{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,10]],"date-time":"2026-10-10T15:29:56Z","timestamp":1791646196339,"version":"4.3.4"},"reference-count":41,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2017,1,18]],"date-time":"2017-01-18T00:00:00Z","timestamp":1484697600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2017,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Sickle cell anaemia (SCA) is a severe hereditary haemoglobinopathy characterised by haemorheological abnormalities, which play a role in the occurrence of several acute and chronic clinical complications. While \u03b2<jats:sup>S<\/jats:sup>\u2010haplotypes and alpha\u2010thalassaemia modulate SCA clinical severity, their effects on blood rheology have been incompletely described. The aim of this study was to test the effects of these genetic modifiers on the haemorheological properties and clinical complication of children with SCA.<\/jats:p><\/jats:sec><jats:sec><jats:title>Procedure<\/jats:title><jats:p>Steady\u2010state haemorheological profile, biological parameters, \u03b2<jats:sup>S<\/jats:sup>\u2010haplotypes, alpha\u2010globin status, vaso\u2010occlusive crisis (VOC) and acute chest syndrome frequencies were analysed in 128 children (aged 5 to 18 years) with SCA.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Patients with alpha\u2010thalassaemia showed increased red blood cell (RBC) deformability and aggregation compared to those without. Median VOC rate was higher in patients with homozygous alpha\u2010thalassaemia compared to those with a normal alpha genotype. Conversely, the haemorheological profile and clinical complications were not influenced by the \u03b2<jats:sup>S<\/jats:sup>\u2010haplotypes in our study.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Our results demonstrate that alpha\u2010thalassaemia is associated with higher risk for VOC events in children with SCA, which may be due in part to its effects on RBC deformability and aggregation.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.26455","type":"journal-article","created":{"date-parts":[[2017,1,18]],"date-time":"2017-01-18T06:53:39Z","timestamp":1484722419000},"source":"Crossref","is-referenced-by-count":40,"title":["Alpha\u2010thalassaemia promotes frequent vaso\u2010occlusive crises in children with sickle cell anaemia through haemorheological changes"],"prefix":"10.1002","volume":"64","author":[{"given":"C\u00e9line","family":"Renoux","sequence":"first","affiliation":[{"name":"Laboratoire Interuniversitaire de Biologie de la Motricit\u00e9 (LIBM) EA7424 Equipe \u201cBiologie vasculaire et du globule rouge\u201d Universit\u00e9 Claude Bernard Lyon 1, COMUE  Lyon France"},{"name":"Laboratoire d'Excellence sur le globule rouge (Labex GR\u2010Ex)  Paris France"},{"name":"UF Pathologie mol\u00e9culaire du globule rouge H\u00f4pital Edouard Herriot  Lyon France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philippe","family":"Connes","sequence":"additional","affiliation":[{"name":"Laboratoire Interuniversitaire de Biologie de la Motricit\u00e9 (LIBM) EA7424 Equipe \u201cBiologie vasculaire et du globule rouge\u201d Universit\u00e9 Claude Bernard Lyon 1, COMUE  Lyon France"},{"name":"Laboratoire d'Excellence sur le globule rouge (Labex GR\u2010Ex)  Paris France"},{"name":"Institut Universitaire de France  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elie","family":"Nader","sequence":"additional","affiliation":[{"name":"Laboratoire Interuniversitaire de Biologie de la Motricit\u00e9 (LIBM) EA7424 Equipe \u201cBiologie vasculaire et du globule rouge\u201d Universit\u00e9 Claude Bernard Lyon 1, COMUE  Lyon France"},{"name":"Laboratoire d'Excellence sur le globule rouge (Labex GR\u2010Ex)  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah","family":"Skinner","sequence":"additional","affiliation":[{"name":"Laboratoire Interuniversitaire de Biologie de la Motricit\u00e9 (LIBM) EA7424 Equipe \u201cBiologie vasculaire et du globule rouge\u201d Universit\u00e9 Claude Bernard Lyon 1, COMUE  Lyon France"},{"name":"Laboratoire d'Excellence sur le globule rouge (Labex GR\u2010Ex)  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Camille","family":"Faes","sequence":"additional","affiliation":[{"name":"Laboratoire Interuniversitaire de Biologie de la Motricit\u00e9 (LIBM) EA7424 Equipe \u201cBiologie vasculaire et du globule rouge\u201d Universit\u00e9 Claude Bernard Lyon 1, COMUE  Lyon France"},{"name":"Laboratoire d'Excellence sur le globule rouge (Labex GR\u2010Ex)  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie","family":"Petras","sequence":"additional","affiliation":[{"name":"Unit\u00e9 Transversale de la Dr\u00e9panocytose Centre Hospitalier Universitaire  Pointe\u2010\u00e0\u2010Pitre Guadeloupe France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yves","family":"Bertrand","sequence":"additional","affiliation":[{"name":"Institut d'h\u00e9matologie et d'oncologie p\u00e9diatrique (IHOP) Hospices Civils de Lyon  Lyon France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nathalie","family":"Garnier","sequence":"additional","affiliation":[{"name":"Institut