title: Liver-spleen scintigraphy in glycogen storage disease (glycogenoses).
abstract: Some forms of glycogen storage disease (GSD) primarily affect the liver, including types I, III, IV, VI, IX and 0. Scanning with Tc-99m sulfur colloid, while not being specific, does reveal some characteristic features. Most experience is with scanning in type I disease, though there are few reports in the literature. Six patients with type I, type III, type IV, and probably type VI disease are presented in this report. GSD should be considered in infants and young children presenting with hepatomegaly and abnormal liver-spleen scans. Sequential imaging is useful in following these patients. When focal defects are present, long term follow-up is indicated to detect hepatocellular adenocarcinoma.
mesh_terms: D000230:Adenocarcinoma / Q000000981:diagnostic imaging; D000293:Adolescent; D000328:Adult; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006008:Glycogen Storage Disease / Q000000981:diagnostic imaging*; D005953:Glycogen Storage Disease Type I / Q000000981:diagnostic imaging; D006009:Glycogen Storage Disease Type II / Q000000981:diagnostic imaging; D006010:Glycogen Storage Disease Type III / Q000000981:diagnostic imaging; D006011:Glycogen Storage Disease Type IV / Q000000981:diagnostic imaging; D006013:Glycogen Storage Disease Type VI / Q000000981:diagnostic imaging; D006801:Humans; D007223:Infant; D008099:Liver / Q000000981:diagnostic imaging*; D008113:Liver Neoplasms / Q000000981:diagnostic imaging; D008297:Male; D011877:Radionuclide Imaging; D013154:Spleen / Q000000981:diagnostic imaging*; D013671:Technetium Tc 99m Sulfur Colloid
chemical_list: D013671:Technetium Tc 99m Sulfur Colloid