Hematologic and hemorheological determinants of resting and exercise-induced hemoglobin oxygen desaturation in children with sickle cell disease.

Xavier Waltz (1, 2, 3) , Marc Romana (1, 3) , Marie-Laure Lalanne-Mistrih (1, 4) , Roberto F. Machado (5) , Yann Lamarre (1, 3) , Vanessa Tarer (6) , Marie-Dominique Hardy-Dessources (1, 3) , Benoit Tressieres (4) , Lydia Divialle-Doumdo (7) , Marie Petras (7) , Frederic Maillard (8) , Maryse Etienne-Julan (6, 7) , Philippe Connes (1, 2, 3)

1 Protéines de la membrane érythrocytaire et homologues non-érythroides
2 ACTES - Adaptations au Climat Tropical, Exercice et Santé
3 Laboratory of Excellence GR-Ex " The red cell : from genesis to death "
4 CIC - Antilles Guyane - Centre d'investigation clinique Antilles-Guyane
5 Section of Pulmonary, Critical Care Medicine, Sleep and Allergy
6 Service d'Orthopédie et de Traumatologie
7 Unité Transversale de la Drépanocytose
8 Service de Pédiatrie

The aim of the study was to determine the factors associated with resting and exercise-induced hemoglobin oxygen desaturation. The well-established six-minute walk test was conducted in 107 sickle cell children (50 with sickle hemoglobin C disease and 57 with sickle cell anemia) at steady state. Hemoglobin oxygen saturation was measured before and immediately after the six-minute walk test. Blood samples were obtained on the same day to measure hematologic and hemorheological parameters. Exercise-induced hemoglobin oxygen desaturation was defined as a drop in hemoglobin oxygen saturation of 3% or more at the end of the six-minute walk test compared to resting levels. No children with sickle hemoglobin C disease, but approximately 50% of children with sickle cell anemia showed mild or moderate oxygen desaturation at rest, which was independently associated with the percentage of reticulocytes. Exercise-induced hemoglobin oxygen desaturation was observed in 18% of children with sickle hemoglobin C disease and 34% of children with sickle cell anemia, and was independently associated with the six-minute walk test, acute chest syndrome rate and the strength of red blood cell aggregates in children with sickle cell anemia. No association was found in children with sickle hemoglobin C disease between exercise-induced hemoglobin oxygen desaturation and the measured parameters. Hemoglobin oxygen desaturation at rest was common in children with sickle cell anemia but not in children with sickle hemoglobin C disease, and was mainly associated with greater hemolysis. Physiological strain during exercise and red blood cell aggregation properties may predict the occurrence of exercise-induced hemoglobin oxygen desaturation in children with sickle cell anemia.

Domaines: Santé publique et épidémiologie

Xavier Waltz, Marc Romana, Marie-Laure Lalanne-Mistrih, Roberto F. Machado, Yann Lamarre, et al.. Hematologic and hemorheological determinants of resting and exercise-induced hemoglobin oxygen desaturation in children with sickle cell disease.. Haematologica, 2013, 98 (7), pp.1039-44. ⟨10.3324/haematol.2013.083576⟩. ⟨inserm-00838989⟩