HYPOGONADISM

From Big Medical Encyclopedia

HYPOGONADISM (hypogonadismus; grech, hypo-+ gone birth, seed + ad[en] of iron; synonym: hypogenitalism, hypogonadotropic insufficiency) — designation of all forms of insufficiency of gonads — the morbid condition arising owing to reduction of secretion of sex hormones and which is characterized by an underdevelopment internal and external genitals and secondary sexual characteristics.

Distinguish primary gonadalny insufficiency — embryonal, dopubertatny, post-pubertal forms G., and also a form G. at Verner's syndrome (see Verner a syndrome) and secondary (hypogonadotropic) gonadalny insufficiency.

The etiology and a pathogeny

Primary G. can develop as a result of a direct injury of a gonad (infectious and inflammatory, traumatic, genetic, embryonal character), at castration, tumors of gonads; the last are often combined with a cryptorchism.

Secondary G. arises owing to insufficient stimulation of gonads hormones of a hypophysis, i.e. at decrease in secretion gonadotropic hormones (see), at damages of a hypophysis and a hypothalamus, under the influence of a stress, tumoral or infectious processes in c. N of page etc. Secondary G. only during the pubertal period and later when a feed-back of a gonad — a hypothalamus — a hypophysis — gonads forms is shown. Secondary G. can develop at an acromegalia, adiposogenital dystrophy, a pituitary nanism, a pituitary cachexia, Itsenko's disease — Cushing, an inborn myxedema, tumors of bark of adrenal glands, a diabetes mellitus. G.'s signs can be also at some not endocrine diseases.

Pathological anatomy

Pathological changes in testicles and ovaries depend on the nature of the disease which was G. Chasto's reason note the phenomena of an atrophy of ferruterous fabric, fibrous dystrophy, hyalinization.

A clinical picture

the Existing G.'s classifications aim to reflect highlights of a pathogeny (primacy or secondariness of defeat of a gonad), terms of manifestation of a syndrome (embryonal, dopubertatny, post-pubertal forms) and degree of manifestation of a disease.

Clinical manifestations of G. depend on degree of insufficiency in an organism of sex hormones, on the period of life of the patient, in Krom there was a disease, and are defined by symptomatology of changes internal both external genitals and degree of manifestation of secondary sexual characteristics.

Embryonal forms G. can clinically be shown by an agenesia or dysgenesis of gonads (see), an incomplete masculinization (see. Virilescence ), testicular feminization (see), cryptorchism (see), anarchy (see), Klaynfelter's syndrome (see. Klaynfeltera syndrome ). At manifestation of embryonal forms G. in the early embryonal period (to 20 weeks of an embryogenesis) develops hermaphroditism (see).

Dopubertatny forms are characterized by absence (or insufficiency) secondary sexual characteristics, generative organs, formation of eunuchoid proportions of a body as a result of a delay of maturing of a skeleton (see. Eunuchoidism ), at girls — lack of periods (see. Amenorrhea ).

Post-pubertal forms are never followed by signs of an eunuchoidism. At men secondary sexual characteristics disappear, develops impotence (see), at women periods stop, the progressing atrophy of internal and outside genitalias comes to light.

Manifestations of dopubertatny or post-pubertal forms of primary G. in combination with symptomatology of the main pathology are characteristic of secondary G. (defeat of c. N of page, system a hypothalamus — a hypophysis, a liver, etc.). E.g., at the dopubertatny form of secondary G. connected with decrease in secretion by a hypophysis not only gonadotropic, but also somatotropic hormone, patients can lag behind in growth. External genitals at these patients are developed correctly, but considerably lag behind age norm. Gonads of a gipoplastichna. Secondary sexual characteristics develop poorly, more often completely are absent. At normal secretion of somatotropic hormone patients usually have the high growth and eunuchoid proportions of a body. At women mammary glands do not develop, at men the high pitched voice remains. Generative function suffers: women have no ovulation, at men spermatozoa do not ripen.

