Idiopathic delayed puberty

　　How is idiopathic delayed puberty caused? Briefly described as follows:

　　The causes of delayed puberty are often divided into three major categories: temporary disorders of gonadotropin and sex hormone secretion, hypothalamic-pituitary lesions causing gonadotropin secretion disorders, and delayed puberty caused by gonadal dysfunction.

　　I, Temporary disorders of gonadotropin and sex hormone secretion

　　1, Malnutrition or chronic consumptive diseases: such as tuberculosis, diabetes, chronic liver disease, malabsorption syndrome of the small intestine, bronchial asthma, etc., affecting the development of the nervous system and body organs, so that although the patient has reached the age of puberty, the bone age indicates that the overall development is far behind the normal level of the same age, and does not have the systemic basis for gonadal development.

　　2, Normal physiological variation: The age of onset of puberty, the speed of development, the age of maturity, and the degree of development all have great individual differences, not only between races and between men and women, but also in normal cases, among the same gender, the same race, and even in general environmental conditions similar, there may be different. Both men and women also have three types: early puberty, average, and late puberty. The reasons for this difference may be genetic, nutritional, emotional, and other environmental, social and economic factors, and can also be familial or sporadic.

　　3, Other: such as growth hormone deficiency in patients with growth delay, bone age delay, and often accompanied by delayed gonadal development, very similar to constitutional delayed puberty. Thyroid hormone deficiency and hyperprolactinemia, or long-term use of corticosteroids may suppress sex hormone secretion, causing delayed puberty.

　　II, Delayed puberty caused by hypothalamic-pituitary lesions

　　1, Kallmann syndrome: It is a genetic disease, often manifested as autosomal dominant inheritance or X-linked recessive inheritance. Patients have low pituitary gonadotropins, often accompanied by hypoplasia of the olfactory bulb and the septum. Leading to loss of smell, cleft palate, cleft lip, and other conditions.

　　2, Monogenic gonadotropin deficiency: it may be scattered or a genetic disease.

　　3, Partial deficiency of gonadotropins: manifested asLuteinizing hormone (LH). LH) secretion is lackingThe pituitary gland secretes follicle-stimulating hormone (FSH),. FSH) is normally secreted. Androgen deficiency, sperm development is受阻.

　　4, Hypopituitarism of full or partial function: pituitary tumors, or suprasellar and parasellar tumors, pituitary infiltrative diseases, radiation therapy. Trauma and surgery can all lead to hypopituitarism.

　　Third, delayed puberty caused by incomplete gonadal function

　　1. Turner syndrome: It is a gonadal developmental abnormality caused by a lack or abnormality of sex chromosomes, with an incidence rate of about 1 in 2500 among women. Patients have a female phenotype, with underdevelopment, infertility, short stature, webbed neck, etc.

　　2. Genetic disorders of sex hormone synthesis and peripheral action deficiency: Often accompanied by abnormal sexual differentiation. A typical example is 17-hydroxylase deficiency, incomplete or partial insensitivity to androgens. Patients may develop breasts, but have primary amenorrhea and lack of pubic hair.

　　3. Acquired primary gonadal hypofunction: Injuries, chemotherapy, radiation, infection can all lead to the obstruction of gonadal development, affecting the synthesis of gonadal hormones and the generation of germ cells.

　　4. Disorders of gonadal development: Delayed puberty can also occur when the development of gonadal function is impaired.

　　What diseases can idiopathic delayed puberty trigger? A brief description is as follows:

　　1. Delayed puberty in women can temporarily affect post-marital fertility, but once normal development occurs, most people will not affect fertility like normal people, because delayed puberty in women no longer exists.

　　2. Idiopathic delayed puberty can lead to malnutrition or chronic消耗性疾病, such as tuberculosis, diabetes, chronic liver disease, malabsorption syndrome of the small intestine, bronchial asthma, etc., affecting the development of the nervous system and body organs, so that although the patient's age has reached puberty, the bone age indicates that the overall development is far behind the level of normal people of the same age, and does not have a systemic basis for gonadal development.

　　What are the symptoms of idiopathic delayed puberty? A brief description is as follows:

　　The child experiences growth arrest, with a height shorter than that of age-matched children, a delayed bone age, but growth rate consistent with bone age. The initial appearance of adrenal cortical function is delayed, which is different from children with isolated gonadotropin deficiency, whose adrenal cortical function appears on schedule. There are no signs of secondary sexual characteristics, and plasma gonadotropins and sex hormones remain at the level of prepubertal children. Often, there is a family history of delayed puberty, with the father's puberty starting at 14-18 years or the mother's breast development or menarche delayed. In addition, development in other aspects of the body is normal, with both testicles descended into the scrotum, no hypospadias, no anosmia, and no congenital physical malformations. PlasmaLuteinizing hormone (LH). LH levels,The pituitary gland secretes follicle-stimulating hormone (FSH),. FSH levels and testosterone or E2 levels are lower than those of age-matched children, but are comparable to the bone age of the child.Gonadotropin-releasing hormone (GnRH). The response to GnRH stimulation reflects the maturity of the hypothalamic-pituitary axis. The response of children with idiopathic delayed puberty is similar to that of prepubertal normal children, showing a weak or no response. If the plasma LH peak is greater than 2.0 U/L after an intravenous push of 100 μg gGnRH, the child will inevitably enter puberty within one year. Among patients with idiopathic delayed puberty, a few have a normal final height, but most cannot reach the expected height, possibly because the growth of their spine is reduced compared to the normal population with puberty starting on time. Some believe that most of these patients also have familial short stature.

