{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,13]],"date-time":"2026-02-13T15:38:39Z","timestamp":1770997119539,"version":"3.50.1"},"reference-count":25,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2012,12,4]],"date-time":"2012-12-04T00:00:00Z","timestamp":1354579200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["European J of Haematology"],"published-print":{"date-parts":[[2013,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>Congenital dyserythropoietic anemia (<jats:styled-content style=\"fixed-case\">CDA<\/jats:styled-content>) is a rare group of red blood cell disorders with ineffective erythropoiesis and secondary hemochromatosis. Inappropriate suppression of hepcidin and high levels of growth differentiation factor 15 (<jats:styled-content style=\"fixed-case\">GDF<\/jats:styled-content>15) have been described in <jats:styled-content style=\"fixed-case\">CDA<\/jats:styled-content> I and <jats:styled-content style=\"fixed-case\">II<\/jats:styled-content> patients, probably contributing to secondary hemochromatosis. Hemojuvelin (<jats:styled-content style=\"fixed-case\">HJV<\/jats:styled-content>) is an important regulator of serum hepcidin, while soluble form of <jats:styled-content style=\"fixed-case\">HJV<\/jats:styled-content> (s\u2010<jats:styled-content style=\"fixed-case\">HJV<\/jats:styled-content>) competitively down\u2010regulates hepcidin.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We determined the soluble hemojuvelin (s\u2010<jats:styled-content style=\"fixed-case\">HJV<\/jats:styled-content>) levels in 17 patients with <jats:styled-content style=\"fixed-case\">CDA<\/jats:styled-content> I and in 17 healthy volunteers (<jats:styled-content style=\"fixed-case\">HV<\/jats:styled-content>) and looked for correlations with other parameters of iron overload and erythropoiesis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Significantly higher levels of s\u2010<jats:styled-content style=\"fixed-case\">HJV<\/jats:styled-content> were found in patients (2.32\u00a0\u00b1\u00a01.40\u00a0mg\/L) compared with healthy volunteers (0. 69\u00a0\u00b1\u00a00.44\u00a0mg\/L) (<jats:italic>P<\/jats:italic>\u00a0=\u00a00.001). Western blot analysis confirmed the presence of high levels of s\u2010<jats:styled-content style=\"fixed-case\">HJV<\/jats:styled-content> in <jats:styled-content style=\"fixed-case\">CDA<\/jats:styled-content> I patients. s\u2010<jats:styled-content style=\"fixed-case\">HJV<\/jats:styled-content> positively correlated with serum ferritin, erythropoietin, soluble transferrin receptor, and <jats:styled-content style=\"fixed-case\">GDF<\/jats:styled-content>15 and negatively correlated with hepcidin to ferritin ratios.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>We for the first time documented high levels of serum s\u2010<jats:styled-content style=\"fixed-case\">HJV<\/jats:styled-content> in <jats:styled-content style=\"fixed-case\">CDA<\/jats:styled-content> I patients, suggesting that it may contribute to iron loading pathology in <jats:styled-content style=\"fixed-case\">CDA<\/jats:styled-content> I and eventually in other anemias with ineffective erythropoiesis.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ejh.12027","type":"journal-article","created":{"date-parts":[[2012,10,24]],"date-time":"2012-10-24T09:56:06Z","timestamp":1351072566000},"page":"31-36","source":"Crossref","is-referenced-by-count":13,"title":["High levels of soluble serum hemojuvelin in patients with congenital dyserythropoietic anemia type I"],"prefix":"10.1111","volume":"90","author":[{"given":"Hanna","family":"Shalev","sequence":"first","affiliation":[{"name":"Hematology Department Soroka Medical Center Faculty of Medicine Ben\u2010Gurion University Beer Sheva Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Galit","family":"Perez\u2010Avraham","sequence":"additional","affiliation":[{"name":"Hematology Department Soroka Medical Center Faculty of Medicine Ben\u2010Gurion University Beer Sheva Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joseph","family":"Kapelushnik","sequence":"additional","affiliation":[{"name":"Pediatric Hematology Oncology Soroka Medical Center Faculty of Medicine Ben\u2010Gurion University Beer Sheva Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Itai","family":"Levi","sequence":"additional","affiliation":[{"name":"Hematology Department Soroka Medical Center Faculty of Medicine Ben\u2010Gurion University Beer Sheva Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anat","family":"Rabinovich","sequence":"additional","affiliation":[{"name":"Hematology Department Soroka Medical Center Faculty of Medicine Ben\u2010Gurion University Beer Sheva Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dorine W.","family":"Swinkels","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine Laboratory of Genetic, Endocrine and Metabolic Diseases (LGEM 830) Radboud University Nijmegen Medical Centre Nijmegen the Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carole","family":"Brasse\u2010Lagnel","sequence":"additional","affiliation":[{"name":"Laboratoire de Biochimie M\u00e9dicale H\u00f4pital Universitaire de Rouen N\u00e9oVAsc EA4309\u2010ERI28 \u201cEndoth\u00e9lium Microvasculaire et L\u00e9sions C\u00e9r\u00e9brales N\u00e9onatales\u201d Universit\u00e9 de Rouen Rouen France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hannah","family":"Tamary","sequence":"additional","affiliation":[{"name":"Hematology Oncology Division Schneider Children's Medical Center of Israel Sackler Faculty of Medicine Tel Aviv University Petah Tikva Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2012,12,4]]},"reference":[{"key":"e_1_2_7_2_1","first-page":"103","article-title":"Congenital dyserythropoietic anemia with karyorrhexis and 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