d'h\u00e9matologie et d'oncologie p\u00e9diatrique (IHOP) Hospices Civils de Lyon  Lyon France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniela","family":"Cuzzubbo","sequence":"additional","affiliation":[{"name":"Institut d'h\u00e9matologie et d'oncologie p\u00e9diatrique (IHOP) Hospices Civils de Lyon  Lyon France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lydia","family":"Divialle\u2010Doumdo","sequence":"additional","affiliation":[{"name":"Unit\u00e9 Transversale de la Dr\u00e9panocytose Centre Hospitalier Universitaire  Pointe\u2010\u00e0\u2010Pitre Guadeloupe France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kamila","family":"Keba\u00efli","sequence":"additional","affiliation":[{"name":"Institut d'h\u00e9matologie et d'oncologie p\u00e9diatrique (IHOP) Hospices Civils de Lyon  Lyon France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C\u00e9cile","family":"Renard","sequence":"additional","affiliation":[{"name":"Institut d'h\u00e9matologie et d'oncologie p\u00e9diatrique (IHOP) Hospices Civils de Lyon  Lyon France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandra","family":"Gauthier","sequence":"additional","affiliation":[{"name":"Laboratoire Interuniversitaire de Biologie de la Motricit\u00e9 (LIBM) EA7424 Equipe \u201cBiologie vasculaire et du globule rouge\u201d Universit\u00e9 Claude Bernard Lyon 1, COMUE  Lyon France"},{"name":"Laboratoire d'Excellence sur le globule rouge (Labex GR\u2010Ex)  Paris France"},{"name":"Institut d'h\u00e9matologie et d'oncologie p\u00e9diatrique (IHOP) Hospices Civils de Lyon  Lyon France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maryse","family":"Etienne\u2010Julan","sequence":"additional","affiliation":[{"name":"Unit\u00e9 Transversale de la Dr\u00e9panocytose Centre Hospitalier Universitaire  Pointe\u2010\u00e0\u2010Pitre Guadeloupe France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giovanna","family":"Cannas","sequence":"additional","affiliation":[{"name":"Laboratoire Interuniversitaire de Biologie de la Motricit\u00e9 (LIBM) EA7424 Equipe \u201cBiologie vasculaire et du globule rouge\u201d Universit\u00e9 Claude Bernard Lyon 1, COMUE  Lyon France"},{"name":"Laboratoire d'Excellence sur le globule rouge (Labex GR\u2010Ex)  Paris France"},{"name":"Clinique de M\u00e9decine Ambulatoire\/H\u00e9matologie H\u00f4pital Edouard Herriot  Lyon France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cyril","family":"Martin","sequence":"additional","affiliation":[{"name":"Laboratoire Interuniversitaire de Biologie de la Motricit\u00e9 (LIBM) EA7424 Equipe \u201cBiologie vasculaire et du globule rouge\u201d Universit\u00e9 Claude Bernard Lyon 1, COMUE  Lyon France"},{"name":"Laboratoire d'Excellence sur le globule rouge (Labex GR\u2010Ex)  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie\u2010Dominique","family":"Hardy\u2010Dessources","sequence":"additional","affiliation":[{"name":"Laboratoire d'Excellence sur le globule rouge (Labex GR\u2010Ex)  Paris France"},{"name":"UMR Inserm 1134 H\u00f4pital Ricou Centre Hospitalier Universitaire  Pointe\u2010\u00e0\u2010Pitre Guadeloupe France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincent","family":"Pialoux","sequence":"additional","affiliation":[{"name":"Laboratoire Interuniversitaire de Biologie de la Motricit\u00e9 (LIBM) EA7424 Equipe \u201cBiologie vasculaire et du globule rouge\u201d Universit\u00e9 Claude Bernard Lyon 1, COMUE  Lyon France"},{"name":"Laboratoire d'Excellence sur le globule rouge (Labex GR\u2010Ex)  Paris France"},{"name":"Institut Universitaire de France  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marc","family":"Romana","sequence":"additional","affiliation":[{"name":"Laboratoire d'Excellence sur le globule rouge (Labex GR\u2010Ex)  Paris France"},{"name":"UMR Inserm 1134 H\u00f4pital Ricou Centre Hospitalier Universitaire  Pointe\u2010\u00e0\u2010Pitre Guadeloupe France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philippe","family":"Joly","sequence":"additional","affiliation":[{"name":"Laboratoire Interuniversitaire de Biologie de la Motricit\u00e9 (LIBM) EA7424 Equipe \u201cBiologie vasculaire et du globule rouge\u201d Universit\u00e9 Claude Bernard Lyon 1, COMUE  Lyon France"},{"name":"Laboratoire d'Excellence sur le globule rouge (Labex GR\u2010Ex)  Paris France"},{"name":"UF Pathologie mol\u00e9culaire du globule rouge H\u00f4pital Edouard Herriot  Lyon France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,1,18]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199107043250103"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2011.047365"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2012.066670"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0158182"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V72.4.1216.1216"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V72.6.2056.2056"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1749-6632.1989.tb24180.x"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.blre.2015.08.005"},{"key":"e_1_2_8_10_1","doi-asserted-by":"crossref","first-page":"1014","DOI":"10.1681\/ASN.V1051014","article-title":"Evidence