From Big Medical Encyclopedia

SHYHENA SINDROM (H. L. Sheehan, English endocrinologist and pathologist, sort. in 1900; synonym: Shikhen's disease, a postnatal hypopituitarism) — the disease which is developing at the women after the delivery who were complicated by massive bleeding, and characterized by depression of function of closed glands.

It is for the first time described in 1913 by the Polish pathologist Glinsky (L. To. Glinski). Gave the detailed characteristic of a disease in 1937 Mr. Shikhen.

Sh.'s frequency of page, according to L. S. La netsky and sotr. (1968), makes about one case on 28 178 childbirth. According to Aarskoga (D. T. Aarskog, 1957), on 10 000 bleedings at the time of delivery develop two clinically expressed and seven latent cases of Sh. of page. Arises at the multigiving birth women more often.

Etiology and pathogeny. Sh.'s emergence by the village is connected with the ischemic necrosis of a front share of a hypophysis (adenohypophysis) which developed after the delivery, complicated by massive bleeding, a collapse, and at nek-ry women and sepsis. Sh.'s cases of the page which arose after the abortion which is followed by big blood loss are known.

According to Shikhen and R. Murdoch, intake of blood to functional hypertrophied hypophysis at the time of delivery decreases. At the expressed circulator frustration caused by acute massive blood loss there come the spasm or thrombosis of vessels of a front share of a hypophysis with development of a necrosis in it. Along with expressiveness of blood loss for developing of a disease also the premorbidal background (the late beginning of periods, the small sizes of the Turkish saddle, late toxicosis of pregnant women) matters. So, according to D.F. Chebotaryov, the kapillyaro-spasm taking place at late toxicosis of pregnant women leads to disturbance of blood circulation, increase in permeability of walls of capillaries, insufficiency of food, consolidation of argyrophil substance and necrotic changes in tissues of a liver, kidneys and especially in a front share of a hypophysis. In a crust, time the great value in development of a necrosis of a front share of a hypophysis is attached to the intravascular disseminated blood coagulation (see. Hemorrhagic diathesis).

According to Plaut (A. Plaut), an important role in Sh.'s development by page is played by a functional condition of cells of a front share of a hypophysis during pregnancy, namely their hypersensitivity to a lack of oxygen and influence of intermediate products of exchange. H. Fasbender attaches significance to discrepancy between the raised metabolism in a front share of a hypophysis during pregnancy and childbirth and poor supply by its oxygen because of massive blood loss. Thus, the conditions contributing to emergence of disturbance of food of a front share of a hypophysis arise even during pregnancy, and massive blood loss at the time of delivery, abortion or in an early puerperal period is the allowing factor. There are messages on adverse influence on a front share of a hypophysis of alkaloids of an ergot and Pituitrinum, to-rye apply to fight against uterine bleedings. These connections cause a vasospasm of a hypophysis that can lead to development of a necrosis in it.

According to many researchers, expressed a wedge, the picture of a disease develops in case of defeat more than 80% of fabric of a front share of a hypophysis. At the smaller volume of defeat the disease proceeds in the erased (latent) form or does not arise absolutely since the unimpaired part of gland completely compensates function of body.

Pathological anatomy. In an early stage find ischemic necroses and blood clots in vessels, later — cicatricial changes and an atrophy of a considerable part of a front share of a hypophysis. In the remained sites of its parenchyma basphilic cells prevail. In a back share of a hypophysis sharply expressed plethora is noted. Also the plethora and hypostasis of a soft cover and substance of a brain are defined. Preferential in diencephalic area sharp circulator frustration (staz, hypostasis, perivascular hemorrhages), the expressed dystrophic changes of nervous cells, focal proliferation of cells of a glia are observed. Morfol. changes in peripheral bodies depend on disease severity. The atrophy of ovaries, a uterus, uterine tubes, a vagina and external genitals is noted. The thyroid gland is reduced in sizes, dense, on a section tyazh of whitish fibrous fabric are visible. The epithelium of follicles of a thyroid gland is flattened, follicles are atrophied, filled with a slaboeozinofilny colloid, growth and lymphoid and cellular infiltration of coarse-fibered connecting fabric is noted. In adrenal glands the phenomena of an atrophy are also sharply expressed, their bark is thinned, cells contain few lipids. The pancreas is reduced, microscopically in it the atrophy of islets of Langerhans comes to light. Reduction and other bodies is noted (hearts, a liver, a spleen, kidneys).

The clinical picture is characterized by symptoms, to-rye are observed at the hypopituitarism resulting from other reasons (see the Apituitarism, Gipopi-shuitarizm). One of the first symptoms is disturbance of a lactation — an agalaxia, the gipolaktiya, caused by reduction and the termination of release of prolactin is more rare (see the Lactation). Weakness, drowsiness, weight loss, a hair loss is observed. The atrophy of mammary glands, outside and internal generative organs, an amenorrhea is noted (see), is more rare — a hypomenorrhea. The hypothyroidism develops (see). The termination of allocation leads the ACT of to a partial atrophy of bark of adrenal glands, decrease in production of hormones by it, hl. obr. glucocorticoids, as a result to-rogo ?ostry adrenal insufficiency can develop (see Adrenal glands).

Dangerous complication of Sh. of page is the hypopituitary coma (see), edges can arise at early stages of a disease. The reasons of a coma at Sh. are not finalized by page. Apparently, major importance has a necrosis of a front share of a hypophysis, and also biochemical change in cells of c. N page, brain anoxia, hypoglycemia. Operative measures and infections can become provocative factors. Drowsiness, an adynamia, vomiting, a fever, profuse sweat, spasms can precede a coma. At a gipopi-tuitarny coma delay of pulse, falloff of the ABP is noted, and most often it comes to an end with death of the patient.

