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Secondary hypogonadism
Last reviewed: 23.04.2024
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Secondary hypogonadism, or hypogonadotropic hypogonadism, most often occurs as a result of primary gonadotropic insufficiency, which can be combined with insufficiency of other tropic hormones in the pituitary gland. With isolated gonadotropic insufficiency, leading clinical symptoms are caused by a low level of androgens, as in the case of primary hypogonadism.
Causes of the secondary hypogonadism
The most common causes of hypopituitarism are tumors, vascular disorders, inflammatory processes in the pituitary and hypothalamus, as well as operations on the first. There are also congenital forms of secondary hypogonadism in disorders of embryonic development of the pituitary gland. In plasma, these patients have a low level of gonadotropins (LH and FSH) and testosterone.
Symptoms of the secondary hypogonadism
Hypogonadotropic hypogonadism is characterized by a decrease in the size of the testicles due to hypoplastic and atrophic changes in the parenchyma of the organ. The seminiferous tubules are diminished in size, devoid of lumen, lined with Sertoli cells. Spermatogonia are rare. Spermatogenesis, if observed, is only up to the stage of spermatocytes of the first order. In interstitium, only precursors of Leydig cells are found.
The severity of the symptoms of hypogonadism depends on the degree of insufficiency of the pituitary gland and the age at which the disease occurred.
Forms
[10], [11], [12], [13], [14], [15]
Congenital secondary hypogonadism
Cullman syndrome is characterized by deficiency of gonadotropins (luteinizing hormone and FSH), hyposmia or anosmia (reduction or absence of smell). These patients have an inborn defect in the development of the hypothalamus, which manifests itself in the deficiency of gonadotropin-releasing hormone, which leads to a decrease in the production of gonadotropins by the pituitary gland and the development of secondary hypogonadotropic hypogonadism. The accompanying defect in the formation of olfactory nerves causes anosmia or hyposmia.
Clinically, these patients are diagnosed with eunuchoidism, which is sometimes accompanied by cryptorchidism. In addition, they have such defects as cleavage of the upper lip ("hare's lip") and the palate ("wolf mouth"), deafness, syndactyly (six fingers), etc. The family illness, therefore, from an anamnesis it can be found that some members families are present or there were described malformations. Karyotype in patients with 46.XY. In a plasma, a low content of luteinizing hormone, FSH and testosterone.
Treatment of the Callman syndrome consists in the long-term administration of chorionic gonadotropin or its analogues (prophase, pregnil, choriogonin, etc.) 1500-2000 ED 2-3 times a week intramuscularly - monthly courses with monthly interruptions. In severe hypoplasia of the testicles, along with the chorionic gonadotropin, androgens are injected: injections of sustanone-250 (or omnadren-250) 1 ml once a month or testaten 10% 1 ml every 10 days throughout the year. With a sufficient level of testosterone in the patient's blood as a result of ongoing treatment with gonadotropin alone, it is possible to treat only the chorionic gonadotropin (or its analogs).
It leads to the development of secondary sexual characteristics, an increase in the penis, the normalization of the copulatory function. To restore the reproductive function, it is possible to use gonadotropins with greater follicle-stimulating activity: hugegon, pergonal, neo-intraminal intramuscularly at 75 IU (in combination with a pregel or another analogue of 1500 ED) 2-3 times a week for 3 months. The further use of these drugs depends on the effectiveness of the treatment. Sometimes fertility is restored.
Isolated deficiency of luteinizing hormone is manifested by the symptomatology characteristic of androgen deficiency. The leading role in the development of this pathology is given to congenital insufficiency of luteinizing hormone production and to a decrease in testosterone secretion. The decrease in fructose concentration in the ejaculate observed in these patients, as well as a decrease in sperm motility, is a manifestation of androgen deficiency.
The level of FSH in plasma in most cases remains within normal limits, which explains the safety of all stages of spermatogenesis. Oligozoospermia, observed with this condition, is apparently associated with a deficiency of androgens. In 1950, Paskualini first reported on such patients. They had a distinctly pronounced clinical picture of eunuchoidism and, at the same time, satisfactory spermatogenesis. Such cases of "fertility" in patients with hypogonadism were called "Pascualini syndrome", or "fertile eunuch syndrome". The latter is unsuccessful, as fertility is in most cases violated.
