Kalman's syndrome

In this article, one of the forms of hypotonadrophic ovarian hypofunction is considered - Kalman's syndrome.

Hypogonadotropic amenorrhea of hypothalamic genesis develops against the background of congenital or acquired deficiency of GnRH synthesis by the hypothalamus, pituitary insufficiency has a mixed hypothalamic-pituitary genesis, and is a leading symptom of hypogonadotropic hypogonadism.

Hypogonadotropic amenorrhea of the hypophyseal genesis can develop as a result of acquired gonadotropin deficiency as a result of surgical intervention, trauma, hemorrhage, neuroinfection, intoxication, infectious lesion. In clinical practice, most often doctors face pathological conditions like the syndrome of the "empty" Turkish saddle, the Shien syndrome, the latter develops due to necrotic changes in the pituitary gland that appear in its anterior lobe after bleeding or bacterial shock during childbirth and abortion.

[1], [2], [3]

Epidemiology

Kalman's syndrome (olfactogenital dysplasia) occurs in women with a frequency of 1: 50,000.

[4], [5], [6]

What causes the Kalman syndrome?

Congenital insufficiency GnRH - Kalman's syndrome (olfactogenital dysplasia) - a violation of the development of the hypothalamus, manifested by deficiency of GnRH and, as a result, gonadotropins. Concomitant symptoms of the disease is a violation of the sense of smell - hyposmosis or anosmia due to partial or complete agenesis of olfactory bulbs.

How is Kalman's syndrome manifested?

In addition to hypoxia, primary amenorrhea and, as a consequence, primary infertility are observed. The constitution of the eunuchoid species, there is rarely a mild development of mammary glands. When gynecological examination, the external genitalia are hypoplastic, there is no development of secondary sexual characteristics, the uterus and ovaries are slightly reduced, which indicates sexual infantilism.

How to recognize Kalman's syndrome?

When hormonal studies reveal a low content of LH, FSH and estradiol, the normal level of prolactin

Criteria for the diagnosis of the Kalman syndrome:

• hypogonadotropic hypogonadism;
• anosmia / hypochma;
• atrophy of olfactory bulbs, determined by MRI;
• positive test with GnRH agonist.

Differential diagnosis

Differential diagnosis is performed with other forms of hypogonadotropic ovarian hypofunction.

[7], [8], [9], [10], [11], [12], [13], [14], [15], [16], [17]

Treatment

The appointment of hormone replacement therapy is indicated.

Recovery of fertility is carried out with the help of gonadotropins and GnRH agonists:

Menotropins intramuscularly at the same time 75-150 ME. 1 time per day from the 3rd day of the menstrual cycle until the dominant follicle reaches a diameter of 18 mm or Follitropin alfa subcutaneously at the same time 75-150 IU once a day from the 3rd day of the menstrual cycle until the dominant follicle reaches a diameter of 18 mm

+ (after the course)

Gonadotropin chorionic intramuscularly 10 000 ME once.

If Л> 15 ME / l:

Triptorelin intramuscularly 3.75 mg once on the 21st day of the menstrual cycle

+ (after the course)

Folitropin alfa subcutaneously at the same time 75-150 IU once a day from the 3rd day of the menstrual cycle until the dominant follicle reaches a diameter of 18 mm

+ (after the course)

Chorionic ganadotropin intramuscularly 5000 - 10 000 IU once. The adequacy of the dose of menotropins and follitropin alfa is estimated by the dynamics of follicle growth (in norm - 2 mm / day). With slow growth of the follicles, the dose increases by 75 ME, with too rapid growth - decreases by 75 ME.

Evaluation of treatment effectiveness

The effectiveness of treatment is assessed by the development of menstrual bleeding and restoration of fertility.

[18], [19], [20], [21], [22], [23], [24], [25]