that microdeletions in the \u03b1 globin gene protect against the development of sickle cell glomerulopathy in humans","volume":"10","author":"Guasch A","year":"1999","journal-title":"J Am Soc Nephrol"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.3233\/CH-131772"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bcmd.2010.06.003"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/ejh.12607"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2008-03-143891"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198206173062402"},{"key":"e_1_2_8_16_1","doi-asserted-by":"crossref","first-page":"475","DOI":"10.1016\/S0889-8588(18)30426-X","article-title":"Beta s\u2010gene\u2010cluster haplotypes in sickle cell anemia. Clinical and hematologic features","volume":"5","author":"Powars DR.","year":"1991","journal-title":"Hematol Oncol Clin North Am"},{"key":"e_1_2_8_17_1","first-page":"55","article-title":"Beta\u2010S gene cluster haplotypes modulate hematologic and hemorheologic expression in sickle cell anemia. Use in predicting clinical severity","volume":"16","author":"Powars DR","year":"1994","journal-title":"Am J Pediatr Hematol Oncol"},{"key":"e_1_2_8_18_1","first-page":"360","article-title":"The variable expression of sickle cell disease is genetically determined","volume":"27","author":"Powars D","year":"1990","journal-title":"Semin Hematol"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V79.8.2154.2154"},{"key":"e_1_2_8_20_1","first-page":"455","article-title":"Flowcharts for the diagnosis and the molecular characterization of hemoglobinopathies","volume":"68","author":"Aguilar\u2010Martinez P","year":"2010","journal-title":"Ann Biol Clin (Paris)"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V95.1.360"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cca.2011.03.025"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.3233\/CH-2009-1202"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.3233\/CH-151979"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.3233\/CH-2009-1178"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.1034\/j.1600-0609.2003.00016.x"},{"key":"e_1_2_8_27_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra0804411"},{"key":"e_1_2_8_28_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.blre.2006.07.001"},{"key":"e_1_2_8_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bcmd.2015.10.005"},{"key":"e_1_2_8_30_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI111181"},{"key":"e_1_2_8_31_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2013-01-480277"},{"key":"e_1_2_8_32_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2015.130435"},{"key":"e_1_2_8_33_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V68.5.985.985"},{"key":"e_1_2_8_34_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.12786"},{"key":"e_1_2_8_35_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.tracli.2008.03.011"},{"key":"e_1_2_8_36_1","doi-asserted-by":"publisher","DOI":"10.3233\/CH-2012-1573"},{"key":"e_1_2_8_37_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.21572"},{"key":"e_1_2_8_38_1","doi-asserted-by":"crossref","first-page":"36","DOI":"10.59566\/IJBS.2012.8036","article-title":"Association between XmnI polymorphism and HbF level in sickle cell disease patients from Chhattisgarh","volume":"8","author":"Bhagat S","year":"2012","journal-title":"Int J Biomed Sci IJBS"},{"key":"e_1_2_8_39_1","doi-asserted-by":"crossref","first-page":"832","DOI":"10.1182\/blood.V79.3.832.832","article-title":"Variation of HbF and F\u2010cell number with the G\u2010gamma Xmn I (C\u2010T) polymorphism in normal individuals","volume":"79","author":"Sampietro M","year":"1992","journal-title":"Blood"},{"key":"e_1_2_8_40_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.12507"},{"key":"e_1_2_8_41_1","doi-asserted-by":"crossref","first-page":"2595","DOI":"10.1182\/blood.V82.8.2595b.2595b","article-title":"Beta\u2010globin gene cluster haplotype and alpha\u2010thalassemia do not correlate with the acute clinical manifestations of sickle cell disease in children","volume":"82","author":"Montalembert M","year":"1993","journal-title":"Blood"},{"key":"e_1_2_8_42_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830360305"}],"container-title":["Pediatric Blood &amp; Cancer"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fpbc.26455","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/pbc.26455","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,5]],"date-time":"2023-10-05T09:18:33Z","timestamp":1696497513000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/pbc.26455"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,1,18]]},"references-count":41,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2017,8]]}},"alternative-id":["10.1002\/pbc.26455"],"URL":"https:\/\/doi.org\/10.1002\/pbc.26455","archive":["Portico"],"relation":{},"ISSN":["1545-5009","1545-5017"],"issn-type":[{"value":"1545-5009","type":"print"},{"value":"1545-5017","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,1,18]]},"article-number":"e26455"}}