Regression of Hepatocellular Adenomas with Strict Dietary Therapy in Patients with Glycogen Storage Disease Type I
Hepatocellular adenomas (HCAs) are a common complication in patients with glycogen storage disease type I (GSD I). In this series, we report regression of HCAs in a cohort of patients who achieved metabolic control with strict dietary therapy. A retrospective review of the clinical records for all patients with GSD I was performed at our institution. All available imaging studies were reviewed in patients with reported regression of HCAs in the medical record. The charts of 163 patients with GSD Ia and 42 patients with GSD Ib were reviewed, and HCAs were documented in 47 subjects (43 Ia/4 Ib). After review of all available imaging studies, eight patients met criteria of being followed with both magnetic resonance imaging and ultrasound and were found to show evidence of regression of HCAs. In these individuals, regression of the HCAs occurred once metabolic control was obtained, as determined by decreasing levels of serum triglyceride levels. The average triglyceride level in all patients prior to regression of HCAs was 753 mg/dL (SD ± 293). The average serum triglyceride level in all patients at the time of regression of HCAs was 340 mg/dL (SD ± 164). These findings suggest that strict dietary therapy may cause regression of HCAs. If HCAs are documented in a patient with suboptimal metabolic control, intensive medical therapy may be an alternative to surgical intervention in some individuals.
KeywordsTriglyceride Level Metabolic Control Glycogen Storage Disease Glycogen Storage Disease Type Hepatocellular Adenoma
Glycogen storage disease
Magnetic resonance imaging
This research was supported by philanthropic support provided by the following funds managed through the University of Florida Office of Development: Scott Miller GSD Program Fund and the GSD Dream Fund. This work was also supported in part by the NIH/NCATS Clinical and Translational Science Award UL1 TR000064 granted to the University of Florida.
- Bianchi L (1993) Glycogen storage disease I and hepatocellular tumours. Eur J Pediatr 152(Suppl 1):534–536Google Scholar
- Bröker ME, Ijzermans JN, van Aalten SM, de Man RA, Terkivatan T (2012) The management of pregnancy in women with hepatocellular adenoma: A plea for an individualized approach. Int J Hepatol. doi:10.1155/2012/725735Google Scholar
- Labrune P (2002) Glycogen storage disease type I: indications for liver and/or kidney transplantation. Eur J Pediatr 161(Suppl):S53–S55Google Scholar
- Lee PJ (2002) Glycogen Storage disease type I: pathophysiology of liver adenomas. Eur J Pediatr 161(Suppl 1):S45–S49Google Scholar