Phosphorylase b kinase deficiency glycogenosis with cirrhosis of the liver

Amir F. Kagalwalla, Yasmeen A. Kagalwalla, Sulaiman Al Ajaji, Waldemer Gorka, M. Ashraf Ali

Research output: Contribution to journalArticlepeer-review

18 Scopus citations


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. (J PEDIATR 1995;127:602-5).

Original languageEnglish (US)
Pages (from-to)602-605
Number of pages4
JournalThe Journal of pediatrics
Issue number4
StatePublished - Oct 1995

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health


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