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An 8\u2010year\u2010old Chinese male child was investigated because of chronic nonspherocytic hemolytic anemia (CNSHA) associated with hepatosplenomegaly. Genetic analysis unraveled co\u2010inheritance of a hemizygous mutation c.1225C&gt;T (p.Pro409Ser) in <jats:italic>G6PD<\/jats:italic> (G6PD Utrecht, previously reported only in The Netherlands) and heterozygote <jats:italic>HBB<\/jats:italic> mutation c.316\u2010197C&gt;T (IVS\u2010\u2161\u2010654 C&gt;T). Because IVS\u2010\u2161\u2010654 C&gt;T on its own does not cause CNSHA, we believe that the clinical manifestations in this patient are essentially due to the <jats:italic>G6PD<\/jats:italic> c.1225C&gt;T mutation. The boy gained transfusion independence after splenectomy.<\/jats:p>","DOI":"10.1002\/pbc.29837","type":"journal-article","created":{"date-parts":[[2022,6,13]],"date-time":"2022-06-13T12:50:07Z","timestamp":1655124607000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["A case of G6PD Utrecht associated with \u03b2\u2010thalassemia responding to splenectomy"],"prefix":"10.1002","volume":"69","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8619-9676","authenticated-orcid":false,"given":"Kun","family":"Yang","sequence":"first","affiliation":[{"name":"Department of Hematology Zigong First People's Hospital  Zigong China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xiaodong","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Hematology Zigong First People's Hospital  Zigong China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kai","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Critical Medicine Zigong First People's Hospital  Zigong China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shan","family":"Luo","sequence":"additional","affiliation":[{"name":"Department of Hematology Zigong First People's Hospital  Zigong China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wenqiang","family":"Kong","sequence":"additional","affiliation":[{"name":"Department of Pharmacy Zigong First People's Hospital  Zigong China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wenying","family":"Huang","sequence":"additional","affiliation":[{"name":"Department of Hematology Zigong First People's Hospital  Zigong China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jian","family":"Xiao","sequence":"additional","affiliation":[{"name":"Department of Hematology Zigong First People's Hospital  Zigong China"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2022,6,13]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(08)60073-2"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.16366"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.hoc.2015.11.006"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bcmd.2003.10.006"},{"issue":"4","key":"e_1_2_6_6_1","first-page":"887","article-title":"Somatic\u2010cell selection is a major determinant of the blood\u2010cell phenotype in heterozygotes for glucose\u20106\u2010phosphate dehydrogenase mutations causing severe enzyme deficiency","volume":"59","author":"Filosa S","year":"1996","journal-title":"Am J Hum Genet"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bcmd.2012.01.001"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.305.6856.760"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1159\/000204087"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.2174\/156652408786241384"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.3109\/03630269.2013.819558"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.hemonc.2017.05.029"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jsb.2015.10.015"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1964.tb00692.x"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1080\/10245330410001714211"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2019000944"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2016.161166"}],"container-title":["Pediatric Blood &amp; 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