Generalized Lipodystrophy - Information Overview

Generalized lipodystrophy is a little-known disease that can be considered not as a set of individual symptoms, but as a single pathological process with its own patterns and developmental features, although the term “generalized lipodystrophy syndrome” (GLS) is quite acceptable.

The main manifestations of the disease are the disappearance of subcutaneous fat over a large surface area of the body or in certain areas, 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 L. H. Ziegler in 1923, and in 1946 RD Lawrence gave its 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 N. T. Starkova et al. (1972) under the name "hypermuscular lipodystrophy".

Despite the fact that the literature provides data on a small number of patients with generalized lipodystrophy syndrome, clinical experience suggests that in reality this disease is much more common. Poor awareness of doctors complicates early diagnosis of the disease. In addition, generalized lipodystrophy syndrome has long been considered only as a cosmetic defect, and other clinical manifestations were usually not associated with the underlying disease. At the same time, the tendency of the syndrome to progress, the occurrence of such severe complications as liver cirrhosis, myocardial infarction, dynamic cerebrovascular accident in a number of patients of working age, the high frequency of patients with generalized lipodystrophy syndrome seeking gynecological help for infertility, necessitate the development of approaches to the treatment and prevention of this disease.

Causes of generalized lipodystrophy. The cause of generalized lipodystrophy syndrome is unknown. The development of the disease can be triggered by various unfavorable factors (infection, craniocerebral trauma, surgery, pregnancy and childbirth, various types of stressful situations). In some cases, the cause of the disease cannot be determined. There are known cases of both congenital and acquired generalized lipodystrophy syndrome. The disease occurs mainly in women and manifests itself in most cases before the age of 40.

Most researchers adhere to the "central" theory of the origin of generalized lipodystrophy syndrome. This theory is convincingly supported by the results of a series of scientific studies conducted in 1963-1972 by L. H. Louis et al. These authors isolated a protein substance from the urine of patients suffering from generalized lipodystrophy syndrome, which, when systematically administered to experimental animals, caused a clinical picture of the disease, and when administered to humans once, it caused hypertriglyceridemia, hyperglycemia, and hyperinsulinemia. According to the authors, this substance has a pronounced fat-mobilizing effect and is of pituitary origin.

Causes and pathogenesis of generalized lipodystrophy

Symptoms of generalized lipodystrophy. The leading symptom of generalized lipodystrophy syndrome is the complete or partial disappearance of the subcutaneous fat layer in patients. According to this feature, 2 clinical forms of generalized lipodystrophy can be distinguished: 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 with a protruding navel. In the partial form, subcutaneous fat disappears mainly from the trunk, limbs, but not from the face, and some patients even have an increase in subcutaneous fat on the face and in the supraclavicular areas. However, both forms of generalized lipodystrophy reveal quite specific, similar metabolic disorders with the same end results in changing carbohydrate and lipid metabolism. The main ones are insulin resistance, hyperinsulinemia, hyperglycemia, hyperlipidemia. In some cases, not only impaired glucose tolerance develops, 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 generalized lipodystrophy syndrome is established based on the characteristic appearance of patients (complete absence of subcutaneous fat or its specific redistribution with excessive development in the face and neck area and disappearance on the trunk and limbs, hypertrophy of skeletal muscles, signs of acromegaly, hypertrichosis) and complaints of headache, pain and heaviness in the right hypochondrium, menstrual irregularities, hirsutism.

Diagnosis 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.

Treatment of generalized lipodystrophy

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