Endocrinology / / May 23, 2016
Cindromy defeat hypothalamic area, accompanied by violation of the production of hormones of the anterior pituitary
The term "adipozo-genital dystrophy" as a distinct disease only included those forms of pathology in which obesity and hypoplasia of the reproductive system apparent in childhood, and can not be establishedthe nature of the process leading to it.For those patients who have identified a particular disease process, damaging the hypothalamus (inflammation, tumor, trauma), obesity and hypogonadism should be considered as symptoms of the underlying disease.In these cases, the diagnosis should identify the main pathological process and adipozo-genital dystrophy syndrome, reflected in the diagnosis, will characterize the features of its clinical manifestations.Apparently, with increasing diagnostic possibilities will occur less and less reasons for the diagnosis "adipozo-genital dystrophy."
Some patients develop adipozo-genital dystrophy can be supplied in connection with intrau
The basis of the pathogenesis of obesity at adipozo-genital dystrophy is a violation of the central regulation of food intake - increase in appetite due to damage or irritation of the paraventricular and ventromedial hypothalamic nuclei.The value of these nuclei in the regulation of appetite and shown subsequently confirmed by numerous experiments and clinical observations.Additional
value apparently has metabolic disorder - a reinforced transition carbohydrates fats observed at hypothalamic obesity, reduction of the fat depots mobilization in response to the impact of sympathetic and lowering motor activity.
at adipozo Hypogonadism-genital dystrophy is associated with a decrease in the production of gonadotropins, whose dependence on the hypothalamic regulation proven by numerous studies.There have been reports which suggests possible value of the direct effects of the nerve through the sympathetic nervous system in the genesis of hypogonadism, and in particular cryptorchidism, when adipozo-genital dystrophy.In the propagation of the disease process in the region supraoptic nuclei or break the nerve pathways tr.supraoptico-hypophyseus some patients adipozo-genital dystrophy develops diabetes insipidus.
Pathological changes in the central nervous system at adipozo-genital dystrophy depend on the primary pathological process (cancer, inflammation, and so on. N.).In many cases it is not possible to reveal any morphological changes.
Adipozo-genital dystrophy as an independent disease is rare.Adipozo-genital dystrophy occurs in both sexes.She observed her three times more often in boys than in girls.Perhaps this is due to the fact that boys in childhood and adolescence more clearly than in girls, the symptoms of hypogonadism noticeable.Adipozo-genital dystrophy is usually detected after the age of 6-7 years, most often - in the prepubertal period - 10-12 years.
syndrome characterized by a general obesity with preferential deposition of fat on the "female pattern" in the abdomen, pelvis, torso, breasts, face.But of course this type of obesity is also complete, and there is no characteristic tsentripetalnogo fat redistribution inherent in Cushing syndrome - Cushing.
- pituitary dwarfism, clinical
- Diagnosis and differential diagnosis of pituitary dwarfism
- treatment of pituitary dwarfism
- Diagnosis and differential diagnosis adipozo-genital dystrophy
- Treatment adipozo-genital dystrophy
bone growth in length is usually not disturbed.Limbs often relative to the length of the body.Often there genu valgum, cubitus valgus, flat feet, excessive mobility in the joints of the hands.In men, there is a wide basin on the female type.
normal skin color, sometimes pale, thin, elastic.Hair thin head.In adolescence and poslepubertatnom period of hair growth of pubic and axillary areas is absent or weakly expressed, in boys - on the female type.
muscular system is underdeveloped.
Mutations voice and growth of the larynx in boys does not usually occur.
characteristic feature adipozo-genital dystrophy, in addition to obesity, is a sharp hypoplasia of the genitals.In boys, it shows a very small size of the testicles and scrotum, sometimes unilateral or bilateral cryptorchidism.The dimensions of the penis is very small.Identify hypoplastic genitalia in girls presents considerable difficulties.
the internal organs - heart, lungs, liver, kidneys, gastrointestinal tract - usually not detected abnormalities.There is a tendency to hypotension.
Many patients have a relative or absolute lymphocytosis.fasting blood sugar is often reduced.Sugar curve is often low, flattened.
In the propagation of the disease process in the area of the hypothalamus, regulates water exchange, there may be signs of diabetes insipidus - thirst and polyuria with the release of a low specific gravity of urine.
Hormonal studies in adolescence and poslepubertatnom period reveal a decreased excretion of gonadotropins and estrogen.There is a tendency to reduce the allocation of a 17-keto steroids.The function of the thyroid, the gland does not usually broken.
Intelligence is usually normal.However, the sharp and obesity - in later years - a delay of sexual development in some patients produce some difficulties in the relationship with the staff, which leads to emotional instability, isolation, inclination towards sedentary lifestyles.