Abstract
Patients with liver glucose-6-phosphatase deficiency are at risk for developing hepatomata in adult life. Investigations during adolescence and early adult life indicate that these patients may have discrete filling defects as well as depressed isotope uptake on liver scan with technitium sulfur colloid but only mild abnormalities of liver related serum chemistries (Table). On hepatic anglograms in two patients, the filling defects appeared as vascular masses consistent with primary or metastatic malignancy; biopsy showed increased glycogen content but no changes of malignancy.
Other storage disorders affecting the liver which have been investigated to date have not shown similar abnormalities on liver scan, nor do they have the tendency for developing malignancy in later life.
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Howell, R., Stevenson, R., Phyliky, R. et al. PSEUDOTUMOR HEPATIS IN TYPE I GLYCOGEN STORAGE DISEASE (VON GIERKE'S). Pediatr Res 8, 433 (1974). https://doi.org/10.1203/00006450-197404000-00560
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DOI: https://doi.org/10.1203/00006450-197404000-00560