Simultaneous liver-kidney transplantation for glycogen storage disease type IA (von Gierke's disease)

Transplantation Proceedings
F PanaroU Valente

Abstract

Glycogen storage disease type Ia (GSDIa) is due to the deficiency of glucose-6-phosphatase activity in the liver, kidney, and intestine. Although significant progress has been achieved in the management of patients with GSDIa, complications still emerge. The potential for development of liver adenomatosis and kidney failure makes these patients candidates for simultaneous liver-kidney transplantation (SLKT). Herein, we describe such a transplantation in a patient affected by this rare storage disease. A 25-year-old female patient with GSDIa developed hepatic adenoma and kidney failure despite dietary therapy. The patient underwent an SLKT from a cadaveric donor. The operative time was 8 hours without hemotransfusion. Only a transitory lactic acidosis was observed. Laboratory results normalized on postoperative day 7. The patient was discharged on postoperative day 9. After 4 months, the patient is in good condition with well-functioning kidney and liver allografts. Patients with end-stage renal disease secondary to GSDIa should be considered for SLKT, especially when the disease is in an early stage.

References

Oct 4, 2003·Liver Transplantation : Official Publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society·Gülçin DemirciBjoern Nashan

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Citations

May 7, 2013·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Hannu Jalanko, Mikko Pakarinen
Apr 11, 2014·Orphanet Journal of Rare Diseases·Susanna J B BoersSabine A Fuchs
May 24, 2011·Orphanet Journal of Rare Diseases·Roseline FroissartPhilippe Labrune
Apr 9, 2008·Journal of Hepato-biliary-pancreatic Surgery·Akihiko OshitaToshimasa Asahara
Apr 11, 2014·Kidney International·Julie ClarFabienne Rajas
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Jan 13, 2018·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Ryszard Grenda, Piotr Kaliciński
Nov 18, 2020·World Journal of Hepatology·Mladen KnotekAnna Mrzljak
Mar 21, 2009·Transplantation Reviews·Srinivas P ChavaNigel D Heaton

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