Re: Glycogen hepatopathy in a 13-year-old male with type 1 diabetes
From the Paediatrics Al-Kindy College of Medicine, Baghdad University
Ann Saudi Med 2012; 32(4): 437
To the Editor: Great thanks are due to Aljabri et al1 for expanding pediatricians’ alertness on the glycogen hepatopathy in children with type 1 diabetes mellitus (T1DM). Though the studied patient had a 3-year history of T1DM, it should be noted that hepatic glycogenosis could occur at any stage of T1DM and might even be one of its earliest manifestations, together with those classically reported at the onset of T1DM. Since long-standing hyperglycemia and overinsulinisation are metabolic prerequisites for hepatic glycogen storage, liver glycogenosis should be expected to be not uncommon during the first phases of T1DM, especially in the cases that are initially treated with supraphysiological insulin doses.2 Also, it should be noted that the studied patient needs to be kept under regular follow-up as recurrent hepatomegaly and elevated transaminases are not uncommon. Should recurrence occur, genetic analysis of the liver glycogen phosphorylase gene is suggested.3
1. Aljabri KS, Bokhari SA, Fageeh SM, Alharbi AM, Abaza MA. Glycogen hepatopathy in a 13-year-old male with type 1 diabetes. Ann Saudi Med 2011; 31: 424-7.
2. Carcione L, Lombardo F, Messina MF, Rosano M, De Luca F. Liver glycogenosis as early manifestation in type 1 diabetes mellitus. Diabetes Nutr Metab 2003; 16: 182-4.
3. Tomihira M, Kawasaki E, Nakajima H, Imamura Y, Sato Y, Sata M, et al. Intermittent and recurrent hepatomegaly due to glycogen storage in a patient with type 1 diabetes: genetic analysis of the liver glycogen phosphorylase gene (PYGL). Diabetes Res Clin Pract 2004; 65: 175-82.