PHOSPHORYLASE-B KINASE-DEFICIENCY GLYCOGENOSIS WITH CIRRHOSIS OF THE LIVER

We describe an Arab girl with complete absence of phosphorylase b kina se activity in the liver, symptomatic hypoglycemia, and persistently e levated serum aminotransferase values whose symptoms did not lessen wi th age; sequential liver biopsies showed progression to cirrhosis, Cir rhosis could not be ascribed to any other known cause, We conclude tha t type IX glycogenosis is not always associated with a benign outcome.