The Role of Liver Transplantation in Propionic Acidemia

Liver Transplantation : Official Publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society
Jesús QuinteroR Charco

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 t...Continue Reading

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Citations

Oct 31, 2018·Liver Transplantation : Official Publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society·Ashwin RammohanMohamed Rela
Oct 22, 2019·Journal of Pediatric Gastroenterology and Nutrition·Sophoclis P AlexopoulosJustin Godown
Nov 13, 2019·Liver Transplantation : Official Publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society·Richard CurnockRoshni Vara
Jul 21, 2020·JIMD Reports·Gerard T BerryWilliam L Nyhan
Jun 30, 2019·Genetics in Medicine : Official Journal of the American College of Medical Genetics·Oleg A ShchelochkovCharles P Venditti
Jan 25, 2020·Journal of Pediatric Gastroenterology and Nutrition·Sophoclis P AlexopoulosJustin Godown
Sep 25, 2020·Journal of Pediatric Gastroenterology and Nutrition·Vikrant SoodPatrick J McKiernan
Feb 18, 2021·Journal of Inherited Metabolic Disease·Patrick FornyMatthias R Baumgartner
May 13, 2021·Journal of Investigative Medicine High Impact Case Reports·Luis I RodriguezAna C Mavarez
Jun 25, 2021·World Journal of Transplantation·Jagadeesh MenonMohamed Rela

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