Perioperative management of glucose and lactate homeostasis in paediatric glycogen storage disease type 1a coming for living donor liver transplant

Abstract

Glycogen storage disease (GSD) is a rare inborn autosomal recessive inherited disorder of carbohydrate metabolism. There are multiple types of GSD, out of which GSD type I, III, IV, VI, and IX show liver involvement. Due to a deficiency of glucose-6-phosphatase enzyme in this disorder, glycogen stored in the liver cannot be metabolised, leading to poor tolerance to fasting and increased risk of hypoglycaemia and lactate acidosis. Inability to metabolise glycogen leads to progressive accumulation of glycogen in liver leading to hepatic adenoma (HA) and/or hepatocellular carcinoma (HCC). Liver transplantation (LT) has been proposed as the preferred therapy for these types of GSD, as it helps in correcting the primary hepatic enzyme defect, thereby improving the quality of life and reducing the risk of HCC. Herein we report our experience of perioperative management of paediatric GSD type 1a (Von Gierke's disease) patient undergoing living donor liver transplant (LDLT).