Skip to main content
Journal cover image

Resection of hepatocellular adenoma in patients with glycogen storage disease type Ia.

Publication ,  Journal Article
Reddy, SK; Kishnani, PS; Sullivan, JA; Koeberl, DD; Desai, DM; Skinner, MA; Rice, HE; Clary, BM
Published in: J Hepatol
November 2007

BACKGROUND/AIMS: Because dietary modifications have prolonged the life expectancy of patients with glycogen storage disease type Ia (GSD Ia), the incidence of hepatocellular adenoma (HCA) to carcinoma (HCC) transformation is increasing. The objective of this retrospective study is to assess the safety and effectiveness of HCA resection in GSD Ia patients. METHODS: Clinicopathologic, peri-operative, and long-term data were reviewed from patients who underwent HCA resection. Comparisons were made with Fisher's exact, Mann-Whitney U, and log-rank tests; survival was estimated with Kaplan-Meier analysis. RESULTS: From 1998 to 2006, 38 patients underwent HCA resection. Seven (22%) had GSD Ia. Post-operative mortality occurred in one GSD Ia patient. GSD Ia patients had greater morbidity (86% vs. 20%) and shorter time to adenoma progression (median 23 months vs. not yet reached) after partial hepatectomy compared to the general population (p<0.05). Six GSD Ia patients had no evidence of HCC and recovered after resection without long-term morbidity. Three GSD Ia patients underwent liver transplantation 77, 32, and 23 months after adenoma resection. CONCLUSIONS: Despite substantial morbidity, partial hepatectomy is feasible in GSD Ia patients and is an effective intermediate step in the prevention of HCC until definitive treatment with liver transplantation.

Duke Scholars

Published In

J Hepatol

DOI

ISSN

0168-8278

Publication Date

November 2007

Volume

47

Issue

5

Start / End Page

658 / 663

Location

Netherlands

Related Subject Headings

  • Treatment Outcome
  • Survival Rate
  • Retrospective Studies
  • Middle Aged
  • Male
  • Liver Transplantation
  • Liver Neoplasms
  • Liver
  • Humans
  • Glycogen Storage Disease Type I
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Reddy, S. K., Kishnani, P. S., Sullivan, J. A., Koeberl, D. D., Desai, D. M., Skinner, M. A., … Clary, B. M. (2007). Resection of hepatocellular adenoma in patients with glycogen storage disease type Ia. J Hepatol, 47(5), 658–663. https://doi.org/10.1016/j.jhep.2007.05.012
Reddy, Srinevas K., Priya S. Kishnani, Jennifer A. Sullivan, Dwight D. Koeberl, Dev M. Desai, Michael A. Skinner, Henry E. Rice, and Bryan M. Clary. “Resection of hepatocellular adenoma in patients with glycogen storage disease type Ia.J Hepatol 47, no. 5 (November 2007): 658–63. https://doi.org/10.1016/j.jhep.2007.05.012.
Reddy SK, Kishnani PS, Sullivan JA, Koeberl DD, Desai DM, Skinner MA, et al. Resection of hepatocellular adenoma in patients with glycogen storage disease type Ia. J Hepatol. 2007 Nov;47(5):658–63.
Reddy, Srinevas K., et al. “Resection of hepatocellular adenoma in patients with glycogen storage disease type Ia.J Hepatol, vol. 47, no. 5, Nov. 2007, pp. 658–63. Pubmed, doi:10.1016/j.jhep.2007.05.012.
Reddy SK, Kishnani PS, Sullivan JA, Koeberl DD, Desai DM, Skinner MA, Rice HE, Clary BM. Resection of hepatocellular adenoma in patients with glycogen storage disease type Ia. J Hepatol. 2007 Nov;47(5):658–663.
Journal cover image

Published In

J Hepatol

DOI

ISSN

0168-8278

Publication Date

November 2007

Volume

47

Issue

5

Start / End Page

658 / 663

Location

Netherlands

Related Subject Headings

  • Treatment Outcome
  • Survival Rate
  • Retrospective Studies
  • Middle Aged
  • Male
  • Liver Transplantation
  • Liver Neoplasms
  • Liver
  • Humans
  • Glycogen Storage Disease Type I