The progressing hypotrophy internal and external genitals with regress of secondary sexual characteristics is characteristic of a post-pubertal form. At women the menstrual cycle is broken or there is an amenorrhea. Men have a sexual weakness, oligozoospermias or an azoospermism.

At patients symptoms of disturbance of exchange processes often come to light: obesity or cachexia, cardiovascular disturbances, nevrol. symptoms, changes of kostnosustavny system, disorder of proteinaceous and fatty exchanges.

Mental disturbances are various depending on time and an origin of G. U of patients with G. caused by castration or a disease of gonads, psychological disturbances consist of manifestations of a kastratsionny syndrome (headaches, the phenomena of the expressed vegetative dystonia and affective lability) and reactive manifestations which development is closely connected with premorbidal features of the personality.

Primary G. which developed in the early childhood usually is followed by the expressed signs of mental infantility (see) with naivety, dependence, cowardice, motor awkwardness, grotesque hyper compensatory aspiration to «maturity», with tendency to reasoning, self-affirmation in fictions of heroic contents. Along with it development patol, fear reactions, neurotic frustration is possible. Emergence of the last is promoted by sneers of peers concerning obesity of these patients, their motor awkwardness, cowardice. Patients at youthful age have no timely awakening of interest in an opposite sex.

At the secondary G. developing owing to infections, intoxications, a brain injury quite often the mental disturbances connected with the main defeat of c are also observed. N of page See also Endocrine mental syndromes.

The diagnosis

the Diagnosis is made on the basis of symptoms of an underdevelopment internal and external genitals, an underdevelopment (absence) of secondary sexual characteristics, results of a genetic research (definition of a karyotype and sex chromatin). For differential diagnosis of primary G. from secondary carry out determination of content of gonadotrophins in blood serum. Test with introduction of a chorionic gonadotrophin indirectly helps differential diagnosis: increase in secretion of sex hormones indicates secondary (hypogonadotropic) G. U presence of patients with secondary G. quite often reveal symptoms of depression of function of a thyroid gland and bark of adrenal glands as a result of reduction of activity of a hypophysis.

Biopsy gonads carry out at all embryonal forms G., a cryptorchism and at suspicion on neoplastic process of tissues of gonads. The condition of tissue of gonads is important not only for specification of the diagnosis, but also for the choice of treatment and the forecast.

Radiodiagnosis bone changes it is carried out in the help of a survey and aim X-ray analysis, and also pictures with direct blowup; apply to specification of degree of a mineralization of a bone tissue X-ray densitometry (see).

On roentgenograms of a skull of sick G. often find the roundish or oval site of calcification of a firm meninx in frontal department and vertically located, «straightened» dorsum sellae (a so-called yuvenilization of a saddle); straightening of a dorsum sellae makes an impression of expansion of an entrance to it. The corner of a back of a saddle is increased (128 ° ±7 ° at norm 106 ° ±9 °). At primary G. the small sizes of the Turkish saddle are frequent. At G. caused by castration, the sizes of the Turkish saddle can be increased that is considered as a result of a hyperplasia of basphilic elements or development of adenoma of a hypophysis. At secondary G. reveal tumors of a hypophysis, a cranyopharyngioma, symptoms of cerebral hypertensia.

Roentgenogram of a brush of the healthy person (at the left) and patient with a hypogonadism (on the right); shortening IV and V metacarpal bones.

At rentgenol. a research of other departments of a skeleton usually find unsharply expressed osteoporosis, is more often in distal departments of beam and elbow bones, bones of a brush, and also in a backbone. Dystrophic changes in a backbone are expressed in decrease in height of front departments of bodies of vertebrae (more often in nizhnegrudny department). The body of a vertebra takes the form of the stupid wedge turned by a top of a kpereda. Note a platyspondylia or cartilaginous hernias less often. The age differentiation of a skeleton at a dopubertatny form G., as a rule, is late. The uneven growth of metaepiphyseal zones of phalanxes of brushes, beam and elbow bones which is expressed in formation of various form and size of deepenings and ledges, and also a subchondral sclerosis is observed. Lengthening and a nek-swarm thinning of bones of extremities is characteristic.

