{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,17]],"date-time":"2026-10-17T20:17:00Z","timestamp":1792268220821,"version":"4.4.0"},"reference-count":47,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2026,4,17]],"date-time":"2026-04-17T00:00:00Z","timestamp":1776384000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,4,17]],"date-time":"2026-04-17T00:00:00Z","timestamp":1776384000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Liver International"],"published-print":{"date-parts":[[2026,5]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background and Aims<\/jats:title>\n                    <jats:p>Acute intermittent porphyria (AIP) is a rare disorder with diverse clinical presentations, ranging from latent at\u2010risk individuals to recurrent acute attacks with severe complications. Differences between clinical states and risk factors for severe outcomes remain incompletely defined.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Approach and Results<\/jats:title>\n                    <jats:p>\n                      In this cross\u2010sectional study, personal, biochemical and clinical data were recorded for non\u2010givosiran treated AIP patients across all clinical states by European porphyria expert centres into the European Porphyria Registry during 2012\u20132018. Logistic regression models assessed associations between clinical factors and outcomes, with disease states defined according to clinical practice at study initiation. Urinary \u03b4\u2010aminolevulinic acid (u\u2010ALA) and porphobilinogen (u\u2010PBG) were normalized to each centre's upper reference limits. Data from 239 participants were included and patients were classified as sporadic attack(s) (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200961), recurrent (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200949), in\u2010remission (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200958), asymptomatic high excreters (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200923) and latent at\u2010risk (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200948). Hospitalization for an acute attack was associated with \u2265\u200910\u2010fold u\u2010PBG increase (adjusted Odds Ratios [aOR]\u2009=\u200922.40, 95% CI\u2009=\u20095.34\u201394.03), 4\u20139\u2010fold (6.35 [2.87\u201314.02]) and \u2265\u200910\u2010fold (177.50 [8.03\u20133923]) u\u2010ALA increase, BMI\u2009&lt;\u200918.5 (12.68 [2.86\u201356.23]) and age 20\u201339 (4.35 [1.82\u201310.42]). A 10\u2010fold u\u2010PBG increase (8.22 [2.31\u201329.28]) and a positive family history at diagnosis (0.30 [0.14\u20130.64]) were associated with recurrent classification. Recurrent AIP (7.28 [2.77\u201319.14]) and sporadic heme\u2010treated acute attacks (5.30 [1.89\u201314.91]) were associated with reduced work capacity and unemployment. Chronic hypertension, chronic kidney disease and primary liver cancer were observed across all clinical groups.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>In this multicentre European study, we describe disease burden across all clinical AIP states including non\u2010givosiran treated recurrent patients and identify risk factors associated with hospitalization and recurrent disease.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/liv.70615","type":"journal-article","created":{"date-parts":[[2026,4,17]],"date-time":"2026-04-17T11:15:36Z","timestamp":1776424536000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Clinical Characteristics and Outcomes of Acute Intermittent Porphyria: Insights From the European Porphyria Registry"],"prefix":"10.1111","volume":"46","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7772-5315","authenticated-orcid":false,"given":"Carl M.","family":"Baravelli","sequence":"first","affiliation":[{"name":"Norwegian Porphyria Centre (NAPOS), Department of Medical Biochemistry and Pharmacology, Haukeland University Hospital  Bergen Norway"},{"name":"Department of Disease Burden Norwegian Institute of Public Health  Bergen Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sverre","family":"Sandberg","sequence":"additional","affiliation":[{"name":"Norwegian Porphyria Centre (NAPOS), Department of Medical Biochemistry and Pharmacology, Haukeland University Hospital  Bergen Norway"},{"name":"Norwegian Organization for Quality Improvement of Laboratory Examinations Haraldsplass Deaconess Hospital  Bergen Norway"},{"name":"Department of Global Public Health and Primary Care University of Bergen  Bergen Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marte H.","family":"Hammersland","sequence":"additional","affiliation":[{"name":"Norwegian Porphyria Centre (NAPOS), Department of Medical Biochemistry and Pharmacology, Haukeland University Hospital  Bergen Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7336-2801","authenticated-orcid":false,"given":"Pauline","family":"Harper","sequence":"additional","affiliation":[{"name":"Department of Medical Biochemistry and Biophysics, Centre for Inherited Metabolic Diseases, Porphyria Centre Sweden Karolinska Institutet, Karolinska University Hospital  Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0290-2828","authenticated-orcid":false,"given":"Michael N.","family":"Badminton","sequence":"additional","affiliation":[{"name":"School of Medicine Cardiff University  Cardiff 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Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matteo","family":"Marcacci","sequence":"additional","affiliation":[{"name":"Department of Medical and Surgical Sciences for Children and Adults, Internal Medicine Unit, University Hospital of Modena University of Modena and Reggio Emilia  Modena Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eliane","family":"Sardh","sequence":"additional","affiliation":[{"name":"Department of Molecular Medicine and Surgery, Centre for Inherited Metabolic Diseases, Porphyria Centre Sweden Karolinska Institutet, Karolinska University Hospital  Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Caroline","family":"Schmitt","sequence":"additional","affiliation":[{"name":"Assistance Publique\u2010H\u00f4pitaux de Paris Centre Fran\u00e7ais Des Porphyries, H\u00f4pital Louis Mourier, Colombes and INSERM UMR1134, Universit\u00e9 Paris Cit\u00e9  Paris 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