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Regression of Hepatocellular Adenomas with Strict Dietary Therapy in Patients with Glycogen Storage Disease Type I

  • Richard D. Beegle
  • Laurie M. Brown
  • David A. Weinstein
Research Report
Part of the JIMD Reports book series (JIMD, volume 18)

Abstract

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.

Keywords

Triglyceride Level Metabolic Control Glycogen Storage Disease Glycogen Storage Disease Type Hepatocellular Adenoma 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Abbreviations

CT

Computed tomography

GSD

Glycogen storage disease

HCA

Hepatocellular adenoma

MRI

Magnetic resonance imaging

Notes

Acknowledgement

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.

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Copyright information

© SSIEM and Springer-Verlag Berlin Heidelberg 2014

Authors and Affiliations

  • Richard D. Beegle
    • 1
  • Laurie M. Brown
    • 2
  • David A. Weinstein
    • 2
  1. 1.Department of RadiologyUniversity of Florida College of MedicineGainesvilleUSA
  2. 2.Glycogen Storage Disease Program, Division of Pediatric EndocrinologyUniversity of Florida College of MedicineGainesvilleUSA

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