STUDY OF THE GLYCEMIC AND LIPID PROFILE IN AN ARGENTINE POPULATION WITH ACUTE INTERMITTENT PORPHYRIA UNDER A HIGH-CARBOHYDRATE DIET

MORA SANDRA; OLIVERI LEDA; MEDINA NANCY; CALCAGNO MARIA LUJÁN; PARERA VICTORIA; ROSSETTI MARÍA VICTORIA; GEREZ, ESTHER

Mar del Plata

Congreso; LXIV Reunión Anual de la Sociedad Argentina de Investigación Clínica (SAIC); 2019

SAIC

Acute intermittent porphyria (AIP) is a hereditary disorder of heme biosynthesis, characterized by a decrease in the activity of porphobilinogen deaminase enzyme, associated with an increase in the expression of -aminolevulinic acid synthetase 1, first and regulatory enzyme of this pathway, and with the accumulation of the neurotoxic precursor -aminolevulinic acid (ALA). In the event of an acute attack, patients diagnosed at CIPYP with AIP receive an overload of glucose (400/500 g/day) intravenously, folic acid and vitamin B complex, then the treatment is continued for life, with a high-carbohydrate diet (300g/day), folic acid and B complex. According to clinical follow-up, this treatment has proven effective in preventing attacks and / or decreasing their intensity. According to numerous studies, type 1 insulin-like growth factor (IGF1) interacts with insulin to modulate carbohydrate metabolism and its decrease alters lipid metabolism. In our laboratory, 40 % of patients with AIP have low IGF1 values, so the aim was to study the influence of a high-carbohydrate diet on the glycemic and lipid profile in a population of AIP patients with low and normal IGF1 levels. The study was done in AIP individuals (n= 33) aged between 19 to 68 years old. IGF1, glucose, insulin, cholesterol, triglycerides, low- and high-density lipoproteins and total lipids level were measured in blood samples. In addition, the levels of ALA, porphobilinogen and total porphyrins in 24-h urine were quantified. Respect to the lipid and glycemic analysis performed in the subpopulation of AIP patients who had normal or low IGF1, no alterations were detected in any of the studied parameters. In the cohort of AIP patients analyzed, no alterations in glycemic or lipid profiles were observed in AIP patients that receive a high and continuous diet of carbohydrates, independently of IGF1 levels.