Table 3:

Characteristics of 5 Inuit children with glycogen storage disease type IIIa at last follow-up visit

PatientFasting tolerance, hCreatine kinase, U/L (normal 53–310)Complications
19 without UCCS1633Cirrhosis, portal hypertension and gastroesophageal varices; stable muscle weakness
27 with UCCS2557Slight clinical muscle involvement
312 without UCCS2755Right ventricular hypertrophy, long corrected QT interval; clinical muscle involvement
44 with UCCS2093Mild clinical muscle involvement
57 with UCCS1225No clinical muscle involvement
  • Note: UCCS = uncooked cornstarch.