Treatment of generalized lipodystrophy

The treatment of patients with the syndrome of generalized lipodystrophy will be successful only when we are able in each case to establish the root cause of the disease. Currently, 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 skeletal muscle hypertrophy, slow down the development of hypertension and myocardial hypertrophy. In some cases, treatment with parlodel was successfully applied, which, in the syndrome of generalized lipodystrophy, not only normalized prolactin levels in the blood, contributed to the disappearance of lactorrhea in patients and restored the normal menstrual cycle, but also caused the above-mentioned positive changes in the clinical and metabolic picture of the disease against the background of a decrease in insulin concentration in blood plasma. Insulin secretion 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 involving endogenous hyperinsulinemia are associated, as a rule, with the hypothalamic insufficiency of these mechanisms, especially in the area of the ventromedial hypothalamus. In this regard, the previously unknown effects of dopamine parlodel synergist on insulin secretion in patients with generalized lipodystrophy syndrome become clear.

Another way to correct metabolic disorders in generalized lipodystrophy is the use of cornitine, the synthesis of which is blocked in the liver by an excess of insulin. Cornitine is necessary for the oxidation of fatty acids with a longer carbon skeleton, which is made difficult by hyperinsulinism. Long-term intake of cornitine normalizes the state of carbohydrate and lipid metabolism. The diet of patients with generalized lipodystrophy should be low-calorie, preferably with nutritional supplements containing short-chain fatty acid residues. Such additives include coconut oil, as well as oil from soflor dye. The oil of these plants contains a large amount of triglycerides with short-chain fatty acid residues, the oxidation of which occurs without the participation of insulin.

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

Forecast and working capacity

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 cases of the disease, death can occur from complications (hepatic coma, bleeding from esophageal varices, stroke, myocardial infarction).

The disability of patients depends on the severity of the disease, the presence of cardiovascular and neuroendocrine complications, such as the clinical course of generalized lipodystrophy syndrome. So, with the early manifestation of the syndrome of generalized lipodystrophy, working capacity is almost not impaired; while manifestation of the syndrome due to pregnancy and childbirth, disability reaches 40%. In the total group of patients with generalized lipodystrophy syndrome, the number of people with disability groups I and II is on average 25%.

[1], [2], [3]