　　How to prevent idiopathic delayed puberty?

　　How to prevent idiopathic delayed puberty? The following is a brief description:

　　1. The causes of delayed puberty are very complex, including congenital hereditary factors, as well as acquired factors such as nutrition and diseases. If delayed puberty is found, it is necessary to seek medical attention in a timely manner.

　　2. For children approaching puberty, parents should explain some knowledge of adolescent hygiene to them in a timely manner. Girls should be educated to pay attention to menstrual hygiene after puberty. When a gynecological examination is needed, do not hesitate to seek medical attention to avoid delaying treatment and causing irreversible damage (such as hymen imperforate).

　　4. For boys, it is necessary to pay attention to the presence and development of testicles in the scrotum from birth. If the testicles have not descended by one year of age, or if other abnormalities are found, it is necessary to go to the hospital for treatment in a timely manner to avoid missing the best treatment period. If cryptorchidism persists until puberty, even after surgical reduction, spermatogenic function may be severely damaged and cannot be restored. Actively prevent (treat) complications of epidemic parotitis and orchitis.

　　5. During adolescence, it is important to protect the reproductive organs, prevent damage to the testicles from improper exercise; avoid exposure to strong radiation, and do not wear tight pants for a long time.

　　6. Reduced intake of vitamin A is one of the causes of delayed puberty, and vitamin A and iron should be supplemented.

　　7. Long-term delayed puberty during adolescence can affect bone mineralization, increasing the risk of fractures in adulthood. Therefore, guided short-term sex hormone treatment should be given to promote the onset of puberty.

　　What should be done for the examination of idiopathic delayed puberty? The following is a brief description:

　　1. Endocrine hormone examination.If the delayed puberty is caused by poor development of the gonads,The pituitary gland secretes follicle-stimulating hormone (FSH),. FSH),Luteinizing hormone (LH). LH) is significantly elevated, T and E2 decrease; in cases with hypofunction above the hypothalamus-pituitary axis, FSH and LH decrease. The examination of TSH, F, and PRL is important for diagnosisHypothyroidism. , Cushing's syndrome, and hyperprolactinemia can lead to delayed puberty.

　　2. Growth hormone stimulation test.It is very important for distinguishing growth retardation caused by delayed puberty and growth hormone deficiency, because the former can promote growth by induction of puberty, while the latter requires treatment with growth hormone. Therefore, it is necessary to perform a growth hormone stimulation test when necessary.

　　3. GnRH stimulation test.Caused by hypothalamic lesionsGonadotropin-releasing hormone (GnRH). The GnRH test may show a reaction, indicating that the pituitary gland is still secreting gonadotropin cells normally, but according to our experience, it is usually necessary to apply GnRH treatment for 5 to 7 days before performing the GnRH test, as the reaction is more pronounced. Poor response to the GnRH stimulation test is caused by pituitary lesions.

　　4, CT or MRI.When there are clinical clues suggesting hypothalamic-pituitary lesions, CT or MRI is of great value.

　　5, Vision and field examination.It helps to judge whether there is tumor compression of the optic nerve.

　　6, Ultrasound.For girls, it can understand the development of ovaries and uterus.

　　7, Testicular biopsy.Generally not used, it can clearly determine whether the differentiation of germ cells is normal.

　　8, Bone Age.In patients with isolated hypogonadotropic hypogonadism, the bone age lag is relatively light, and the bone age lag is more obvious due to constitutional and hypopituitarism.

　　What should be paid attention to in the diet and health care of patients with idiopathic pubertal delay? Briefly described as follows:

　　During puberty, when the body is growing, it is necessary to supplement nutrition and have a reasonable diet. In addition, reduced intake of vitamin A is one of the causes of delayed puberty, and vitamin A and iron should be supplemented.

　　What are the treatment methods for idiopathic pubertal delay? Briefly described as follows:

　　Male patients should be given oral undecanoate testosterone, 40mg/d, or undecanoate testosterone injection or testosterone enanthate injection 100mg, intramuscularly once every 4 weeks, with 4 months as one course. A comprehensive evaluation of the treatment response should be made after the course of treatment is completed.

　　Female patients should be given ethinylestradiol (diethylstilbestrol) 5ug/d or combined estrogen 0.3mg/d orally starting from 13 years old, with 3 to 4 months as one course. It may be sufficient for one course to initiate the development of secondary sexual characteristics without causing premature bone age and hindering the achievement of the expected final height.

　　If spontaneous puberty does not start within 3 to 6 months after the end of a course of treatment, the second course of treatment can be given. Usually, only 1 to 2 courses of treatment are needed to achieve the goal. When the bone age of boys reaches 13 years and girls reach 12 years, puberty will start within one year.

　　There is still controversy about the treatment of short stature children with growth hormone therapy, and although the treatment of idiopathic pubertal delay can temporarily increase growth rate, it cannot significantly increase the final height, and it is not recommended to use growth hormone therapy.