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

Research Report
First Online:
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

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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.

References

  1. Ault GT, Wren SM, Ralls PW, Reynolds TB, Stain SC (1996) Selective management of hepatic adenomas. Am Surg 62:825–829PubMedGoogle Scholar
  2. Bianchi L (1993) Glycogen storage disease I and hepatocellular tumours. Eur J Pediatr 152(Suppl 1):534–536Google Scholar
  3. 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
  4. Cobey FC, Salem RR (2004) A review of liver masses in pregnancy and a proposed algorithm for their diagnosis and management. Am J Surg 187:181–191CrossRefPubMedGoogle Scholar
  5. Coire CI, Qizilbash AH, Castelli MF (1987) Hepatic adenomata in type Ia glycogen storage disease. Arch Pathol Lab Med 111:166–169PubMedGoogle Scholar
  6. Davis MK, Weinstein DA (2008) Liver transplantation in children with glycogen storage disease: controversies and evaluation of the risk/benefit of this procedure. Pediatr Transplant 12:137–145CrossRefPubMedGoogle Scholar
  7. Deodhar A, Brody LA, Covey AM, Brown KT, Getrajdman GI (2011) Bland embolization in the treatment of hepatic adenomas: preliminary experience. J Vasc Interv Radiol 22:795–799CrossRefPubMedGoogle Scholar
  8. Kishnani PS, Chuang TP, Bali D et al (2009) Chromosomal and genetic alteration in human hepatocellular adenomas associated with type Ia glycogen storage disease. Hum Mol Genet 18:4781–4790CrossRefPubMedGoogle Scholar
  9. Kudo M (2001) Hepatocellular adenoma in type Ia glycogen storage disease. J Gastroenterol 35:65–66CrossRefGoogle Scholar
  10. Labrune P (2002) Glycogen storage disease type I: indications for liver and/or kidney transplantation. Eur J Pediatr 161(Suppl):S53–S55Google Scholar
  11. Lee PJ (2002) Glycogen Storage disease type I: pathophysiology of liver adenomas. Eur J Pediatr 161(Suppl 1):S45–S49Google Scholar
  12. Limmer J, Fleig WE, Leupold D, Bittner R, Ditschuneit H, Beger HG (1988) Hepatocellular carcinoma in type I glycogen storage disease. Hepatology 8:531–537CrossRefPubMedGoogle Scholar
  13. Maheshwari A, Rankin R, Segev DL, Thuluvath PJ (2012) Outcomes of liver transplantation for glycogen storage disease: a matched-control study and a review of literature. Clin transplant 26:432–436CrossRefPubMedGoogle Scholar
  14. Matern D, Starzl TE, Arnaout W et al (1999) Liver transplantation for glycogen storage disease types I, III, and IV. Eur J Pediatr 158(Suppl 2):S43–S48CrossRefPubMedCentralPubMedGoogle Scholar
  15. McDaniel JD, Kukreja K, Ristagno RL, Yazigi N, Nathan JD, Tiao G (2013) Radiofrequency ablation of a large hepatic adenoma in a child. J Pediatr Surg 48:E19–E22CrossRefPubMedGoogle Scholar
  16. Parker P, Burr I, Slonim A, Ghishan FK, Greene H (1981) Regression of hepatic adenomas in type Ia glycogen storage disease with dietary therapy. Gastroenterology 81:534–536PubMedGoogle Scholar
  17. Rake JP, Visser G, Labrune P, Leonard JV, Ullrich K, Smit GP (2002a) Glycogen storage disease type 1: Diagnosis, management, clinical course and outcome. Results of the European study on glycogen storage disease type I (ESGSD I). Eur J Pediatr 161(Suppl):S20–S34CrossRefPubMedGoogle Scholar
  18. Rake JP, Visser G, Labrune P, Leonard JV, Ullrich K, Smit GP (2002b) Guidelines for management of glycogen storage disease type I – European study on glycogen storage disease type I (ESGSD I). Eur J Pediatr 161(Suppl):S112–S119CrossRefPubMedGoogle Scholar
  19. Reddy SK, Kishnani PS, Sullivan JA et al (2007) Resection of hepatocellular adenoma in patients with glycogen storage disease type Ia. J Hepatol 47:658–663CrossRefPubMedGoogle Scholar
  20. Reddy SK, Austin SL, Spencer-Manzon M et al (2009) Liver transplantation for glycogen storage disease type Ia. J Hepatol 51:483–490CrossRefPubMedGoogle Scholar
  21. Rhim H, Lim HK, Kim YS, Choi D (2008) Percutaneous radiofrequency ablation of hepatocellular adenoma: initial experience in 10 patients. J Gastroenterol Hepatol 23:e422–e427CrossRefPubMedGoogle Scholar
  22. Wang DQ, Fiske LM, Carreras CT, Weinstein DA (2011) Natural history of hepatocellular adenoma formation in glycogen storage disease type I. J Pediatr 159:442–446CrossRefPubMedCentralPubMedGoogle Scholar
  23. Weinstein DA, Wolfsdorf JI (2002) Effect of continuous glucose therapy with uncooked cornstarch on the long-term clinical course of type IA glycogen storage disease. Eur J Pediatr 161(Suppl):S35–S39CrossRefPubMedGoogle Scholar

Copyright information

© SSIEM and Springer-Verlag Berlin Heidelberg 2014

Authors and Affiliations

  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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