Reasons | Suspicious symptomes | Principles of LD treatment
Reasons for the development of LD
- mutations (changes) in genes leading to impaired synthesis, differentiation, and premature death of fat cells (adipocytes)
- autoimmune disorders
- the reasons for the development of some forms of LDM have not yet been clarified
Lipodystrophies are hereditary or acquired according to the degree of loss of adipose tissue, LD are divided into:
- generalized LD (complete loss of adipose tissue, more common in early childhood)
- partial LD (partial loss of adipose tissue, more often in adolescence and adults)
Suspicious symptomes
In what cases can LD be suspected:
- complete or partial loss of subcutaneous fat from the first months of life (with congenital form) or during adolescence (with acquired form)
- pronounced relief of skeletal muscles, increased venous pattern on the limbs
- Diabetes mellitus with severe insulin resistance, ketosis resistance, requiring high doses of insulin
- disorders of fat metabolism: high levels of triglycerides in the blood, frequent pancreatitis
- increased hepatic transaminases (ALT, AST), an increase in liver size, signs of fatty hepatitis, steatohepatitis
- in girls, manifestations of hyperandrogenism are possible: an increase in the clitoris, hirsutism, menstrual irregularities, polycystic ovary syndrome
- family history: similar appearance or loss of PFA in relatives
In case of suspicion of LD, an endocrinologist decides on the need for a molecular genetic study. Genetic testing makes it possible to clarify the type of LD, predict the course of the disease, and determine the tactics of treatment for such patients.
Principles of LD treatment
- Following a strict diet of less than 30% fat / day
- Regular physical activity
- Compensation of carbohydrate metabolism: metformin, high doses of insulin, thiazolidinediones (with partial LD, in patients over 18 years of age)
- Lipid-lowering therapy: fibrates, HMG-CoA reductase inhibitors
- Recombinant human leptin (Metreleptin) can be used to treat generalized LD.