Aggressive therapy improves cirrhosis in glycogen storage disease type IX
LA Tsilianidis, LM Fiske, S Siegel, C Lumpkin… - Molecular genetics and …, 2013 - Elsevier
Glycogen storage disease type IX (GSD IX) is described as a benign condition that often
does not require treatment. Most patients with the disease are thought to outgrow the
childhood manifestations, which include hepatomegaly, poor growth, and ketosis with or
without hypoglycemia. Long term complications including fibrosis and cirrhosis have seldom
been reported in the most common subtype, GSD IXα. We present two cases of children with
GSD IXα who had fibrosis at the time of diagnosis in addition to the commonly reported …
does not require treatment. Most patients with the disease are thought to outgrow the
childhood manifestations, which include hepatomegaly, poor growth, and ketosis with or
without hypoglycemia. Long term complications including fibrosis and cirrhosis have seldom
been reported in the most common subtype, GSD IXα. We present two cases of children with
GSD IXα who had fibrosis at the time of diagnosis in addition to the commonly reported …