{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T14:22:14Z","timestamp":1791469334444,"version":"4.3.4"},"reference-count":35,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"4","license":[{"start":{"date-parts":[[2021,1,29]],"date-time":"2021-01-29T00:00:00Z","timestamp":1611878400000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":["Liver Transpl."],"published-print":{"date-parts":[[2021,4]]},"abstract":"<jats:p>Recurrent attacks of acute intermittent porphyria (AIP) result in poor quality of life and significant risks of morbidity and mortality. Liver transplantation (LT) offers a cure, but published data on outcomes after LT are limited. We assessed the pretransplant characteristics, complications, and outcomes for patients with AIP who received a transplant. Data were collected retrospectively from the European Liver Transplant Registry and from questionnaires sent to identified transplant and porphyria centers. We studied 38 patients who received transplants in 12 countries from 2002 to 2019. Median age at LT was 37\u00a0years (range, 18\u201058), and 34 (89%) of the patients were women. A total of 9 patients died during follow\u2010up, and 2 patients were retransplanted. The 1\u2010year and 5\u2010year overall survival rates were 92% and 82%, which are comparable with other metabolic diseases transplanted during the same period. Advanced pretransplant neurological impairment was associated with increased mortality. The 5\u2010year survival rate was 94% among 19 patients with moderate or no neuropathy at LT and 83% among 10 patients with severe neuropathy (<jats:italic toggle=\"yes\">P<\/jats:italic>\u00a0=\u00a00.04). Pretransplant renal impairment was common. A total of 19 (51%) patients had a GFR &lt; 60 mL\/minute. Although few patients improved their renal function after LT, neurological impairments improved, and no worsening of neurological symptoms was recorded. No patient had AIP attacks after LT, except for a patient who received an auxiliary graft. LT is a curative treatment option for patients with recurrent attacks of AIP. Severe neuropathy and impaired renal function are common and increase the risk for poor outcomes. If other treatment options fail, an evaluation for LT should be performed early.<\/jats:p>","DOI":"10.1002\/lt.25959","type":"journal-article","created":{"date-parts":[[2020,12,1]],"date-time":"2020-12-01T18:44:47Z","timestamp":1606848287000},"page":"491-501","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":51,"title":["Liver Transplantation for Acute Intermittent Porphyria"],"prefix":"10.1097","volume":"27","author":[{"given":"Mattias","family":"Lissing","sequence":"first","affiliation":[{"name":"Hepatology Division,Department of Upper GI Diseases,Porphyria Centre Sweden,Karolinska Institutet and Karolinska University Hospital,Stockholm,Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Greg","family":"Nowak","sequence":"additional","affiliation":[{"name":"Department of Clinical Science, Intervention and Technology (CLINTEC),Karolinska Institutet and Karolinska University Hospital,Stockholm,Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ren\u00e9","family":"Adam","sequence":"additional","affiliation":[{"name":"Paul Brousse Hospital,University Paris\u2010Sud,Inserm U935,Villejuif,France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincent","family":"Karam","sequence":"additional","affiliation":[{"name":"Paul Brousse Hospital,University Paris\u2010Sud,Inserm U935,Villejuif,France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexander","family":"Boyd","sequence":"additional","affiliation":[{"name":"The Queen Elizabeth University Hospital,Birmingham,UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laurent","family":"Gouya","sequence":"additional","affiliation":[{"name":"Centre Francais des Porphyries,H\u00f4pital Louis Mourier,Assistance Publique\u2010H\u00f4pitaux de Paris,Paris,France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wouter","family":"Meersseman","sequence":"additional","affiliation":[{"name":"Department of General Internal Medicine,Universitair Ziekenhuis (UZ) Leuven,Leuven,Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Espen","family":"Melum","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Urszula","family":"O\u0142dakowska\u2010Jedynak","sequence":"additional","affiliation":[{"name":"Department of Nephrology, Dialysis and Internal Diseases,Medical University of Warsaw,Warsaw,Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Florian P.","family":"Reiter","sequence":"additional","affiliation":[{"name":"Liver Center Munich,Department of Medicine II,University Hospital,Ludwig Maximilian University (LMU) Munich,Munich,Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jordi","family":"Colmenero","sequence":"additional","affiliation":[{"name":"Liver Transplant Unit,Hospital Cl\u00ednic de Barcelona,Institut d'Investigacions Biom\u00e8diques August Pi i Sunyer,Centro de Investigaci\u00f3n Biom\u00e9dica en Red en Enfermedades Hep\u00e1ticas y Digestivas,Universitat de Barcelona,Barcelona,Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rosario","family":"Sanchez","sequence":"additional","affiliation":[{"name":"Institute of Sanitary and Biomedical Investigation of Alicante,Alicante,Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Uta","family":"Herden","sequence":"additional","affiliation":[{"name":"Department of Visceral Transplant Surgery,University Medical Center Hamburg\u2010Eppendorf,Hamburg,Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janneke","family":"Langendonk","sequence":"additional","affiliation":[{"name":"Erasmus MC,University Medical Center Rotterdam,Porphyria Center Rotterdam,Rotterdam,The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paolo","family":"Ventura","sequence":"additional","affiliation":[{"name":"Department of Medical and Surgical Sciences for Children and Adults,University of Modena and Reggio Emilia,Unit of Internal Medicine,Policlinico Hospital of Modena,Modena,Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Helena","family":"Isoniemi","sequence":"additional","affiliation":[{"name":"Department of Transplantation and Liver Surgery,Helsinki University Hospital,Helsinki,Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olivier","family":"Boillot","sequence":"additional","affiliation":[{"name":"Liver Transplant Unit, Edouard Herriot Hospital,Lyon,France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Felix","family":"Braun","sequence":"additional","affiliation":[{"name":"Department of General, Visceral, Thoracic, Transplantation and Pediatric Surgery,Universit\u00e4tsklinikum Schleswig\u2010Holstein (UKSH),Campus Kiel,Kiel,Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2767-3219","authenticated-orcid":false,"given":"St\u00e9phanie","family":"Perrodin","sequence":"additional","affiliation":[{"name":"Department of Visceral Surgery and Medicine,Inselspital University Hospital of Bern,Bern,Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth","family":"Mowlem","sequence":"additional","affiliation":[{"name":"The Liver Unit,Addenbrooke's Hospital,Cambridge University Hospitals,Cambridge,UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Staffan","family":"Wahlin","sequence":"additional","affiliation":[{"name":"Hepatology Division,Department of Upper GI Diseases,Porphyria Centre Sweden,Karolinska Institutet and Karolinska University Hospital,Stockholm,Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"European Liver and Intestine Transplant Association","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2021,1,29]]},"reference":[{"key":"lt25959-bib-0001-20250830","doi-asserted-by":"crossref","first-page":"849","DOI":"10.1007\/s10545-012-9544-4","article-title":"The incidence of inherited porphyrias in Europe","volume":"36","year":"2013","journal-title":"J Inherit Metab Dis"},{"key":"lt25959-bib-0002-20250830","doi-asserted-by":"crossref","first-page":"809","DOI":"10.1007\/s10545-018-0178-z","article-title":"Medical and 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