Assessment of Nutritional Status and Glycemic Control in Patients with Hepatic Glycogenosis

Abstract

ietary intervention is the corner stone of treatment of GSD. We reviewed 40 patients diagnosed with GSD, analyze their medical data, nutritional assessment and interventions Results: These 40 patients included 11 cases with GSD I and 29 cases with GSD III. Their ages range from 1 to17 years old. Biochemical abnormalities: Hypoglycemia, Hyperlipidemia, Hyperuricemia were common in GSD I patients. They were also present among patients with GSD III Nutritional management condition: All the patients received nutritional assessment, dietary modulation and nutritional support. UCCS was prescribed every 4-6 hours for patients with GSD I and GSD III respectively, in addition to a high protein intake of 3 gram/kg/day in patients with type III GSD and received dietary guidance and modulation. Anthropometric measurements were done before and after dietary modulation and there were improvement in weight, height, BMI and MUAC (for age) as measured by Z score The mean follow up duration was 6 months for patients with GSD types I and III respectively. All the patients that were compliant to diet modification reported alleviated symptoms of hypoglycemia and decrease the frequency of hypoglycemic attacks.