EFFECT OF A HIGH-CARBOHYDRATE DIET ON THE GLYCEMIC AND LIPIDIC PROFILE IN AN ARGENTINE POPULATION WITH ACUTE INTERMITTENT PORPHYRIA

OLIVERI LEDA; CALCAGNO MARIA; GEREZ ESTHER; MORA SANDRA; BUZALEH ANA MARIA; ROSSETTI MARIA V; MEDINA NANCY; PARERA VICTORIA

MILAN

Congreso; INTERNATIONAL CONGRESS PORPHYRINS & PORPHYRIAS 2019; 2019

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). During an acute attack, AIP patients diagnosed in the CIPYP receive an overload of glucose (400/500 g/day) intravenously, folic acid and vitamin B complex. After the remission of the crisis, the treatment consists of diet with high carbohydrate consumption (300 g glucose/day) combined also with folic acid and vitamin B complex. This treatment has been shown to be effective in preventing attacks and/or decreasing its intensity. Aims: To study the influence of a high-carbohydrate diet on the glycemic and lipid profile in a population of AIP patients. Methods: The study we done in AIP individuals (n=33) aged between 19 to 68 years old. Glucose, insulin, cholesterol, triglycerides, low- and high-density lipoproteins and total lipids levels were measured in blood samples. In addition, the levels of ALA, porphobilinogen and total porphyrins in 24-hour urine were determined in all individuals.Result: According to the analysis, no alterations were detected in any of the parameters studied with respect to the reference values. Conclusion: In spite of the high and continuous consumption of carbohydrates, AIP patients did not present alterations in the glycemic or lipid profiles.