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Hemorheological risk factors of acute chest syndrome and painful vaso-occlusive crisis in children with sickle cell disease.
Haematologica 2012; 97(11):1641-7H

Abstract

BACKGROUND

Little is known about the effects of blood rheology on the occurrence of acute chest syndrome and painful vaso-occlusive crises in children with sickle cell anemia and hemoglobin SC disease.

DESIGN AND METHODS

To address this issue, steady-state hemorheological profiles (blood viscosity, red blood cell deformability, aggregation properties) and hematologic parameters were assessed in 44 children with sickle cell anemia and 49 children with hemoglobin SC disease (8-16 years old) followed since birth. Clinical charts were retrospectively reviewed to determine prior acute chest syndrome or vaso-occlusive episodes, and rates of these complications were calculated.

RESULTS

Multivariate analysis revealed that: 1) a higher steady-state blood viscosity was associated with a higher rate of vaso-occlusive crises in children with sickle cell anemia, but not in children with hemoglobin SC disease; 2) a higher steady-state red blood cell disaggregation threshold was associated with previous history of acute chest syndrome in children with hemoglobin SC disease and boys with sickle cell anemia.

CONCLUSIONS

Our results indicate for the first time that the red blood cell aggregation properties may play a role in the pathophysiology of acute chest syndrome in children with hemoglobin SC disease and boys with sickle cell anemia. In addition, whereas greater blood viscosity is associated with a higher rate of vaso-occlusive crises in children with sickle cell anemia, no association was found in children with hemoglobin SC disease, underscoring differences in the etiology of vaso-occlusive crises between sickle cell anemia and hemoglobin SC disease.

Authors+Show Affiliations

Inserm U665, CHU de Pointe à Pitre, Hôpital Ricou, 97159 Pointe-à-Pitre, Guadeloupe. pconnes@yahoo.fr.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Clinical Trial
Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

22689686

Citation

Lamarre, Yann, et al. "Hemorheological Risk Factors of Acute Chest Syndrome and Painful Vaso-occlusive Crisis in Children With Sickle Cell Disease." Haematologica, vol. 97, no. 11, 2012, pp. 1641-7.
Lamarre Y, Romana M, Waltz X, et al. Hemorheological risk factors of acute chest syndrome and painful vaso-occlusive crisis in children with sickle cell disease. Haematologica. 2012;97(11):1641-7.
Lamarre, Y., Romana, M., Waltz, X., Lalanne-Mistrih, M. L., Tressières, B., Divialle-Doumdo, L., ... Connes, P. (2012). Hemorheological risk factors of acute chest syndrome and painful vaso-occlusive crisis in children with sickle cell disease. Haematologica, 97(11), pp. 1641-7. doi:10.3324/haematol.2012.066670.
Lamarre Y, et al. Hemorheological Risk Factors of Acute Chest Syndrome and Painful Vaso-occlusive Crisis in Children With Sickle Cell Disease. Haematologica. 2012;97(11):1641-7. PubMed PMID: 22689686.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Hemorheological risk factors of acute chest syndrome and painful vaso-occlusive crisis in children with sickle cell disease. AU - Lamarre,Yann, AU - Romana,Marc, AU - Waltz,Xavier, AU - Lalanne-Mistrih,Marie-Laure, AU - Tressières,Benoît, AU - Divialle-Doumdo,Lydia, AU - Hardy-Dessources,Marie-Dominique, AU - Vent-Schmidt,Jens, AU - Petras,Marie, AU - Broquere,Cedric, AU - Maillard,Frederic, AU - Tarer,Vanessa, AU - Etienne-Julan,Maryse, AU - Connes,Philippe, Y1 - 2012/06/11/ PY - 2012/6/13/entrez PY - 2012/6/13/pubmed PY - 2013/11/8/medline SP - 1641 EP - 7 JF - Haematologica JO - Haematologica VL - 97 IS - 11 N2 - BACKGROUND: Little is known about the effects of blood rheology on the occurrence of acute chest syndrome and painful vaso-occlusive crises in children with sickle cell anemia and hemoglobin SC disease. DESIGN AND METHODS: To address this issue, steady-state hemorheological profiles (blood viscosity, red blood cell deformability, aggregation properties) and hematologic parameters were assessed in 44 children with sickle cell anemia and 49 children with hemoglobin SC disease (8-16 years old) followed since birth. Clinical charts were retrospectively reviewed to determine prior acute chest syndrome or vaso-occlusive episodes, and rates of these complications were calculated. RESULTS: Multivariate analysis revealed that: 1) a higher steady-state blood viscosity was associated with a higher rate of vaso-occlusive crises in children with sickle cell anemia, but not in children with hemoglobin SC disease; 2) a higher steady-state red blood cell disaggregation threshold was associated with previous history of acute chest syndrome in children with hemoglobin SC disease and boys with sickle cell anemia. CONCLUSIONS: Our results indicate for the first time that the red blood cell aggregation properties may play a role in the pathophysiology of acute chest syndrome in children with hemoglobin SC disease and boys with sickle cell anemia. In addition, whereas greater blood viscosity is associated with a higher rate of vaso-occlusive crises in children with sickle cell anemia, no association was found in children with hemoglobin SC disease, underscoring differences in the etiology of vaso-occlusive crises between sickle cell anemia and hemoglobin SC disease. SN - 1592-8721 UR - https://www.unboundmedicine.com/medline/citation/22689686/Hemorheological_risk_factors_of_acute_chest_syndrome_and_painful_vaso_occlusive_crisis_in_children_with_sickle_cell_disease_ L2 - http://www.haematologica.org/cgi/pmidlookup?view=long&pmid=22689686 DB - PRIME DP - Unbound Medicine ER -