Generalized lipodystrophy: an overview of information

Generalized lipodystrophy is a little-known disease that can be viewed not as a combination of individual symptoms, but as a single pathological process with inherent patterns and developmental features, although the term "generalized lipodystrophy syndrome" (SGL) is quite acceptable.

The main manifestation of the disease is the disappearance of subcutaneous fat on the large body surface or in certain areas of it, as well as the presence of metabolic disorders such as insulin resistance and / or hyperinsulinemia, hyperlipidemia, hyperglycemia.

The first report of this syndrome was made by LH Ziegler in 1923, and in 1946, RD Lawrence gave a detailed clinical description. In the world literature, this disease is also referred to as "lipoatrophic diabetes", "total lipodystrophy", "Lawrence syndrome". This syndrome was first described by NT Starkova and co-authors. (1972) under the name "hypermuscular lipodystrophy".

Despite the fact that the literature contains data on a small number of patients with generalized lipodystrophy syndrome, clinical experience suggests that in fact this disease occurs much more often. Poor knowledge of doctors makes early diagnosis of the disease difficult. In addition, the syndrome of generalized lipodystrophy for a long time was considered only as a cosmetic defect, and other clinical manifestations with the underlying disease usually did not bind. At the same time, the tendency of the syndrome to progress, the emergence of such severe complications in a number of patients of working age such as liver cirrhosis, myocardial infarction, dynamic cerebral circulation disorder, the high circulation of patients with generalized lipodystrophy syndrome to infertility gynecologists, necessitate the development of approaches to treatment and prevention of the disease.

Causes of generalized lipodystrophy. The cause of the syndrome of generalized lipodystrophy is unknown. The impetus to the development of the disease can serve a variety of adverse factors (infection, craniocerebral trauma, surgical intervention, pregnancy and childbirth, various kinds of stressful situations). In some cases, the cause of the disease can not be established. There are cases of both congenital and acquired syndrome of generalized lipodystrophy. The disease occurs mainly in women and is manifested in most cases up to 40 years.

Most researchers adhere to the "central" theory of the origin of the syndrome of generalized lipodystrophy. The results of a series of scientific studies conducted in 1963-1972 convincingly testify to this theory. LH Louis et al .. These authors isolated from the urine of patients suffering from the syndrome of generalized lipodystrophy, a protein substance that, when administered systematically to experimental animals, caused a clinical picture of the disease, and with a single injection of hypertriglyceridemia, hyperglycaemia and hyperinsulinaemia. According to the authors, this substance has a pronounced fat-mobilizing effect and has pituitary origin.

Causes and pathogenesis of generalized lipodystrophy

Symptoms of generalized lipodystrophy. The leading symptom of the syndrome of generalized lipodystrophy is complete or partial disappearance in patients with the subcutaneous fat layer. On this basis, there are 2 clinical forms of generalized lipodystrophy: total and partial.

The total form of generalized lipodystrophy is characterized by the disappearance of subcutaneous fat from the face and all other parts of the body, often there is protrusion of the navel. In the partial form, the subcutaneous adipose tissue disappears mainly from the trunk and limbs, but not from the face, and in some patients there is even an increase in subcutaneous fat on the face and in the supraclavicular areas. However, in both forms of generalized lipodystrophy, very definite, similar metabolic disturbances with the same end results in changing carbohydrate and lipid metabolism are revealed. The main ones are insulin resistance, hyperinsulinemia, hyperglycemia, hyperlipidemia. In some cases, not only a violation of glucose tolerance, but also diabetes mellitus. The disease can occur at any age: in children and in the elderly.

Symptoms of generalized lipodystrophy

Diagnosis of generalized lipodystrophy. The diagnosis of the syndrome of generalized lipodystrophy is established on the basis of the characteristic appearance of patients (complete absence of subcutaneous fatty tissue or its specific redistribution with excessive development in the face and neck and disappearance on the trunk and extremities, hypertrophy of skeletal muscles, signs of acromegaly, hypertrichosis) and headache complaints , pain and heaviness in the right hypochondrium, violation of the menstrual cycle, hirsutism.

Diagnosis 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, promoted 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 the concentration of insulin 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.

Treatment of generalized lipodystrophy

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