Scintigraphic abnormalities in glycogen storage disease.
Fifteen patients with glycogen-storage disease type 1 (von Gierke's disease) were evaluated by serial scintigraphy, with a clearly recognizable pattern of an enlarged liver with diminished radionuclide accumulation, splenomegaly with considerably increased uptake and renomegaly. In seven of these patients with GSD-1 scintigraphy demonstrated focal defects of varying size. Small or stable defects suggest benign hepatic adenomata, whereas malignant change occurred in growing large lesions. The potential malignant end-point of hepatic-cell carcinoma in GSD-1 warrants careful serial liver scintigraphy with scintiangiography on a routine basis.
D000236:Adenoma / Q000150:complications / Q000000981:diagnostic imaging; D000293:Adolescent; D000328:Adult; D006528:Carcinoma, Hepatocellular / Q000150:complications / Q000000981:diagnostic imaging; D002648:Child; D002675:Child, Preschool; D003102:Colloids; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000150:complications / Q000000981:diagnostic imaging*; D006529:Hepatomegaly / Q000000981:diagnostic imaging; D006801:Humans; D007223:Infant; D007674:Kidney Diseases / Q000000981:diagnostic imaging; D008113:Liver Neoplasms / Q000150:complications / Q000000981:diagnostic imaging; D008297:Male; D011877:Radionuclide Imaging; D013163:Splenomegaly / Q000000981:diagnostic imaging; D013455:Sulfur; D013667:Technetium
D003102:Colloids; D013455:Sulfur; D013667:Technetium