Processo de cuidado em nutrição no atendimento ambulatorial de pacientes com síndrome da quilomicronemia familiar

Abstract

Familial Chylomicronemia Syndrome (FCS) is a rare autosomal recessive genetic disease, which is characterized by the accumulation of chylomicrons in the bloodstream. This accumulation is due to a defect in the production and activity of lipoprotein lipase (LPL), resulting in severe hypertriglyceridemia that can reach values ​​10 times higher than the standard. Nutritional therapy is the most effective treatment for controlling the disease. The present work was carried out with the main objective of developing an expected care plan, based on the Nutrition Care Process (NCP), for patients with FCS, in order to optimize assistance, as well as the medical record update and recording process. For this, the NCP Terminology was filtered, reducing the number of terms by more than 90%, moreover, an evolution model for the electronic medical record was produced, structured according to the NCP steps. In addition, other materials were created - a document for recommending supplements, a sheet of nutritional recommendations and a decision-making diagram - to assist the Nutritionist in outpatient care for this population. Therefore, after validation in future studies, the materials developed in this work will be made available free of charge, considering the importance of these instruments for stimulating critical thinking and evidence-based conduct, increasing the quality of nutritional assistance for patients with FCS.