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Liver Transplantation for Acute Intermittent Porphyria
Mattias Lissing1, Greg Nowak, René Adam
1Hepatology DivisionDepartment of Upper GI Diseases Porphyria Centre SwedenKarolinska Institutet and Karolinska University Hospital Stockholm Sweden Department of Clinical Science, Intervention and Technology (CLINTEC) Karolinska Institutet and Karolinska University Hospital Stockholm Sweden Paul Brousse Hospital University Paris-SudInserm U935 Villejuif France The Queen Elizabeth University Hospital Birmingham UK Centre Francais des Porphyries Hôpital Louis MourierAssistance Publique-Hôpitaux de Paris Paris France Department of General Internal Medicine Universitair Ziekenhuis (UZ) Leuven Leuven Belgium Section for GastroenterologyNorwegian Primary Sclerosing Cholangitis (PSC) Research CenterDepartment of Transplantation MedicineResearch Institute of Internal MedicineDivision of SurgeryInflammatory Diseases and TransplantationOslo University Hospital RikshospitaletHybrid Technology Hub-Centre of ExcellenceInstitute of Basic Medical SciencesInstitute of Clinical MedicineFaculty of Medicine University of Oslo Oslo Norway Department of Nephrology, Dialysis and Internal Diseases Medical University of Warsaw Warsaw Poland Liver Center MunichDepartment of Medicine II University HospitalLudwig Maximilian University (LMU) Munich Munich Germany Liver Transplant UnitHospital Clínic de BarcelonaInstitut d'Investigacions Biomèdiques August Pi i SunyerCentro de Investigación Biomédica en Red en Enfermedades Hepáticas y Digestivas Universitat de Barcelona Barcelona Spain Institute of Sanitary and Biomedical Investigation of Alicante Alicante Spain Department of Visceral Transplant Surgery University Medical Center Hamburg-Eppendorf Hamburg Germany Erasmus MCUniversity Medical Center RotterdamPorphyria Center Rotterdam Rotterdam The Netherlands Department of Medical and Surgical Sciences for Children and Adults University of Modena and Reggio EmiliaUnit of Internal MedicinePoliclinico Hospital of Modena Modena Italy Department of Transplantation and Liver Surgery Helsinki University Hospital Helsinki Finland Liver Transplant Unit, Edouard Herriot Hospital Lyon France Department of General, Visceral, Thoracic, Transplantation and Pediatric Surgery Universitätsklinikum Schleswig-Holstein (UKSH) Campus Kiel Kiel Germany Department of Visceral Surgery and Medicine Inselspital University Hospital of Bern Bern Switzerland The Liver Unit Addenbrooke's HospitalCambridge University Hospitals Cambridge UK.
Liver transplantation (LT) offers a cure for acute intermittent porphyria (AIP) attacks, improving quality of life. However, severe neuropathy and renal impairment before LT increase mortality risk, necessitating early evaluation.
Area of Science:
- Hepatology
- Transplantation Medicine
- Rare Diseases
Background:
- Recurrent acute intermittent porphyria (AIP) attacks severely impact patient quality of life and pose significant health risks.
- Liver transplantation (LT) is a potential cure for AIP, but comprehensive outcome data are limited.
Purpose of the Study:
- To assess pretransplant characteristics, complications, and outcomes of patients with AIP undergoing liver transplantation.
- To identify factors influencing survival and recovery post-LT for AIP patients.
Main Methods:
- Retrospective analysis of data from the European Liver Transplant Registry and international transplant centers.
- Study included 38 AIP patients who received LT between 2002 and 2019.
- Evaluation of pretransplant neurological and renal function, complications, and survival rates.
Main Results:
- Overall 1-year and 5-year survival rates were 92% and 82%, comparable to other metabolic diseases.
- Advanced pretransplant neurological impairment was linked to increased mortality (5-year survival 83% in severe neuropathy vs. 94% in moderate/no neuropathy).
- Pretransplant renal impairment (GFR < 60 mL/minute) was common (51%); neurological symptoms improved post-LT, and no AIP attacks occurred except with an auxiliary graft.
Conclusions:
- Liver transplantation is a curative treatment for recurrent acute intermittent porphyria.
- Severe pretransplant neuropathy and renal dysfunction are significant risk factors for poorer outcomes.
- Early evaluation for LT is recommended for AIP patients who fail other treatments.
