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Relationships between systemic vascular resistance, blood rheology and nitric oxide in children with sickle cell anemia or sickle cell-hemoglobin C disease. ; Relationships between systemic vascular resistance, blood rheology and nitric oxide in children with sickle cell anemia or sickle cell-hemoglobin C disease. : Hemodynamics in sickle cell disease
Auteur(s) : Lamarre, Yann Hardy-Dessources, Marie-Dominique Romana, Marc Lalanne-Mistrih, Marie-Laure Waltz, Xavier Petras, Marie Doumdo, Lydia Blanchet-Deverly, Anne
Auteurs secondaires : 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) Centre d'investigation clinique Antilles-Guyane ; Institut National de la Santé et de la Recherche Médicale (INSERM) - CH Cayenne Unité Transversale de la Drépanocytose ; CHU de Pointe-à-Pitre/Abymes Service d'Orthopédie et de Traumatologie ; Université des Antilles et de la Guyane (UAG) - CHU Pointe-à-Pitre/Abymes Unité d'explorations cardiovasculaire ; CHU de Pointe-à-Pitre/Abymes Service de Cardiologie ; CHU de Pointe-à-Pitre/Abymes Service de Pédiatrie ; CHU de Pointe-à-Pitre/Abymes The study has been funded by a Projet Hospitalier Recherche Clinique (PHRC) Interregional. The funder had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript. This work was also supported by European funds and the Conseil Régional de la Guadeloupe (fellowship to YL), as well as Inserm funding. The authors have declared that no competing interests exist.

Éditeur(s) : HAL CCSD IOS Press
Résumé : International audience
Vascular function has been found to be impaired in patients with sickle cell disease (SCD). The present study investigated the determinants of systemic vascular resistance in two main SCD syndromes in children: sickle cell anemia (SCA) and sickle cell-hemoglobin C disease (SCC). Nitric oxide metabolites (NOx), hematological, hemorheological, and hemodynamical parameters were investigated in 61 children with SCA and 49 children with SCC. While mean arterial pressure was not different between SCA and SCC children, systemic vascular resistance (SVR) was greater in SCC children. Although SVR and blood viscosity (ηb) were not correlated in SCC children, the increase of ηb (+18%) in SCC children compared to SCA children results in a greater mean SVR in this former group. SVR was positively correlated with ηb, hemoglobin (Hb) level and RBC deformability, and negatively with NOx level in SCA children. Multivariate linear regression model showed that both NOx and Hb levels were independently associated with SVR in SCA children. In SCC children, only NOx level was associated with SVR. In conclusion, vascular function of SCC children seems to better cope with higher ηb compared to SCA children. Since the occurrence of vaso-occlusive like complications are less frequent in SCC than in SCA children, this finding suggests a pathophysiological link between the vascular function alteration and these clinical manifestations. In addition, our results suggested that nitric oxide metabolism plays a key role in the regulation of SVR, both in SCA and SCC.
ISSN: 1386-0291

inserm-00849031
http://www.hal.inserm.fr/inserm-00849031
http://www.hal.inserm.fr/inserm-00849031/document
http://www.hal.inserm.fr/inserm-00849031/file/2013_Lamarre_-_Relationships_between_SVR_BR_and_NO_in_children_with_sickle_cell_anemia_or_sickle_cell-hemoglobin_C_disease.pdf
DOI : 10.3233/CH-121661

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