B Complex Vitamins Profile in Patients with Hepatic Glycogen Storage Disease and its Possible Determinants

Abstract Hepatic glycogen storage diseases (GSD) are a group of rare hereditary metabolic diseases characterized by abnormalities in enzymes that regulate glycogen synthesis or degradation. Treatment is essentially dietary, with frequent administration of raw uncooked cornstarch (UCCS) to avoid hypoglycemia, in addition to the restriction of sucrose, fructose, and lactose. Nutritional deficiencies in these patients are believed to result from dietary restrictions that exclude key sources of essential vitamins. This study aimed to investigate serum levels and adequacy of dietary intake of B vitamins in patients with GSD. This is a cross-sectional study, with patients over 5 years old, diagnosed with GSD and undergoing treatment with UCCS. Clinical data were collected, in addition to the dosage of B vitamins and a 3-day food record. Fifteen patients were included, six of them reported daily use of multivitamins. Serum dosage showed that all B complex vitamins, except for vitamin B2, were within reference values. The adequacy of dietary intake of vitamins was at least 78%, except for vitamin B9. There was no correlation between dosage and vitamin intake.