Diagnosis. Despite characteristic a wedge, the picture appearing soon after the delivery the page is diagnosed by Sh., as a rule, in several months, and sometimes in several years after the beginning of a disease when there are heavy symptoms of a disease connected with insufficiency of function of a thyroid gland, adrenal glands and gonads. So, according to S. B. Ha-nina and sotr. (1967), in 90% of observations the diagnosis is made not earlier than in two years, and in 70% — in five years and more from the beginning of a disease. Instructions on acute massive blood loss are important for Sh.'s diagnosis by the village during delivery. At a lab. a research decrease in excretion of gonadotrophins and sexual steroids is noted, and also 17-ketoste-roidov (17-KC) and 17-oksikortiko-steroids (17-OKC), absorption of a radioiodine a thyroid gland decreases (see). The great value at diagnosis is attached to definition of a functional condition of a hypophysis by means of test with metopirony (see). At a lab. a research hypochromia anemia, acceleration ROE, a leukopenia, a neutrocytosis, decrease in level of sugar in blood is defined. Symptoms of a diffusion hypoxia of a myocardium (decrease in a voltage, a smoothness of a tooth of T) are defined on an ECG. Data of an elektroentsefalografichesky research confirm dominance of processes of braking in a cerebral cortex. Make a gistero-salpingografiya for identification of an atrophy of a uterus (see Metrosal-pingografiya), a pnevmopelviografiya and ultrasonic scanning.

Differential diagnosis is carried more often out with a tumor of a hypophysis (see the Hypophysis), primary hypothyroidism (see), an addisonovy disease (see). Lack of instructions on bleedings in labor, data rentgenol testifies to a tumor of a hypophysis. researches and emergence of neuroophthalmologic disturbances. At primary hypothyroidism there are no signs of defeat of other endocrine organs, improvement at treatment is noted by thyroid hormones. At differential diagnosis of Sh. of page with an addisonovy disease consider anamnestic data (absence of bleedings in labor), the strengthened hyperpegmentation of skin and mucous membranes.

Treatment includes replacement therapy and is carried out during all life of patients. Appoint corticosteroids (a cortisone, a hydrocortisone, Prednisolonum, dexamethasone) bucketed repeated discontinuous courses in 3 — 4 months and in addition during the periods of flu epidemics, the raised nervous and exercise stress; sex hormones (also gestagena are oestrogenic), according to indications — Thyreoidinum. At a gipopitui-tare coma intravenously enter 100 — 300 mg of a hydrocortisone of gemi-succinate (or 100 — 200 mg of Prednisolonum of a gemisuktsinat), 500 — 1000 ml of isotonic solution of sodium chloride, according to indications — the phenylephine hydrochloride, noradrenaline, apply hyperbaric oxygenation (see).

Forecast. Timely begun rational therapy allows to recover in some cases working capacity (more often — not completely), and sometimes and reproductive function. Late diagnosis and out of time begun treatment lead to disability and disability. Patients can die from a hypopituitary coma and acute adrenal insufficiency, and also from the complications joining owing to falloff of body resistance an infection.

Prevention comes down to timely treatment of genital infantility and normalization of a menstrual cycle, the prevention of bleedings at the time of delivery and an early puerperal period (recovery of coagulative properties of blood, increase in sokratitelny ability of a uterus at the time of delivery); to timely and adequate compensation of acute massive blood loss (better svezhestabiliziro-bathing blood), to recovery of rheological properties of blood, timely removal of the patient from a kollaptoidny state, and also to the prevention and intensive treatment inf. complications in a puerperal period.

See also Pituitary cachexia, the Bibliography: Kanter X. and Gross Yu.,

Shikhen's Syndrome, Klin, medical, t. 43, No. 6, page 21, 1965; L and N e c to and I am L. S., Thick A. S. and Stepanova N. M. Some questions of clinic and treatment of a syndrome Shikhena, Probl. endocrinins., t. 14, No. 1, page 12, 1968; Likht L. L. and Neymark E. 3. Clinic and pathological anatomy of a syndrome of Shikhen, Klien, medical, t. 43, Kya 6, page 143, 1965;

Pasha S. P. Etiologiya and pathogeny of a syndrome Shikhana, Akush. and ginek., No. 5, page 20, 1972; P l e sh to about in A. M. and

Uspenskaya K. F. To a question of a puerperal hypopituitarism or Shikhen's disease, Rubbed. arkh., t. 40, No. 6, page 112, 1968; Serov V. N. Puerperal neuroendocrinal diseases, M., 1978; Khanin S. B., Chudinov-sky A. Ya. and Melnikova A. E. About clinic and treatment of an early stage of a syndrome of Shikhan, Rubbed. arkh., t. 39, No. 4,

page 100, 1967; Chryssikopulos A *, and. lake of Sheehan-Syndrom und Graviditat, Geburtsh. u. Frauenheilk., Bd 34, S. 121, 1974; GR i m e s H. G. a. Brooks M. H. Pregnancy in Sheehan's syndrome, Obstet, gynec. Surv., v. 35, p. 481, 1980; Husslein H. u. Stoger H. Klinik und Therapie des Sheehan-Synd-roms, Wien. klin. Wschr., S. 489, 1974; Shanmanesh M. o. Pituitary function tests in Sheehan's syndrome, Clin. Endocr., v. 12, p. 303, 1980;

S h e e-h a n H. L. The repair of post-partum necrosis of the anterior lobe of the pituitary gland, Acta endocr, (Kbh.), v. 48, p. 40, 1965. V. P. Baskakov.