Hepatic Adenomata with Type 1 Glycogen Storage Disease

R. Rodney Howell, Roger E. Stevenson, Yoram Ben-Menachem, Robert L. Phyliky, D. H. Berry

Research output: Contribution to journalArticlepeer-review

111 Scopus citations


In older patients with glucose-6-phosphatase deficiency adenomatous nodules develop within the liver parenchyma. Investigation of eight such patients, age 3 to 28 years, using radioisotopic scans, has demonstrated areas of depressed isotope uptake in the liver in all except the one preteenaged child. Three patients were further studied with hepatic angiograms and liver biopsy specimens. A diffuse nodularity, more widespread than apparent on isotopic scans, was demonstrated on angiograms. Although initial histologic study in each case showed adenomatous tissue without evidence of neoplasm, the development of hepatocellular carcinoma in one of our patients and in others from the literature suggests that the nodules may be premalignant.

Original languageEnglish (US)
Pages (from-to)1481-1484
Number of pages4
JournalJAMA: The Journal of the American Medical Association
Issue number13
StatePublished - Sep 27 1976
Externally publishedYes

ASJC Scopus subject areas

  • Medicine(all)


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