Clinical Analysis Of 12 Children With Hepatic Glycogen Storage Disease

ObjectiveThis study summarized clinical features of hepatic glycogen storage disease to improve the diagnosis and treatment and decrease misdiagnosis.MethodsRetrospective analysis characteristics of 12 hepatic glycogen storage disease cases in Shengjing Hospital from May,2007 to November,2009.ResultsThe average age for treatment of the 12 cases is 1.43±1.97 years,2 cases were misdiagnosed in the 5 final diagnosed cases at first treatment. Liver dysfunction is the main symptom,4 cases with hypoglycemia and 8 cases with growth retardation. In final diagnosed group,4 cases were screened positive result for epinephrine stimulating test, and in suspected diagnosed group, the positive percentage is 33.3%. The positive percetage of other clinical manifestation and laboratory tests in final diagnosed group is higher than suspected diagnosed group.ConclusionsThe clinical manifestations of hepatic glycogen storage disease are multiple. Therefore, physicians must have sufficient recognition for this disease, and be on the alert for hepatic glycogen storage disease, give a right and prompt diagnosis combined with family history, physical examinations and laboratory findings.