Hematologic and hemorheological determinants of resting and exercise-induced hemoglobin oxygen desaturation in children with sickle cell disease
Xavier Waltz,
Marc Romana,
Marie-Laure Lalanne-Mistrih,
Roberto F. Machado,
Yann Lamarre,
Vanessa Tarer,
Marie-Dominique Hardy-Dessources,
Benoît Tressières,
Lydia Divialle-Doumdo,
Marie Petras,
Frederic Maillard,
Maryse Etienne-Julan,
Philippe Connes
Affiliations
Xavier Waltz
UMR Inserm 665, Pointe-à-Pitre, Guadeloupe, Université des Antilles et de la Guyane, Pointe-à-Pitre, France;Laboratoire ACTES (EA 3596), Département de Physiologie, Université des Antilles et de la Guyane, Pointe-à-Pitre, Guadeloupe, France;Laboratory of Excellence GR-Ex «The red cell: from genesis to death», PRES Sorbonne Paris Cité, Paris, France
Marc Romana
UMR Inserm 665, Pointe-à-Pitre, Guadeloupe, Université des Antilles et de la Guyane, Pointe-à-Pitre, France;Laboratory of Excellence GR-Ex «The red cell: from genesis to death», PRES Sorbonne Paris Cité, Paris, France
Marie-Laure Lalanne-Mistrih
UMR Inserm 665, Pointe-à-Pitre, Guadeloupe, Université des Antilles et de la Guyane, Pointe-à-Pitre, France;CIC-EC 802 Inserm, Centre Hospitalier Universitaire de Pointe-à-Pitre, Pointe-à-Pitre, France
Roberto F. Machado
Section of Pulmonary, Critical Care Medicine, Sleep and Allergy, University of Illinois at Chicago, Chicago, IL, USA
Yann Lamarre
UMR Inserm 665, Pointe-à-Pitre, Guadeloupe, Université des Antilles et de la Guyane, Pointe-à-Pitre, France;Laboratory of Excellence GR-Ex «The red cell: from genesis to death», PRES Sorbonne Paris Cité, Paris, France
Vanessa Tarer
Centre de référence maladies rares pour la drépanocytose aux Antilles-Guyane, Centre Hospitalier et Universitaire de Pointe-à-Pitre, Pointe-à-Pitre, France
Marie-Dominique Hardy-Dessources
UMR Inserm 665, Pointe-à-Pitre, Guadeloupe, Université des Antilles et de la Guyane, Pointe-à-Pitre, France;Laboratory of Excellence GR-Ex «The red cell: from genesis to death», PRES Sorbonne Paris Cité, Paris, France
Benoît Tressières
CIC-EC 802 Inserm, Centre Hospitalier Universitaire de Pointe-à-Pitre, Pointe-à-Pitre, France
Lydia Divialle-Doumdo
Unité Transversale de la Drépanocytose, Centre Hospitalier et Universitaire de Pointe-à-Pitre, Pointe-à-Pitre, France
Marie Petras
Unité Transversale de la Drépanocytose, Centre Hospitalier et Universitaire de Pointe-à-Pitre, Pointe-à-Pitre, France
Frederic Maillard
Service de Pédiatrie du Centre Hospitalier et Universitaire de Pointe-à-Pitre, Pointe-à-Pitre, Guadeloupe
Maryse Etienne-Julan
Centre de référence maladies rares pour la drépanocytose aux Antilles-Guyane, Centre Hospitalier et Universitaire de Pointe-à-Pitre, Pointe-à-Pitre, France;Unité Transversale de la Drépanocytose, Centre Hospitalier et Universitaire de Pointe-à-Pitre, Pointe-à-Pitre, France
Philippe Connes
UMR Inserm 665, Pointe-à-Pitre, Guadeloupe, Université des Antilles et de la Guyane, Pointe-à-Pitre, France;Laboratoire ACTES (EA 3596), Département de Physiologie, Université des Antilles et de la Guyane, Pointe-à-Pitre, Guadeloupe, France;Laboratory of Excellence GR-Ex «The red cell: from genesis to death», PRES Sorbonne Paris Cité, Paris, France
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.