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<title lang=en>Alpha-thalassaemia promotes frequent vaso-occlusive crises in children with sickle cell anaemia through haemorheological changes</title>
<creator>Renoux, Céline</creator>
<creator>Connes, Philippe</creator>
<creator>Nader, Elie</creator>
<creator>Skinner, Sarah</creator>
<creator>Faes, Camille</creator>
<creator>Petras, Marie</creator>
<creator>Bertrand, Yves</creator>
<creator>Garnier, Nathalie</creator>
<creator>Cuzzubbo, Daniela</creator>
<creator>Divialle-Doumdo, Lydia</creator>
<creator>Kebaili, Kamila</creator>
<creator>Renard, Cécile</creator>
<creator>Gauthier, Alexandra</creator>
<creator>Etienne-Julan, Maryse</creator>
<creator>Cannas, Giovanna</creator>
<creator>Martin, Cyril</creator>
<creator>Hardy-Dessources, Marie-Dominique</creator>
<creator>Pialoux, Vincent</creator>
<creator>Romana, Marc</creator>
<creator>Joly, Philippe</creator>
<contributor>Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM ) ; Université Claude Bernard Lyon 1 (UCBL) - Université Jean Monnet [Saint-Étienne] (UJM) - Université Savoie Mont Blanc (USMB [Université de Savoie] [Université de Chambéry])</contributor>
<contributor>Dynamique des Structures et Interactions des Macromolécules Biologiques- Pôle de La Réunion (DSIMB Réunion) ; Dynamique des Structures et Interactions des Macromolécules Biologiques (DSIMB) ; Institut National de la Transfusion Sanguine [Paris] (INTS) - Université Paris Diderot - Paris 7 (UPD7) - Institut National de la Santé et de la Recherche Médicale (INSERM) - Institut National de la Transfusion Sanguine [Paris] (INTS) - Université Paris Diderot - Paris 7 (UPD7) - Institut National de la Santé et de la Recherche Médicale (INSERM) - Université de la Réunion (UR)</contributor>
<contributor>Unité Transversale de la Drépanocytose ; CHU de Pointe-à-Pitre/Abymes</contributor>
<contributor>Amélioration génétique et adaptation des plantes méditerranéennes et tropicales (UMR AGAP) ; Institut national de la recherche agronomique [Montpellier] (INRA Montpellier) - Centre international d'études supérieures en sciences agronomiques (Montpellier SupAgro) - Centre de Coopération Internationale en Recherche Agronomique pour le Développement (CIRAD) - Institut national d’études supérieures agronomiques de Montpellier (Montpellier SupAgro)</contributor>
<contributor>Service d'hématologie : Immuno-Hématologie pédiatrique et transplantation de moelle osseuse ; Hospices Civils de Lyon - Hôpital Debrousse</contributor>
<contributor>Unité Transversale de la Drépanocytose ; CHU Pointe-à-Pitre/Abymes</contributor>
<contributor>Hôpital Edouard Herriot ; Hospices Civils de Lyon - Hôpital Edouard Herriot</contributor>
<contributor>Centre de Recherche et d'Innovation sur le Sport (EA647) (CRIS) ; Université Claude Bernard Lyon 1 (UCBL)</contributor>
<contributor>Protéines de la membrane érythrocytaire et homologues non-érythroides ; Université des Antilles et de la Guyane (UAG) - Institut National de la Transfusion Sanguine [Paris] (INTS) - Université Paris Diderot - Paris 7 (UPD7) - Université de la Réunion (UR) - Institut National de la Santé et de la Recherche Médicale (INSERM)</contributor>
<contributor>Institut de recherche en informatique de Toulouse (IRIT) ; Institut National Polytechnique [Toulouse] (INP) - Université Toulouse 1 Capitole (UT1) - Université Toulouse 2 (UT2) - Université Paul Sabatier - Toulouse 3 (UPS) - Centre National de la Recherche Scientifique (CNRS)</contributor>
<description>International audience</description>
<source>ISSN: 1545-5009</source>
<source>EISSN: 1545-5017</source>
<source>Pediatric Blood and Cancer</source>
<publisher>Wiley</publisher>
<identifier>hal-01668317</identifier>
<identifier>https://hal.univ-antilles.fr/hal-01668317</identifier>
<source>https://hal.univ-antilles.fr/hal-01668317</source>
<source>Pediatric Blood and Cancer, Wiley, 2017, 64 (8), 〈10.1002/pbc.26455〉</source>
<identifier>DOI : 10.1002/pbc.26455</identifier>
<relation>info:eu-repo/semantics/altIdentifier/doi/10.1002/pbc.26455</relation>
<identifier>PUBMED : 28097791</identifier>
<relation>info:eu-repo/semantics/altIdentifier/pmid/28097791</relation>
<language>en</language>
<subject lang=en>alpha-thalassaemia</subject>
<subject lang=en> haemorheology</subject>
<subject lang=en> sickle cell disease</subject>
<subject lang=en> vaso-occlusive crisis</subject>
<subject lang=en> βS-haplotypes</subject>
<subject>[SDV.GEN.GH] Life Sciences [q-bio]/Genetics/Human genetics</subject>
<type>info:eu-repo/semantics/article</type>
<type>Journal articles</type>
<description lang=en>BACKGROUND: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 βS -haplotypes and alpha-thalassaemia 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.PROCEDURE:Steady-state haemorheological profile, biological parameters, βS -haplotypes, alpha-globin status, vaso-occlusive crisis (VOC) and acute chest syndrome frequencies were analysed in 128 children (aged 5 to 18 years) with SCA.RESULTS:Patients with alpha-thalassaemia showed increased red blood cell (RBC) deformability and aggregation compared to those without. Median VOC rate was higher in patients with homozygous alpha-thalassaemia compared to those with a normal alpha genotype. Conversely, the haemorheological profile and clinical complications were not influenced by the βS -haplotypes in our study.CONCLUSION:Our results demonstrate that alpha-thalassaemia 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.</description>
<date>2017-08</date>
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