Glycogen storage disease type III surgery
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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 indicated for patients developing histologiocal evidence of cirrhosis. There is resolution of metabolic derangements after liver transplantation in patients with glycogen storage disease type 3 (GSD 3). As hepatic abnormalities in GSD 3 are due to single-gene and cell-autonomous defect, the recurrence of primary liver disease in the transplanted allograft in not possible. However, skeletal manifestations or cardiac manifestations does not correct even after liver transplantation.
Indications
- Indications for liver transplantation in glycogen storage disease type 3 include:[1]
- Patients developing histological evidence of cirrhosis.
Surgery
- There is resolution of metabolic derangements after liver transplantation in patients with GSD type 3.[2]
- "Model for end-stage liver disease” (MELD) score is used to govern priority for liver transplantation.[3]
- The score range between 6 and 40 and is calculated using a logarithmic assessment of three objective and reproducible variables including:
- Total serum bilirubin
- Creatinine concentrations
- International normalized ratio
- As hepatic abnormalities in GSD type 3 are due to single-gene and cell-autonomous defect, the recurrence of primary liver disease in the transplanted allograft in not possible.
- However, skeletal manifestations or cardiac manifestations does not correct even after liver transplantation.
References
- ↑ 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 (2): 137–45. doi:10.1111/j.1399-3046.2007.00803.x. PMID 18307661.
- ↑ Iyer SG, Chen CL, Wang CC, Wang SH, Concejero AM, Liu YW; et al. (2007). "Long-term results of living donor liver transplantation for glycogen storage disorders in children". Liver Transpl. 13 (6): 848–52. doi:10.1002/lt.21151. PMID 17539004.
- ↑ 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.