Phosphorylase b kinase deficiency glycogenosis with cirrhosis of the liver.
case_report · Level V
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- Record sourced from PubMed, PMID 7562285.
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Abstract
We describe an Arab girl with complete absence of phosphorylase b kinase activity in the liver, symptomatic hypoglycemia, and persistently elevated serum aminotransferase values whose symptoms did not lessen with age; sequential liver biopsies showed progression to cirrhosis. Cirrhosis could not be ascribed to any other known cause. We conclude that type IX glycogenosis is not always associated with a benign outcome.
Medical subject headings
- Glycogen Storage Disease
- Liver
- Liver Cirrhosis
- Phosphorylase Kinase
- Phosphorylase b