Treatment of generalized lipodystrophy

Treatment of patients with generalized lipodystrophy syndrome will be successful only when we can establish the root cause of the disease in each specific case. Currently, treatment is mainly symptomatic. A promising direction is the fight against hyperinsulinemia. It makes it possible to reduce insulin resistance and hyperglycemia, significantly improve lipid metabolism indices, somewhat reduce skeletal muscle hypertrophy, slow down the development of hypertension and myocardial hypertrophy. In a number of cases, treatment with parlodel was successfully used, which in generalized lipodystrophy syndrome not only normalized the content of prolactin in the blood, contributed to the disappearance of lactorea in patients and the restoration of a 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 the concentration of insulin in the blood plasma. Insulin secretion is modulated by the ventromedial hypothalamus, with the hypothalamus' effect on insulin secretion being primarily inhibitory and mediated by dopaminergic mechanisms.

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

Another way to correct metabolic disorders in generalized lipodystrophy is the use of cornitin, 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 in hyperinsulinism. Long-term use of cornitin 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. Such additives include coconut oil, as well as safflower oil. 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 of the above suggests that further development of approaches to the treatment of generalized lipodystrophy syndrome requires joint efforts of biochemists, endocrinologists, geneticists and pediatricians in order to finally clarify the pathogenesis of this disease.

Prognosis and working capacity

The prognosis for life is favorable. According to the few literary data, the natural duration of generalized lipodystrophy syndrome is 35-50 years. In severe cases of the disease, death may occur from complications (hepatic coma, bleeding from varicose veins of the esophagus, stroke, myocardial infarction).

The ability to work of patients depends on the severity of the disease, the presence of cardiovascular and neuroendocrine complications, and the type of clinical course of generalized lipodystrophy syndrome. Thus, with early manifestation of generalized lipodystrophy syndrome, ability to work is practically not impaired; at the same time, with manifestation of the syndrome due to pregnancy and childbirth, disability reaches 40%. In the general group of patients with generalized lipodystrophy syndrome, the number of those with disability groups I and II is on average 25%.

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