What is Adiposogenital Dystrophy (Perchkranz – Babinsky – Frohlich Disease)?
The disease is characterized by a combination of obesity and hypogenitalism. As an independent disease, it can be considered only if symptoms develop in childhood.
Causes of Adiposogenital Dystrophy (Perchkranz – Babinsky – Frohlich Disease)
The cause of the disease is unknown.
Pathogenesis during Adiposogenital Dystrophy (Perchkranz – Babinsky – Frohlich Disease)
Pathological changes in the hypothalamic-pituitary system with a pronounced clinical picture is not observed. The pathogenesis is determined by the insufficiency of the gonadotropic function of the pituitary gland.
Symptoms of Adiposogenital Dystrophy (Perchkranz – Babinsky – Frohlich Disease)
Adiposogenital dystrophy (AGD) begins to manifest from 10-2 years old (in prepubertal disease). Obesity is characterized by a uniform (general) distribution of fat with a predominant deposition in the abdomen, pelvis, thighs, face, chest (false gynecomastia). The face is large, moon-shaped, with no plethoria or eruptions (as in the case of Itsenko-Cushing’s disease). Limbs relatively thin. The skin is soft, dry.
In children, there are signs of hypogenitalism: the scrotum and penis are hypoplastic, the testicles are reduced, cases of cryptorchism are described. Gradually, eunuchoid body proportions are formed, teenagers have little or no sexual hair growth. The bone age lags behind the true one due to the delay in ossification of the epiphyses. There is weakness in the ligamentous apparatus and the skeleton (flatfoot, genu valgum, cubitas valgus).
There are no deviations on the side of the internal organs. Sometimes there are signs of diabetes insipidus. The mental development of the individual does not suffer, is characterized by emotional instability due to consciousness of their own inferiority.
In adults, characteristic obesity, hypogonadism and hypogenitalism, eunuchoid skeletal features, often sexual dysfunction and infertility. With age, due to dystrophic changes in the heart and hypotonia of the vessels of the internal organs, shortness of breath, flatulence, biliary dyskinesia can develop.
Diagnosis of Adiposogenital Dystrophy (Perchkranz – Babinsky – Frohlich Disease)
In diagnostics, the presence of characteristic signs of obesity and hypogonadism in prepubertal age, when obesity in adiposogenital syndrome must be differentiated from alimentary obesity, which is less pronounced puberty delay, disappearing by the period of puberty (almost like obesity itself). Unlike other forms of endocrine and metabolic obesity, obesity with adiposogenital dystrophy is not a symptom of certain diseases with distinct specific clinical picture (pituitary – Cushing, obesity with the hyper-kortitsizme, hypothyroidism, Lawrence syndrome – Moon – Biedl, Morgagni syndrome – Stuart – Morel). The AGD clinic is close to Lawrence-Moon-Beadle syndrome, however, with the first one there is no mental retardation and only congenital anomalies are characteristic of the second (retinitis pigmentosa, six fatigue). From all forms of primary hypogonadism with obesity, AGD is characterized by low levels of gonadotropic hormones.
The diagnosis is determined by the determination of bone age, the study of radiographs of the Turkish saddle (no changes), detection of low basal metabolic rate, detection of increased tolerance to carbohydrates (hypoglycemia on an empty stomach, low rise in glycemia after glucose load), low levels in blood and urine gonadotropic and sex hormones .
Treatment of Adiposogenital Dystrophy (Perchkranz – Babinsky – Frohlich Disease)
The complex of therapeutic measures includes a sub-calorie diet (1200-1500 calories with fasting days), adiposin injections of 50-100 U per day in 20-day courses, anorectics (dezopimon or fepranon in 0.025 30 minutes before meals 2 times a day, in a course 20 days), diuretic drugs, blind sensing, hormonal drugs: human chorionic gonadotropin 1500 – 3000 IU intramuscularly 2 times a week in courses for a month, for a long time. In severe hypoandrogenization, it is helpful to prescribe androgenic drugs in parallel.