Nutritional intervention in berardinelli syndrome or generalized congenital lipoatrophy: case report

Abstract

Introduction and Objectives: Generalized Congenital Lipoatrophy is a rare, autosomal recessive, low prevalence genetic disorder whose diagnosis accompanies several metabolic and systemic alterations, most notably diabetes mellitus, systemic arterial hypertension, hepatic steatosis, dyslipidemia and renal failure. The main objective is to report how the Nutritional Interference in Generalized Congenital Lipoatrophy improves the health status of patients. Materials and Methods: This is an exploratory case study with a qualitative approach, carried out in the city of Juazeiro do Norte-CE. From the reading and interpretation of the medical record, information and menu application were obtained to improve biochemical rates. Discussion: Within the many metabolic changes installed, characteristics of the syndrome, dietary intervention is a form of treatment. Together with the practice of physical activity and medications, they improved the quality of life and, consequently, the life expectancy of the patients. The achievement of nutritional goals in treatment is variable and depends on adherence to diet. Dietary guidelines recommend: restriction of total energy intake, saturated fat and simple carbohydrates, giving preference to the consumption of complex carbohydrates, soluble fibers, medium chain triglycerides and unsaturated fatty acids and drug therapy. Results and Conclusion: Regarding the literature on the most adequate diet therapy in Generalized Congenital Lipodystrophy, the relevance of the topic is still relevant, since the use of adequate diet therapy improves the patient's metabolic status, improving the biochemical rates and the expectation of life of the carriers.