At patients with primary G. connected with genetic pathology (see. Turner syndrome ), anomalies of a skeletogeny (e.g., shortening of phalanxes of the IV—V fingers of brushes and feet, shortening IV and V metacarpal bones) — a positive metacarpal syndrome (fig.), deformation of Madelunga, inborn wedge-shaped vertebrae, fan-shaped merge of edges, deformations of a breast and medial condyles of tibial bones meet.

Treatment

At primary G., since pubertal age, continuous replacement therapy is shown by sex hormones (in the past an opotherapy). To men administer the drugs with androgenic activity, to women — estrogen (before emergence of the induced periods), passes further to a combination therapy with estrogen and progesterones. At secondary G. carry out treatment by gonadotrophins, if necessary — to combinations with sex hormones, according to indications — Clomifencitratum (see). Drugs, their doses and a method of introduction select individually depending on a form of a disease, age of the patient and expressiveness of symptoms of G.; it is necessary to carry out treatment of also basic disease.

Attempts of substitution nonfunctioning (or absent) a gonad functionally active (from the donor or a corpse) made surgical treatment at the end of the last century. Development of this direction in G.'s treatment is promoted by improvement of the surgical equipment, methods of preservation of bodies, studying of compatibility of fabrics and research of active immuno-depressants.

Operation of change of ovaries and testicles is quite difficult, demands knowledge of the equipment of a seam on vessels of small caliber. The gonad of the donor is taken with preservation of a vascular leg (a distal branch from a. uterina to an ovary or and. et v. testicularis to a small egg). The anastomosis of vessels of the replaced gonad is made or with branches of a femoral artery and vein, or with the lower epigastriß artery and a vein, at preservation of a seed cord of the recipient — with and. et v. testicularis. The small egg can be fixed in a scrotum, under skin of a stomach or from an inner surface of a hip; the ovary is fixed under skin of a hip. At an underdevelopment of a penis attempts plastically it become to reconstruct. Options of operations come down to formation of a bucket-handle graft at preservation of tissues of penis for ensuring erotogenny sensitivity at the sexual intercourse (see. Coitus ).

Sometimes with the cosmetic purpose apply replanting of synthetic plastic materials («small egg») in a scrotum (for the purpose of imitation of existence of a small egg).

The forecast

the Forecast for life favorable. However in the absence of treatment increase of an inferiority complex can lead in certain cases to suicide attempts. At systematic treatment development of secondary sexual characteristics is reached, there are induced periods, a libido, a potentiality, the possibility of sex life is provided, epiphenomena disappear (osteoporosis, lag in formation of a skeleton etc.).


Bibliography: Buchmann A. I. Radiodiagnosis in endocrinology, M., 1975; Zhukovsky M. A. Children's endocrinology, page 341, M., 1971; L of e of e d and N * with to and I am K. S. Mental disturbances at children with pathology of rate of puberty, M., 1969, bibliogr.; The multivolume guide to internal diseases, under the editorship of E. M. Tareeva, t. 7, page 620, M., 1966; Smirnova E. D. and Kirpa-tovsky I. D. Bases of the operational technology of organ transplantation, page 206, M., 1972; Starkova N. T., Buchmann A. I. and Kharitonov of E. I. Sostoyaniye of kostnosustavny system at a men's hypogonadism (a clinicoradiological research), Probl, endocrinins, and a gormonoter., t. 15, Ne 3, page 3, 1969; BleulerM. Endokrinologische Psychiatrie, Stuttgart, 1954, Bibliogr.

H. T. Starkova; A. I. Buchmann (rents.), I. V. Golubeva (hir.), K. S. Lebedinskaya (psikhiat.).

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