Five patients had GSD1a [four harboring c.247C > T (p.Arg83Cys), one harboring c.620del (p.Lys207Argfs*26)] associated with poor metabolic control, severe hypoglycemia and growth failure (median age of 5 years; range: 0.5–5 years), and one patient with GSD IV was transplanted at the age of 2 years because of worsening portal hypertension. This evidence concerns the gene G6PC1 and liver disorder.