Liver transplantation in glycogen storage disease type Ib: The role of SGLT2 inhibitors

Simona Murko; Manuela Peschka; Konstantinos Tsiakas; Sebastian Schulz-Jürgensen; Uta Herden; René Santer

doi:10.1016/j.ymgmr.2023.100977

Liver transplantation in glycogen storage disease type Ib: The role of SGLT2 inhibitors

Mol Genet Metab Rep. 2023 May 4:35:100977. doi: 10.1016/j.ymgmr.2023.100977. eCollection 2023 Jun.

Authors

Simona Murko¹, Manuela Peschka¹, Konstantinos Tsiakas¹, Sebastian Schulz-Jürgensen¹, Uta Herden², René Santer¹

Affiliations

¹ Department of Pediatrics, University Medical Center Eppendorf, Hamburg, Germany.
² Department of Transplant Surgery, University Medical Center Eppendorf, Hamburg, Germany.

Abstract

We report on liver transplantation in two patients with GSD Ib on treatment with empagliflozin. The use of this SGLT2 inhibitor resulted in a marked decrease of 1,5-anhydroglucitol which has an important role in the development of neutropenia in this condition. As intended, this caused a significant rise of neutrophil numbers. Liver transplantation alone did not produce the desired effect and our observation argues for continuing SGLT2 inhibitor treatment after transplantation.

Keywords: Empagliflozin; GSD1b; Glycogen storage disease Ib; Liver transplantation; SGLT2 inhibitor.