Abstract
Two cases of type III glycogen storage disease are reported in adults; the occurrence of cirrhosis in one case illustrates the potential development of chronic liver disease in this condition. The other was the oldest patient with this condition found in a review of published reports. Electron microscopy of peripheral blood leucocytes to demonstrate excess glycogen was found to be a quick and useful aid to diagnosis. Histology of these adult cases showed a distribution of hepatocyte vacuolation which has not been previously recorded.
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- Fernandes J., Koster J. F., Grose W. F., Sorgedrager N. Hepatic phosphorylase deficiency. Its differentiation from other hepatic glycogenoses. Arch Dis Child. 1974 Mar;49(3):186–191. doi: 10.1136/adc.49.3.186. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Garancis J. C., Panares R. R., Good T. A., Kuzma J. F. Type 3 glycogenosis. A biochemical and electron microscopic study. Lab Invest. 1970 May;22(5):468–477. [PubMed] [Google Scholar]
- Hug G. Glycogen storage diseases. Birth Defects Orig Artic Ser. 1976;12(6):145–175. [PubMed] [Google Scholar]
- Levin S., Moses S. W., Chayoth R., Jagoda N., Steinitz K. Glycogen storage disease in Israel. A clinical, biochemical and genetic study. Isr J Med Sci. 1967 May-Jun;3(3):397–410. [PubMed] [Google Scholar]
- McAdams A. J., Hug G., Bove K. E. Glycogen storage disease, types I to X: criteria for morphologic diagnosis. Hum Pathol. 1974 Jul;5(4):463–487. doi: 10.1016/s0046-8177(74)80024-9. [DOI] [PubMed] [Google Scholar]
- Senior B., Sadeghi-Nejad A. The glycogenoses and other inherited disorders of carbohydrate metabolism. Clin Perinatol. 1976 Mar;3(1):79–98. [PubMed] [Google Scholar]
- Starzl T. E., Putnam C. W., Porter K. A., Halgrimson C. G., Corman J., Brown B. I., Gotlin R. W., Rodgerson D. O., Greene H. L. Portal diversion for the treatment of glycogen storage disease in humans. Ann Surg. 1973 Oct;178(4):525–539. doi: 10.1097/00000658-197310000-00015. [DOI] [PMC free article] [PubMed] [Google Scholar]