{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,14]],"date-time":"2025-05-14T01:16:22Z","timestamp":1747185382886,"version":"3.40.5"},"reference-count":26,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2023,1,3]],"date-time":"2023-01-03T00:00:00Z","timestamp":1672704000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Int J Lab Hematology"],"published-print":{"date-parts":[[2023,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>The wide variation in hemoglobin (Hb) F levels has been observed in patients with Hb EE disease. This study aimed to describe hematologic features and determine the effect of genetic variants on Hb F expression in young children with Hb EE disease.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Hematologic features and Hb profiles of Laotian children aged 6\u201323\u2009months, who originally enrolled in the Lao\u2010Zinc study, were retrospectively reviewed. Only children with Hb EE disease, as indicated by DNA analysis, were included in this current analysis. Genetic variants, including the <jats:sup><jats:italic>G<\/jats:italic><\/jats:sup><jats:italic>\u03b3\u2010XmnI<\/jats:italic> polymorphism (C&gt;T) of the <jats:italic>HBG2<\/jats:italic> gene, the <jats:italic>HBS1L\u2010MYB<\/jats:italic> intergenic region on chromosome 6, and the <jats:italic>BCL11A<\/jats:italic> on chromosome 2 as well as the mutations occurring on the Kr\u00fcppel\u2010like factor 1 (<jats:italic>KLF1<\/jats:italic>) gene, were examined.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In total, 205 children were diagnosed as having Hb EE disease with Hb F ranged from 1.2 to 43.7%. Most of the children had mild to moderate anemia with a remarkable microcytosis. Analysis of the genetic variants revealed an extremely high frequency of the <jats:sup><jats:italic>G<\/jats:italic><\/jats:sup><jats:italic>\u03b3\u2010XmnI<\/jats:italic> (93.7%). Applying multiple regression analysis adjusted for age, sex, and \u03b1\u2010thal gene, a positive relation was observed for the rs4671393 (coefficient\u00a0=\u00a03.87, <jats:italic>p<\/jats:italic>\u00a0=\u2009.005) and the rs2297339 (coefficient\u00a0=\u00a02.48, <jats:italic>p<\/jats:italic>\u00a0=\u2009.046), but not the <jats:sup><jats:italic>G<\/jats:italic><\/jats:sup><jats:italic>\u03b3\u2010XmnI.<\/jats:italic> A statistically non\u2010significant relation was noted for the rs9399137 and the \u2212154 (C&gt;T) <jats:italic>KLF1<\/jats:italic> mutation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Our findings provide insight into complex situation of Hb F variability in young children with Hb EE disease; and this can guide to appropriate care and counseling to affected families.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ijlh.14017","type":"journal-article","created":{"date-parts":[[2023,1,4]],"date-time":"2023-01-04T06:03:12Z","timestamp":1672812192000},"page":"387-393","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Hemoglobin <scp>EE<\/scp> disease in young Laotian children: Hematologic features and the contributions of genetic variations to Hb F expression"],"prefix":"10.1111","volume":"45","author":[{"given":"Areeda","family":"Arong","sequence":"first","affiliation":[{"name":"Graduate School Khon Kaen University  Khon Kaen Thailand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hathichanok","family":"Srivorakun","sequence":"additional","affiliation":[{"name":"Centre for Research and Development of Medical Diagnostic Laboratories (CMDL), Faculty of Associated Medical Sciences Khon Kaen University  Khon Kaen Thailand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Attawut","family":"Chaibunruang","sequence":"additional","affiliation":[{"name":"Centre for Research and Development of Medical Diagnostic Laboratories (CMDL), Faculty of Associated Medical Sciences Khon Kaen University  Khon Kaen Thailand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9142-5079","authenticated-orcid":false,"given":"Supan","family":"Fucharoen","sequence":"additional","affiliation":[{"name":"Centre for Research and Development of Medical Diagnostic Laboratories (CMDL), Faculty of Associated Medical Sciences Khon Kaen University  Khon Kaen Thailand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Goonnapa","family":"Fucharoen","sequence":"additional","affiliation":[{"name":"Centre for Research and Development of Medical Diagnostic Laboratories (CMDL), Faculty of Associated Medical Sciences Khon Kaen University  Khon Kaen Thailand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sengchanh","family":"Kounnavong","sequence":"additional","affiliation":[{"name":"Lao Tropical and Public Health Institute Ministry of Health  Vientiane Lao People's Democratic Republic"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7453-9218","authenticated-orcid":false,"given":"Kanokwan","family":"Sanchaisuriya","sequence":"additional","affiliation":[{"name":"Centre for Research and Development of Medical Diagnostic Laboratories (CMDL), Faculty of Associated Medical Sciences Khon Kaen University  Khon Kaen Thailand"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2023,1,3]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/300768a0"},{"key":"e_1_2_7_3_1","first-page":"647","article-title":"Thalassemia in Southeast Asia: problems and strategy for prevention and control","volume":"23","author":"Fucharoen S","year":"1992","journal-title":"Southeast Asian J Trop Med Public Health"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1081\/HEM-120040334"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-008-0490-z"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.3109\/03630269.2011.637149"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1159\/000438739"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/ijlh.13164"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2009.07650.x"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-1-4939-7299-9_2"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2018.11.022"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1186\/s40795-018-0247-6"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1017\/S0007114519001715"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bcmd.2014.02.005"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/ejh.12201"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1159\/000096857"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinbiochem.2019.06.005"},{"key":"e_1_2_7_18_1","first-page":"1092","article-title":"Interaction of hemoglobin E and several forms of alpha\u2010thalassemia in Cambodian families","volume":"88","author":"Fucharoen S","year":"2003","journal-title":"Haematologica"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bcmd.2008.09.002"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1159\/000449120"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.4084\/mjhid.2019.038"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-015-2335-x"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bcmd.2016.04.010"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bcmd.2011.09.008"},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-006-0093-5"},{"key":"e_1_2_7_26_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12288-019-01150-5"},{"key":"e_1_2_7_27_1","doi-asserted-by":"publisher","DOI":"10.1159\/000048600"}],"container-title":["International Journal of Laboratory Hematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ijlh.14017","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/ijlh.14017","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ijlh.14017","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,19]],"date-time":"2023-08-19T22:51:51Z","timestamp":1692485511000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/ijlh.14017"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,1,3]]},"references-count":26,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2023,6]]}},"alternative-id":["10.1111\/ijlh.14017"],"URL":"https:\/\/doi.org\/10.1111\/ijlh.14017","archive":["Portico"],"relation":{},"ISSN":["1751-5521","1751-553X"],"issn-type":[{"type":"print","value":"1751-5521"},{"type":"electronic","value":"1751-553X"}],"subject":[],"published":{"date-parts":[[2023,1,3]]},"assertion":[{"value":"2022-10-01","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-12-29","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-01-03","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}