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. 2018 Dec;24(12):1736-1745.
doi: 10.1002/lt.25344.

The Role of Liver Transplantation in Propionic Acidemia

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The Role of Liver Transplantation in Propionic Acidemia

Jesús Quintero et al. Liver Transpl. 2018 Dec.

Abstract

Despite optimal medical treatment and strict low-protein diet, the prognosis of propionic acidemia (PA) patients is generally poor. We aim to report our experience with liver transplantation (LT) in the management of PA patients. Six patients with PA received a LT at a mean age of 5.2 years (1.3-7.5 years). The indications for LT were frequent metabolic decompensations in the first 4 patients and preventative in the last 2 patients. Two patients presented hepatic artery thromboses that were solved through an interventional radiologist approach. These patients showed a very high procoagulant state that was observed by thromboelastography. Arterial vasospasm without thrombus was observed in 2 patients during the LT surgery. In order to avoid hepatic artery thrombosis, an arterial conduit from the recipient aorta to the hepatic artery of the donor was used in the fifth patient. After LT, patients presented improvement in propionyl byproducts without complete normalization, but no decompensations have been observed. In conclusion, LT could be a good therapeutic option to improve the metabolic control and the quality of life of PA patients. Improved surgical strategies along with new techniques of interventional radiology allow us to perform the LT minimizing the complications derived from the higher risk of hepatic artery thrombosis.

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Comment in

  • The Role of Liver Transplantation in Propionic Acidemia.
    Rammohan A, Gunasekaran V, Reddy MS, Rela M. Rammohan A, et al. Liver Transpl. 2019 Jan;25(1):176-177. doi: 10.1002/lt.25373. Liver Transpl. 2019. PMID: 30375139 No abstract available.
  • Reply.
    Quintero J, Molera C, Juamperez J, Redecillas S, Meavilla S, Nuñez R, García-Volpe C, Del Toro M, Garcia-Cazorla Á, Ortega J, Segarra Ó, de Carpi JM, Bilbao I, Charco R. Quintero J, et al. Liver Transpl. 2019 Jan;25(1):178-179. doi: 10.1002/lt.25389. Liver Transpl. 2019. PMID: 30472769 No abstract available.

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