly elevated in FSH and the LH/FSH ratio is low, it may be early stage of simple premature breast development or central precocious puberty in combination with the clinical condition, and the latter needs regular follow-up and repeat examination if necessary.
3. Utero-ovarian B-ultrasound. Unilateral ovarian volume ≥1-3 ml and multiple follicles ≥4 mm in diameter can be considered to have entered pubertal development; uterine length >3.4-100 px can be considered to have entered pubertal development, and endometrial shadows can be seen indicating a meaningful increase in estrogen. However, B ultrasound findings alone cannot be used as a basis for the diagnosis of CPP.
4.Bone age. It is an important basis for predicting adult height, but is not specific for identifying central and peripheral.
If it is diagnosed to be, gonadotropin-releasing hormone (GnRH)-dependent precocious puberty, the etiological diagnosis should be completed: brain CT or MRI examination (focusing on the saddle area) is needed, especially for the following cases.
1. all boys with a confirmed diagnosis of CPP.
2. Girls under 6 years of age with onset of the disease.
3. Those with rapid sexual maturation or other central pathologies.
And to confirm the diagnosis of non-gonadotropin-releasing hormone (GnRH)-dependent precocious puberty, further endocrine examinations are performed according to specific clinical features and after the initial screening of endocrine hormones, and imaging of gonads, adrenal glands or other related organs are done as needed. Only those with a clear history of exogenous sex steroid hormone intake may be exempted from complex examinations as appropriate, but regular follow-up examinations are required.
[Treatment].
If a child is unfortunately diagnosed with gonadotropin-releasing hormone (GnRH)-dependent precocious puberty, parents are faced with the questions of whether treatment is needed, whether medication should be used, the side effects of medication, and for how long. First of all, even if a child is diagnosed, he or she does not necessarily need treatment. The goal of treatment is to suppress premature or rapid sexual development and to prevent or slow down the social or psychological problems associated with precocious puberty (e.g. early menarche) in the child or parents; improving adult height that is impaired by early bone age is also an important goal.
Indications for application if the aim is to improve adult height.
1.Bone age is 2 years or more older than age, but need to be ≤11.5 years for girls and ≤12.5 years for boys.
2, predicted adult height: girls <3750px, boys <4000px.
3) or height judged by bone age SDS <-2SD (judged by normal population reference value or genetic target height).
4. Rapid developmental progression with bone age growth/age growth > 1.