Recurrence of Acute Intermittent Porphyria After Liver Transplantation
Authors: Hanny Al-Samkari, Arpan A. Patel, Thomas D. Schiano, David J. Kuter
Journal: Annals of Internal Medicine (2019)

Letters18 June 2019Recurrence of Acute Intermittent Porphyria After Liver TransplantationHanny Al-Samkari, MD, Arpan A. Patel, MD, Thomas D. Schiano, MD, and David J. Kuter, MD, DPhilHanny Al-Samkari, MDMassachusetts General Hospital, Harvard Medical School, Boston, Massachusetts (H.A., D.J.K.), Arpan A. Patel, MDIcahn School of Medicine, New York, New York (A.A.P., T.D.S.), Thomas D. Schiano, MDIcahn School of Medicine, New York, New York (A.A.P., T.D.S.), and David J. Kuter, MD, DPhilMassachusetts General Hospital, Harvard Medical School, Boston, Massachusetts (H.A., D.J.K.)Author, Article, and Disclosure Informationhttps://doi.org/10.7326/L18-0623 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail Background: Acute intermittent porphyria (AIP) is a rare indication for liver transplantation, but published cases describe resolution of disease after transplantation, with clinical remission and normalization of biochemical parameters within 72 hours (1, 2). To our knowledge, AIP recurrence after transplantation has not been described. Recipients without porphyria who receive livers from donors with AIP, however, subsequently may have symptoms of acute porphyria (3), underscoring the need to avoid transplanting livers from donors with known porphyria.Objective: To illustrate the need for genetic testing in patients with autosomal dominant disorders who are receiving organs from a sibling.Case Report: We ...References1. Dowman JK, Gunson BK, Mirza DF, Bramhall SR, Badminton MN, Newsome PN; UK Liver Selection and Allocation Working Party. Liver transplantation for acute intermittent porphyria is complicated by a high rate of hepatic artery thrombosis. Liver Transpl. 2012;18:195-200. [PMID: 21618697] doi:10.1002/lt.22345 CrossrefMedlineGoogle Scholar2. Soonawalla ZF, Orug T, Badminton MN, Elder GH, Rhodes JM, Bramhall SR, et al. Liver transplantation as a cure for acute intermittent porphyria. Lancet. 2004;363:705-6. [PMID: 15001330] CrossrefMedlineGoogle Scholar3. Tan A, Florman SS, Schiano TD. Genetic, hematological, and immunological disorders transmissible with liver transplantation. Liver Transpl. 2017;23:663-678. [PMID: 28240807] doi:10.1002/lt.24755 CrossrefMedlineGoogle Scholar4. DiMartini A, Dew MA, Liu Q, Simpson MA, Ladner DP, Smith AR, et al. Social and financial outcomes of living liver donation: A prospective investigation within the adult-to-adult living donor liver transplantation cohort study 2 (A2ALL-2). Am J Transplant. 2017;17:1081-1096. [PMID: 27647626] doi:10.1111/ajt.14055 CrossrefMedlineGoogle Scholar5. Lentine KL, Kasiske BL, Levey AS, Adams PL, Alberú J, Bakr MA, et al. KDIGO clinical practice guideline on the evaluation and care of living kidney donors. Transplantation. 2017;101:S1-S109. [PMID: 28742762] doi:10.1097/TP.00000000000017695. CrossrefMedlineGoogle Scholar Author, Article, and Disclosure InformationAffiliations: Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts (H.A., D.J.K.)Icahn School of Medicine, New York, New York (A.A.P., T.D.S.)Financial Support: Dr. Al-Samkari is a recipient of the National Hemophilia Foundation–Shire Clinical Fellowship Award, which provides partial salary support.Disclosures: Disclosures can be viewed at www.acponline.org/authors/icmje/ConflictOfInterestForms.do?msNum=L18-0623.This article was published at Annals.org on 5 February 2019. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byAGA Clinical Practice Update on Diagnosis and Management of Acute Hepatic Porphyrias: Expert ReviewMarginal parental donors for pediatric living donor liver transplantationReappraisal of liver transplantation for erythropoietic protoporphyria: A deadly combination of disease recurrence and biliary complicationLiver transplantation for intermittent acute porphyria resistant to medical treatmentTrasplante hepático por porfiria aguda intermitente resistente a tratamiento médicoRecurrence of mild neuropathic pain, following deceased donor liver transplantation, in patient with acute intermittent porphyria: Could timing explain it? A case reportThe acute porphyriasHemin 18 June 2019Volume 170, Issue 12Page: 904-905KeywordsAutosomal dominant diseasesCirrhosisDisclosureGenetic testingLiver diseasesLiver transplantationPorphyriaSplenomegalyTransplantationVomiting ePublished: 5 February 2019 Issue Published: 18 June 2019 Copyright & PermissionsCopyright © 2019 by American College of Physicians. All Rights Reserved.PDF downloadLoading ...