Glycogen storage disease type I surgery: Difference between revisions
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{{Glycogen storage disease type I}} | {{Glycogen storage disease type I}} | ||
{{CMG}}; {{AE}} | {{CMG}}; {{AE}} {{Anmol}} | ||
==Overview== | ==Overview== | ||
Revision as of 15:52, 20 November 2017
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Anmol Pitliya, M.B.B.S. M.D.[2]
Overview
Liver transplantation is the final treatment for patients with metabolic disease associated associated with GSD type 1.
Indications
Indications for liver transplantation include:[1]
- Patients with multifocal lesions
- Growing lesions that do not regress with improved dietary regimens
- Patients who do not have evidence of distant metastatic disease.
Surgery
Liver transplantation
- Liver transplantation may improve the survival of patients with glycogen storage disease type 1.
- Survival rates of patients after liver transplantation are:[2]
- 1 year survival rate of 82%
- 5 year survival rate of 76%
- 10 year survival rate of 64%
- There is resolution of metabolic derangements (correction of hypoglycemia, lactic acidosis, hyperuricemia, and hyperlipidemia) after liver transplantation in patients with GSD type 1.[3]
- "Model for end-stage liver disease” (MELD) score is used to govern priority for liver transplantation.[4]
- The score range between 6 and 40 and is calculated using a logarithmic assessment of three objective and reproducibe variables including:
- Total serum bilirubin
- Creatinine concentrations
- International normalized ratio
- As hepatic abnormalities in GSD type 1 are due to single-gene and cell-autonomous defect, the recurrence of primary liver disease in the transplanted allograft in not possible.
References
- ↑ Kishnani, Priya S.; Austin, Stephanie L.; Abdenur, Jose E.; Arn, Pamela; Bali, Deeksha S.; Boney, Anne; Chung, Wendy K.; Dagli, Aditi I.; Dale, David; Koeberl, Dwight; Somers, Michael J.; Burns Wechsler, Stephanie; Weinstein, David A.; Wolfsdorf, Joseph I.; Watson, Michael S. (2014). "Diagnosis and management of glycogen storage disease type I: a practice guideline of the American College of Medical Genetics and Genomics". Genetics in Medicine. doi:10.1038/gim.2014.128. ISSN 1098-3600.
- ↑ 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 (3): 432–6. doi:10.1111/j.1399-0012.2011.01549.x. PMID 22066793.
- ↑ O'Leary JG, Lepe R, Davis GL (2008). "Indications for liver transplantation". Gastroenterology. 134 (6): 1764–76. doi:10.1053/j.gastro.2008.02.028. PMID 18471553.
- ↑ Wiesner R, Edwards E, Freeman R, Harper A, Kim R, Kamath P; et al. (2003). "Model for end-stage liver disease (MELD) and allocation of donor livers". Gastroenterology. 124 (1): 91–6. doi:10.1053/gast.2003.50016. PMID 12512033.