Symptoms depend on the severity of the luteinizing hormone deficiency. This syndrome is characterized by underdevelopment of the penis, sparse hair embolism pubis, axillae and face, eunuchoid body proportions, violation of sexual functions. Patients rarely have gynecomastia, no cryptorchidism. Sometimes they go to a doctor about infertility. The results of the study of ejaculate, as a rule, are of the same type: a small volume, oligozoospermia, low mobility of spermatozoa, a sharp decrease in the content of fructose in the seminal fluid. Karyotype 46.XY.
Treatment of an isolated deficiency of luteinizing hormone is advisable to treat with chorionic gonadotropin or its analogues (pregel, prophase, etc.) 1500-2000 units intramuscularly 2 times a week - monthly courses with monthly interruptions throughout the reproductive life period. As a result of the treatment, both copulatory and reproductive functions are restored.
Maddock's syndrome is a rare disease that occurs as a result of simultaneous insufficiency of gonadotrophic and adrenocorticotropic functions of the pituitary gland. Appears after puberty. In the blood, low levels of luteinizing hormone, FSH, ACTH and cortisol. A small amount in the urine of 17-ACS. With the introduction of chorionic gonadotropin, the plasma testosterone level increases. The thyrotropic function is preserved. The etiology of this disease is unknown.
Symptoms. Patients develop a clinic of eunuchoidism in conjunction with signs of chronic adrenal insufficiency of the central genesis, so there is no hyperpigmentation of the skin and mucous membranes, which is typical for secondary hypokorticism. In the literature there is a description of single cases of this pathology.
Treatment of such patients is carried out along with gonadotropins according to the usual scheme: drugs that stimulate the function of the adrenal cortex (cortico-tropins), or with the help of replacement therapy with glucocorticoids.
[16], [17], [18], [19], [20], [21]
Acquired secondary hypogonadism
These forms of the disease can develop after the transferred infectious and inflammatory processes of the hypothalamic-pituitary region. Thus, in patients with tuberculous meningitis, in some cases signs of hypogonadism develop, accompanied by loss of other functions of the pituitary (thyrotropic, somatotropic), and sometimes - polydipsia. With the radiographic examination of the skull, in some patients it is possible to reveal petrification impregnations over the Turkish saddle - an indirect sign of the tuberculous process in the hypothalamic region. The forms of secondary hypogonadism are subject to replacement therapy as described above. In addition, such patients show substitution therapy with those hormones deficient in the body.
Adiposo-genital degeneration
An independent disease it can be considered only if its signs appeared in childhood and the cause of the disease could not be established. Symptoms of this pathology can develop with organic lesions of the hypothalamus or pituitary gland (tumor, neuroinfection). When establishing the nature of the process that damages the hypothalamus (inflammation, swelling, trauma), obesity and hypogonadism should be considered as symptoms of the underlying disease.
Violation of the functions of the hypothalamus leads to a decrease in the gonadotropic function of the pituitary gland, which in turn leads to the development of secondary hypogonadism. Adiposo-genital dystrophy is most often detected at the prepubertal age (10-12 years). The syndrome is characterized by a general obesity with the deposition of subcutaneous fat by the "female type": in the abdomen, pelvis, trunk, face. Some patients have a false gynecomastia. The proportions of the body are eunuchoid (broad pelvis, relatively long limbs), the skin is pale, there is no hair on the face, in the armpits and pubic (or very meager). The penis and testicles are diminished in size, some patients have cryptorchidism. Sometimes non-diabetes is diagnosed.
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Treatment of the secondary hypogonadism
Treatment of hypogonadism in adiposis-genital dystrophy: injections of chorionic gonadotropin or its analogues (pregnil, prophase, etc.) from 1500 to 3000 units (depending on the severity of obesity) 2 times a week, monthly courses with monthly interruptions. If there is insufficient effect from gonadotropin therapy, simultaneous injections of androgens can be prescribed: sustan-250 (or omnadren-250) 1 ml once a month or 10% injection of testate 1 ml every 10 days. With abnormal liver function, oral administration of the drug is advisable in the patient: andriol 1 capsule 2-4 times a day. Must take measures to reduce weight: a subcaloric diet, drugs that reduce appetite, exercise therapy.