Adipozogenitalny dystrophy – the neuroendocrine pathology which is shown the excess weight and an underdevelopment of gonads. Is followed by obesity on ginoidny type and lag in sexual development. Diagnostics is based on fizikalny survey of the pediatrician or endocrinologist, a research of a hormonal background. The X-ray analysis and a tomography of gipotalamo-hypophysial area is in addition appointed. Therapy of the main disease which caused dystrophy is shown. Symptomatic treatment includes introduction of a horionichesky gonadotrophin, replaceable hormonal therapy, dietary food.
Adipozogenitalny dystrophy (syndrome of Pekhkrantsa-Babinskogo-Frelikha) – a pathological state which results from violations of work of gipotalamo-hypophysial system. The disease was for the first time described at the end of the XIX century by the Russian doctor Pekhkrants. In several years symptoms of a disease were reflected in works of the French neuropathologist Joseph Babinsky and the Austrian pharmacologist Alfred Frelikh. Pathology arises in the childhood or the pubertatny period and a thicket affects boys aged from 6 up to 13 years. The syndrome can be an independent disease or manifestation of other pathological process. In the latter case symptoms can appear at any age.
Reasons of adipozogenitalny dystrophy
In spite of the fact that symptoms of a disease are usually shown at teenage age, formation of pathological process can begin at a stage of pre-natal development. Carry to the main reasons for developing of pathology:
- Brain tumors. The syndrome can arise at the kraniofaringioma, hromofobny adenoma of a hypophysis, other malignant and good-quality new growths located in a zone of a hypothalamus and hypophysis.
- Pre-natal infections. Pathology is provoked by the viral and bacterial infections transferred the woman during pregnancy: flu, toxoplasmosis, clamidiosis, scarlet fever, measles, typhus.
- Some infectious diseases. To number of the infections capable to cause development of dystrophy, carry the meningitis, encephalitis, tuberculosis, syphilis postponed at children's age.
- Traumatic damages. Lead the patrimonial injuries of newborns closed and open ChMT with damage of a hypothalamus and hypophysis to development of a syndrome.
Violations of work of a hypophysis and hypothalamus are the main reason for developing of pathology. As a result of disorder of endocrine function secretion of gonadotropny hormones adenogipofizy decreases, the gipogonadizm is formed. In certain cases lag of sexual development is combined with violation of production of tireotropny, somatotropny and antidiuretichesky hormones. Damage of a hypothalamus leads to pathological irritation of its kernels and increase in appetite. There are bulimia episodes, patients consume excess amount of food, obesity develops.
Symptoms of adipozogenitalny dystrophy
The disease begins at children's and teenage age, is shown by a weight increase, increased fatigue, drowsiness, decline in the ability to training. At patients violation of appetite as bulimia is observed. During the pubertatny period boys have a ginekomastiya, the kriptorkhizm, a penis underdevelopment is noted. At girls the delay of the beginning of periods up to an amenorea comes to light, an underdevelopment of mammary glands, a uterus and appendages. There are no secondary sexual characteristics (existence of hair in axillary hollows and on a pubis, on a face at boys).
Formation of bone and articulate system is broken, flat-footedness, a valgusny curvature of shins is found. Formation of a skeleton on evnukhoidny type is characteristic. Patients have the high growth, disproportionately long extremities, the big size of a leg. The libido is lowered or is absent. Due to obesity and insufficient food of a cardiac muscle the miokardiodistrofiya which leads to reduction of warm emission and violations of a rhythm develops. The syndrome does not influence mental capacities, intellectual development corresponds to age.
Integuments are pale, smooth, dry. Violation of pigmentation of skin, an inflammation of a cornea and dryness of eyes is often observed. Fatty deposits are localized in a stomach, hips, a breast, the person. At boys increase in weight proceeds on ginoidny (female) type. Adipozogenitalny dystrophy is often combined with other endocrine pathologies: autoimmune tireoidit, hypothyroidism, akromegaliya, gipersomniy. In most cases patients lead an inactive life that aggravates obesity.
At late diagnostics or immunity to therapy there are complications from cardiovascular system: heart failure, sclerous changes of vessels, violations of a rhythm. At increase in the size of a tumor the sdavleniye of surrounding fabrics which is followed by sight violations (high degree of a miopiya and a gipermetropiya, a cornea ulcer) and increase in intra cranial pressure is noted. Lag in formation of gonads leads to erectile dysfunction and impotence at men, amenory and to infertility at women. The complications connected with development of obesity are characteristic: diabetes 2 types, coronary heart disease. Dyskinesia of biliary tract promotes stagnation of bile and developing of a zhelchekamenny disease. With a growth of a tumor change of behavior, increase in nervous excitability, violation of consciousness and development of a coma is possible.
Diagnosis of adipozogenitalny dystrophy is based on data of survey of the pediatrician and endocrinologist, studying of the anamnesis of life and the course of pregnancy of mother, laboratory and tool researches. At suspicion on a delay of sexual development patients go for consultation to the gynecologist or the andrologist. The diagnosis is confirmed in the presence of preobesity or obesity with primary adjournment of fat in the top half of a body, a hypoplasia of gonads and lack of secondary sexual characteristics. During inspection carry out:
- Definition of the hormonal status. Make blood test on hypophysis hormones, sex hormones (FSG, LG, testosterone and estrogen).
- Radiological and tomographic researches. During a X-ray analysis of a skull, KT of the Turkish saddle, MRT of a brain and a hypophysis tumors, hemorrhages, dropsy, increase in the sizes and deformation of the Turkish saddle can be visualized.
Differential diagnosis of dystrophy is performed with a syndrome and Itsenko-Cushing's disease of which normal sexual development is characteristic. Pathology should be distinguished from the hereditary alimentary and constitutional obesity having family character and normal stages of puberty. The disease is also differentiated with Lorensa-Muna-Bidl, Morganyi-Styuarta-Morelya, Shereshevsky-Turner, Klaynfelter's syndromes. For diagnosis of the listed pathologies genetic researches with detection of chromosomal anomalies are used.
Treatment of adipozogenitalny dystrophy
Etiotropny treatment is directed to elimination of the disease which entailed development of a pathological syndrome. At tumoral process surgical intervention, X-ray therapy, chemotherapy is carried out. Medicamentous therapy of infectious and inflammatory diseases includes purpose of antibacterial or antiviral medicines, vitamin and mineral complexes. Symptomatic treatment assumes change of a diet and way of life, introduction of hormonal medicines. In the absence of contraindications from the musculoskeletal device physiotherapy exercises, swimming, the Scandinavian walking are shown.
To all patients the low-calorie diet with the lowered content of digestible carbohydrates and vegetable fats is appointed. Enter fresh vegetables and complex carbohydrates which increase feeling of saturation into a diet. 6-7 times a day are recommended to eat food, without allowing long starvation. At development of bulimia the anoreksigenny medicines suppressing appetite are shown. Therapy of a gipogonadizm assumes introduction of HGCh. At achievement of pubertatny age to boys appoint gonadotropny medicines in combination with testosterone, to girls – estrogen with progesterone.
Forecast and prevention
The current of a syndrome depends on an etiology of the main disease. At timely diagnostics and treatment the forecast favorable. Late detection of a syndrome, development of complications and the progressing obesity cause disability, complicate normal activity. Prevention of a disease consists in sanitation of the chronic centers of an inflammation at children and planning of pregnancy of future mother. The healthy nutrition, refusal of addictions and replaceable hormonal therapy can slow down development of a disease. At competent treatment in the pubertatny period patients develop on age. At observance of a diet it is possible to control body weight and to avoid the complications connected with obesity.