Hypoglycemia Associated with Von Gierke’s Disease Reversal with Liver Transplant

Presentation Number: MON 596
Date of Presentation: April 3rd, 2017

Mahnaz Mellati*, Eun Kyung Koh and Jing H. Chao
University of Washington, Seattle, WA

Abstract

Von Gierke’s disease, or glycogen storage disease (GSD) type Ia, is a rare metabolic disorder (1/100,000) caused by glucose-6-phosphatase deficiency mainly in the liver and kidneys. Affected patients develop major metabolic derangements, including life-threatening hypoglycemia, hepatic adenomas and renal insufficiency. Approximately 10% of patients develop hepatocellular carcinoma (HCC).

We report a patient who was diagnosed with von Gierke’s disease in infancy and developed hypoglycemia requiring frequent feedings and continuous D50 (50% dextrose) infusion. He was found to have multiple hepatic adenomas in early teenage. Despite good compliance with his diet, he had at least one severe hypoglycemic episode yearly. At the age of 31 years, he developed malignant transformation to HCC and underwent liver transplantation. His weight was 75kg and BMI of 25kg/m2. Supraphysiologic steroid doses were used peri-operatively. On operative day and POD1, he received methylprednisolone 1000 mg, POD2 methylprednisolone 75 mg, POD3 methylprednisolone 70 mg, and subsequently prednisone 20 mg daily. Two hours into his transplant surgery, his blood glucose (BG) rose from 127 mg/dl to 226 mg/dl, and IV insulin was initiated per hospital protocol. He remained on IV insulin until POD4 when insulin was discontinued. Daily insulin requirement was 163 units, 103 units, 57 units, and 2.5 units from POD0-4, respectively, with minimal dietary intake on POD0-3. Since POD5, he remained euglycemic without dietary support or insulin despite supraphysiologic steroid.

Liver transplant is a curative treatment for GSD Ia patients with malignant transformation of hepatic adenomas and life-threatening hypoglycemia. Peri-operative use of steroid followed by a rapid taper leads to very high and rapidly-changing insulin requirement. Therefore, we recommend using IV insulin guided by well-established algorithms and close monitoring of BG, followed by transitioning off or to subcutaneous insulin. Euglycemia may be restored post-operatively when steroid dose is reduced sufficiently.

 

Nothing to Disclose: MM, EKK, JHC