Simultaneous Liver-Kidney Transplantation from a Deceased Donor for Glycogen Storage Disease Type Ia: A Case Report

Glycogen storage disease type Ia (GSD Ia) is an indication for simultaneous liver-kidney transplantation (SLKT) due to multiple hepatocellular adenomas and chronic renal failure. In this report, we describe a deceased-donor SLKT in a patient with GSD Ia who presented with hypoglycemic attacks, progressive multiple hepatocellular adenomas, and chronic renal failure.

The patient was diagnosed with hepatomegaly at the age of 1 year and 6 months, with a diagnosis of GSD Ia, and was started on a special diet. She had been aware of liver tumors since 24 years of age. At 29 years of age, she underwent left lateral segmentectomy and radiofrequency ablation for multiple liver tumors. She began to suffer from renal dysfunction around 39 years of age, and then at 40 years of age, she was diagnosed with chronic renal failure and therefore was started on hemodialysis. She began having recurrent hypoglycemic attacks after the introduction of dialysis. Due to repeated hypoglycemic attacks, multiple hepatocellular adenomas, and chronic renal failure, she was deemed eligible for SLKT and thus was registered for deceased-donor SLKT at 41 years of age. She underwent SLKT at 46 years of age. The liver graft weight was 1060 g, and the graft-to-recipient body weight ratio was 2.41. The kidney graft weight was 170 g. The weight of the excised liver was 2710 g. Although no malignant findings were observed in the excised liver, multiple hepatocellular adenomas were identified. She was able to urinate independently immediately after the SLKT and was able to discontinue dialysis immediately afterward. Immunosuppressive therapy was initiated with basiliximab, ciclosporin A, methylprednisolone, and mycophenolate mofetil (MMF). However, due to abdominal pain and liver dysfunction caused by MMF, she was switched to mizoribine on POD 46, and her abdominal pain improved thereafter. She progressed well and was discharged on POD 49. Her liver and kidney functions remained good approximately 2 years after the SLKT.

Adult patients with GSD Ia require transplantation due to hypoglycemic attacks, multiple liver tumors, and chronic renal failure.