[Nutritional management of glycogenosis]

Three cases of glycogenosis are presented. They were recognized by clinical evidence and hepatic biopsy specimens. Dietary treatment with a normocaloric diet and raw corn starch every 6 hours was given. In cases 1 and 2 hepatic size returned to normal and they showed an initial catch up growth without signs of renal disease or other complications. Case 3 showed persistent hepatomegaly, normal growth pattern and no hypoglycemic episodes independently of nutritional management. These cases represent our initial experience on nutritional intervention in glycogenosis, even though enzymatic tests were not done, which are considered essential for optimal diagnosis and nutritional therapy of glycogen storage disease.