《Table 1Epidemiological, clinical and biochemical data, summarizing the litera-ture regarding patien

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《Glycogenic hepatopathy》

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Data were expressed as mean±SD or number（percentage）.

Hepatomegaly was reported in 103（81.7%）patients，while right upper quadrant tenderness was described in 59（46.8%）.Combined hepatocellular and cholestatic liver enzyme elevation was described in 93%，even though hepatocellular predominance was described.Mean levels were as follows:aspartate aminotransferase（AST）692 U/L，alanine aminotransferase（ALT）465 U/L，gammaglutamyl transferase（GGT）305 U/L，alkaline phosphatase（ALP）365 U/L.Bilirubin was elevated in 15%of the cases where its level was reported，with a maximum level of 2.5 mg/d L.International normalized ratio（INR）was normal in all cases.Median hemoglobin（Hb）A1C level was 11.8%.Our findings are summarized in Table 1，including demographic information，clinical findings and laboratory tests of all the patients as reported on presentation.A comparison between the adults and the pediatric groups can be seen in Table 2.