ADIPOSOGENITAL DYSTROPHY (dystrophia adiposogenitalis; lat. adiposus — fat and genitalis — sexual, genital; synonym Pekhkrants's disease — Babinsky — Frelikh) — the disease of neuroendocrinal system which is characterized by the progressing obesity, an underdevelopment of outside and internal generative organs and depression of function of gonads.
For the first time this syndrome was described in 1899 by the Russian doctor Pekhkrants, then in 1900 J. Babinski and in 1901 A. Frohlich. The disease meets seldom, a current its chronic, is often shown from children's age.
Organic lesions of a hypophysis or a hypothalamus, a tumor of these educations, neuroinfections, a nanocephalia, functional frustration of podbugorny area and a hypophysis can be a cause of illness.
Obesity at And. - of develops as a result of disturbance of the central regulation of feeling of saturation owing to damage or irritation of paraventrikulyarny and ventro-medial kernels of a hypothalamus. At patients appetite sharply increases, obesity progresses. Dysfunction of a hypothalamus leads to decrease in gonadotropic function of a hypophysis and as a result of it to an underdevelopment of a reproductive system (hypogonadism).
The similar syndrome can be caused in animals by defeat of a medial eminention and ventro-medial kernel of a hypothalamus that leads to disturbance of release of the follicle-stimulating and luteinizing hormones and lack of feeling of saturation. At some patients with And. - of along with disturbances in a hypothalamus also structural changes in a hypophysis are found.
Pathoanatomical changes at And. - of depend on the basic pathological process (a tumor, neuroinfection), sometimes it is not possible to find any morphological changes.
The clinical picture
the Disease is characterized by slow development of clinical symptoms. At patients the considerable adiposity on a stomach, hips on female type (fig. 1) is noted. Pilosis on a pubis and in axillary hollows is absent or scanty. Men have no hair of a pas the person, the voice remains high, the underdevelopment of genitalias, testicles is noted (one or both) do not fall to a scrotum. At women also outside and internal generative organs are underdeveloped, periods are absent.
Integuments at patients usually pale, their dryness is often noted. On site mammary glands excess adiposity, nipples pale. Age izmenennya in bones (bone age) lag behind those at healthy people. Patients complain of decrease in sexual feeling and sexual weakness, of headaches, bystry fatigue, the increased appetite, sometimes I am eager.
If the disease develops from children's age, decrease in intelligence and working capacity is possible. And. - of at children is characterized by sharply gross obesity of all body (fig. 2) and clinically does not differ from the course of a disease at adults.
And. - of can be combined with emergence in sick symptoms of a hypothyroidism, insufficiency of functions of bark of adrenal glands and with the phenomena of not diabetes mellitus.
On the electroencephalogram — decrease in electroactivity of a cerebral cortex.
The progressing obesity can lead to cardiovascular disturbances, elephantiasis, nonspecific arthroses. As a rule, patients are infertile. At tumors of a hypophysis or a hypothalamus decrease or restriction of fields of vision, brain frustration can be found.
The diagnosis is made on the basis of symptoms of a disease: obesity and hypogonadism. Content in urine of estrogen at women and testosterone at men is usually reduced. The lowered content in urine of gonadotrophins is noted. In the presence of a tumor of a hypophysis on the roentgenogram of a skull changes of a form and size of the Turkish saddle are defined.
To differentiate And. - it is necessary for of with the syndromes of primary damage of gonads and genetic diseases which are followed by secondary obesity and also with Laurence's syndrome — Muna — Bidlya, for to-rogo obesity, a hypogonadism, a pigmental retinitis, mental retardation, a polydactylia is characteristic (see Laurence — Muna — Bidlya a syndrome).
The forecast concerning life favorable; the absolute recovery comes seldom.
Is carried out treatment of a basic disease (a roentgenotherapy, an operational oncotomy); at inflammatory processes antibiotics, biyokhinol.
Food of patients shall not contain more than 1200 — 1600 kcal a day. Fasting days of 1 — 2 time a week are recommended (in day of 800 — 900 kcal). Use the drugs reducing appetite, in certain cases — diuretic. Appoint Adiposinum on 50 — 100 PIECES a day intramusculary courses for 20 days.
The chorionic gonadotrophin is applied to treatment of a hypogonadism. Treatment is carried out in doses from 1500 to 3000 PIECES intramusculary every other day or 2 times a week during 3 — 4 weeks by bucketed repeated courses of 2 — 3 months. To children up to 10 years a chorionic gonadotrophin two times a week enter in a dose 500 — 1000 PIECES.
The serumal gonadotrophin is recommended to apply on 1000 PIECES intramusculary 2 times a week during 6 — 8 weeks; use together with a chorionic gonadotrophin is possible. Treatment by gonadotrophins in many cases promotes omission of testicles in a scrotum at boys if treatment is begun timely. When treatment by a chorionic gonadotrophin inefficiently, is appointed replacement therapy sex hormones. To men — propionate of testosterone in a dose of 0,01 — 0,025 g and 0,05 g intramusculary 2 — 3 times a week within 1 — 2 month. To women — oestradiol propionate 2 — 3 times a week on 0,001 g intramusculary.
Prevention of progressing of process at the beginning obesity comes down to strict observance of a diet, at a cryptorchism — to bringing down of testicles in a scrotum. At functional deviations in sexual development at young men and girls in the pubertal period dynamic observation and timely use of therapeutic actions is necessary.
Bibliography: Zhukovsky M. A. Children's endocrinology, page 315, M., 1971; The Mzhogotomny management on internal * «sheznyam, under the editorship of E. M. Tareev, t. 7, page 373, L., 1966.
N. T. Starkova.