{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T02:52:32Z","timestamp":1791082352872,"version":"4.1.0"},"reference-count":27,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2015,9,2]],"date-time":"2015-09-02T00:00:00Z","timestamp":1441152000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100002598","name":"American Porphyria Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100002598","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["HL 117199"],"award-info":[{"award-number":["HL 117199"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["U54 DK083909"],"award-info":[{"award-number":["U54 DK083909"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100008155","name":"Carolinas HealthCare System","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008155","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Eur J Clin Investigation"],"published-print":{"date-parts":[[2015,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Erythropoietic protoporphyria (EPP) and X\u2010linked protoporphyria (XLP) are genetic abnormalities of heme synthesis that result in excess production of protoporphyrin and that manifest as severe photosensitivity. These disorders are often associated with iron deficiency anaemia (<jats:styled-content style=\"fixed-case\">IDA<\/jats:styled-content>). Our aim was to determine whether hepcidin is increased in <jats:styled-content style=\"fixed-case\">EPP<\/jats:styled-content>\/<jats:styled-content style=\"fixed-case\">XLP<\/jats:styled-content> patients, resulting in decreased enteral iron absorption and <jats:styled-content style=\"fixed-case\">IDA<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Material and methods<\/jats:title><jats:p>Eight subjects with <jats:styled-content style=\"fixed-case\">EPP<\/jats:styled-content>, one with <jats:styled-content style=\"fixed-case\">XLP<\/jats:styled-content> and nine controls had baseline blood and urine samples collected, and thereafter were given oral ferrous sulphate (660\u00a0mg). Post\u2010iron blood and urine samples were collected at 2, 4, 6 and 8\u00a0h. Blood counts, serum cytokines, ferritin and iron studies were analysed at baseline. Serum iron studies, serum and urine hepcidin, and erythropoietin (Epo) were analysed at baseline and subsequent time points.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>At baseline, <jats:styled-content style=\"fixed-case\">EPP<\/jats:styled-content>\u2013<jats:styled-content style=\"fixed-case\">XLP<\/jats:styled-content> subjects had lower mean blood haemoglobin (13\u00b79\/15\u00b73\u00a0g\/dL) and serum ferritin (31\u00b76\/115\u00a0ng\/mL) than controls. Serum iron levels increased markedly in both cohorts. Mean serum and urine hepcidin levels were significantly lower in the <jats:styled-content style=\"fixed-case\">EPP<\/jats:styled-content>\u2013<jats:styled-content style=\"fixed-case\">XLP<\/jats:styled-content> group at 4 and 8\u00a0h post\u2010iron (serum \u2013 4\u00a0h, 3\u00b779\/26\u00b76, 8\u00a0h, 5\u00b779\/34\u00b76\u00a0nM; urine \u2013 4\u00a0h, 0\u00b785\/2\u00b750, 8\u00a0h, 1\u00b744\/6\u00b763\u00a0nM\/mM creatinine). Serum cytokines and Epo were normal and not different between groups.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>We conclude that serum and urine hepcidin are not inappropriately increased in <jats:styled-content style=\"fixed-case\">EPP<\/jats:styled-content>\/<jats:styled-content style=\"fixed-case\">XLP<\/jats:styled-content> subjects at baseline and do not increase over time as serum iron increases after oral ferrous sulphate. Levels of serum cytokines and Epo are normal in <jats:styled-content style=\"fixed-case\">EPP<\/jats:styled-content>\/<jats:styled-content style=\"fixed-case\">XLP<\/jats:styled-content>. The molecular basis for the iron\u2010deficient phenotype in <jats:styled-content style=\"fixed-case\">EPP<\/jats:styled-content>\/<jats:styled-content style=\"fixed-case\">XLP<\/jats:styled-content> remains unknown.<\/jats:p><\/jats:sec>","DOI":"10.1111\/eci.12503","type":"journal-article","created":{"date-parts":[[2015,7,21]],"date-time":"2015-07-21T15:23:04Z","timestamp":1437492184000},"page":"1032-1041","source":"Crossref","is-referenced-by-count":24,"title":["Homeostasis of iron and hepcidin in erythropoietic protoporphyria"],"prefix":"10.1111","volume":"45","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6595-4548","authenticated-orcid":false,"given":"Krista","family":"Bossi","sequence":"first","affiliation":[{"name":"The Liver\u2010Biliary\u2010Pancreatic Center Carolinas HealthCare System Charlotte NC USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jingyun","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Research Carolinas HealthCare System Charlotte NC USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Schmeltzer","sequence":"additional","affiliation":[{"name":"The Liver\u2010Biliary\u2010Pancreatic Center Carolinas HealthCare System Charlotte NC USA"},{"name":"Department of Medicine Carolinas HealthCare System Charlotte NC USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric","family":"Holburton","sequence":"additional","affiliation":[{"name":"The Liver\u2010Biliary\u2010Pancreatic Center Carolinas HealthCare System Charlotte NC USA"},{"name":"Department of Research Carolinas HealthCare System Charlotte NC USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gale","family":"Groseclose","sequence":"additional","affiliation":[{"name":"The Liver\u2010Biliary\u2010Pancreatic Center Carolinas HealthCare System Charlotte NC USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Siddesh","family":"Besur","sequence":"additional","affiliation":[{"name":"The Liver\u2010Biliary\u2010Pancreatic Center Carolinas HealthCare System Charlotte NC USA"},{"name":"Department of Medicine Carolinas HealthCare System Charlotte NC USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sunil","family":"Hwang","sequence":"additional","affiliation":[{"name":"Department of Research Carolinas HealthCare System Charlotte NC USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Herbert L.","family":"Bonkovsky","sequence":"additional","affiliation":[{"name":"The Liver\u2010Biliary\u2010Pancreatic Center Carolinas HealthCare System Charlotte NC USA"},{"name":"Department of Research Carolinas HealthCare System Charlotte NC USA"},{"name":"Department of Medicine Carolinas HealthCare System Charlotte NC USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2015,9,2]]},"reference":[{"key":"e_1_2_7_2_1","first-page":"2991","volume-title":"The Metabolic & Molecular Bases of Inherited Disease","author":"Anderson KE","year":"2001"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/cphy.c120006"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.2119\/molmed.2012.00340"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2133.2012.11160.x"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-142-6-200503150-00010"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/lt.20620"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.510270627"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/0011-5029(89)90003-5"},{"key":"e_1_2_7_10_1","unstructured":"SchrierSL.Causes and Diagnosis of Iron Deficiency Anemia in the Adult. 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