Treatment of generalized lipodystrophy

Treatment of patients with generalized lipodystrophy syndrome will be successful only when we are able in each case to establish the root cause of the disease. Currently, the treatment is mostly symptomatic. A promising direction is the fight against hyperinsulinemia. It makes it possible to reduce insulin resistance and hyperglycemia, significantly improve lipid metabolism, somewhat reduce hypertrophy of skeletal muscles, slow the development of hypertension and myocardial hypertrophy. In a number of cases, parlodel treatment was successfully used, which, in the syndrome of generalized lipodystrophy, not only normalized the blood levels of prolactin, facilitated the disappearance of lactorrhoea in patients and the restoration of the normal menstrual cycle, but also caused the above positive changes in the clinical and metabolic picture of the disease against the background of a decrease in insulin concentration in the blood plasma. The secretion of insulin is modulated by the ventromedial region of the hypothalamus, and the effect of the hypothalamus on insulin secretion is mainly inhibitory and is due to dopaminergic mechanisms.

Clinical conditions accompanied by endogenous hyperinsulinemia are associated, as a rule, with hypothalamic insufficiency of these mechanisms, especially in the area of the ventromedial hypothalamus. In this connection, the previously unknown effects of synergist dopamine parodolium on insulin secretion in patients with generalized lipodystrophy syndrome become clear.

Another way that corrects metabolic disorders in generalized lipodystrophy is the use of cinnitine, the synthesis of which in the liver is blocked by excess insulin. Cornitin is necessary for the oxidation of fatty acids with a longer carbon skeleton, which is difficult for hyperinsulinism. Long-term use of cinnitus normalizes the state of carbohydrate and lipid metabolism. The diet of patients with generalized lipodystrophy should be low-calorie, preferably with food additives containing short-chain fatty acid residues. To such additives can be attributed coconut oil, as well as oil from the soflora dye. In the oil of these plants contains a large number of triglycerides with short-chain fatty acid residues, the oxidation of which occurs without the participation of insulin.

All of the foregoing suggests that the further development of approaches to the treatment of generalized lipodystrophy syndrome requires the joint efforts of biochemists, endocrinologists, geneticists and pediatricians in order to finally elucidate the pathogenesis of this disease.

Forecast and ability to work. The prognosis for life is favorable. According to a few literary data, the natural duration of the syndrome of generalized lipodystrophy is 35-50 years. In severe disease, death can come from complications (hepatic coma, bleeding from varicose-esophageal veins, stroke, myocardial infarction).

The ability to work of patients depends on the severity of the disease, the presence of cardiovascular and neuroendocrine complications, such as the clinical course of the syndrome of generalized lipodystrophy. Thus, with an early manifestation of the syndrome of generalized lipodystrophy, the capacity for work is practically unaffected; At the same time, when the syndrome is manifested in connection with pregnancy and childbirth, invalidization reaches 40%. In the general group of patients with generalized lipodystrophy syndrome, the number of I and II disability groups is on average 25%.