title: Hematologic and hemorheological determinants of resting and exercise-induced hemoglobin oxygen desaturation in children with sickle cell disease.
abstract: 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.
authors.affiliation: UMR Inserm 665, Pointe-à-Pitre, Guadeloupe, Université des Antilles et de la Guyane, Pointe-à-Pitre, France.
authors.forename: Xavier
authors.initials: X
authors.lastname: Waltz
authors.forename: Marc
authors.initials: M
authors.lastname: Romana
authors.forename: Marie-Laure
authors.initials: ML
authors.lastname: Lalanne-Mistrih
authors.forename: Roberto F
authors.initials: RF
authors.lastname: Machado
authors.forename: Yann
authors.initials: Y
authors.lastname: Lamarre
authors.forename: Vanessa
authors.initials: V
authors.lastname: Tarer
authors.forename: Marie-Dominique
authors.initials: MD
authors.lastname: Hardy-Dessources
authors.forename: Benoît
authors.initials: B
authors.lastname: Tressières
authors.forename: Lydia
authors.initials: L
authors.lastname: Divialle-Doumdo
authors.forename: Marie
authors.initials: M
authors.lastname: Petras
authors.forename: Frederic
authors.initials: F
authors.lastname: Maillard
authors.forename: Maryse
authors.initials: M
authors.lastname: Etienne-Julan
authors.forename: Philippe
authors.initials: P
authors.lastname: Connes
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000097:blood* / Q000175:diagnosis* / Q000473:pathology; D002648:Child; D015444:Exercise / Q000502:physiology*; D005260:Female; D006451:Hemoglobin, Sickle / Q000378:metabolism*; D018056:Hemorheology / Q000502:physiology*; D006801:Humans; D008297:Male; D010101:Oxygen Consumption / Q000502:physiology*; D012146:Rest / Q000502:physiology*; D012189:Retrospective Studies
chemical_list: D006451:Hemoglobin, Sickle
references.citation: Br J Haematol. 2005 Oct;131(1):129-34
references.pmid: 16173973
references.citation: J Appl Physiol Respir Environ Exerc Physiol. 1981 Jul;51(1):131-4
references.pmid: 7263405
references.citation: Am J Hematol. 2012 Dec;87(12):1070-3
references.pmid: 22911571
references.citation: Am J Emerg Med. 1996 Jan;14(1):16-8
references.pmid: 8630147
references.citation: Haematologica. 2012 Aug;97(8):1136-41
references.pmid: 22315500
references.citation: Clin Hemorheol Microcirc. 2001;25(1):1-11
references.pmid: 11790865
references.citation: J Clin Invest. 1970 Apr;49(4):623-34
references.pmid: 5443167
references.citation: N Engl J Med. 1991 Jul 4;325(1):11-6
references.pmid: 1710777
references.citation: Microcirculation. 2004 Mar;11(2):153-65
references.pmid: 15280089
references.citation: Haematologica. 2009 Aug;94(8):1060-5
references.pmid: 19644138
references.citation: PLoS One. 2012;7(12):e52471
references.pmid: 23285055
references.citation: Sports Med. 2000 Jul;30(1):47-61
references.pmid: 10907757
references.citation: Blood. 2003 Feb 1;101(3):846-8
references.pmid: 12393400
references.citation: Lancet. 2003 Nov 1;362(9394):1450-5
references.pmid: 14602439
references.citation: J Appl Physiol (1985). 1999 May;86(5):1460-7
references.pmid: 10233105
references.citation: Clin Lab Haematol. 1997 Mar;19(1):17-22
references.pmid: 9146942
references.citation: Blood. 1993 Jun 15;81(12):3422-7
references.pmid: 7685205
references.citation: Clin Hemorheol Microcirc. 2009;42(2):75-97
references.pmid: 19433882
references.citation: Blood. 2013 Apr 11;121(15):3054-6
references.pmid: 23580637
references.citation: Br J Haematol. 2009 Nov;147(3):352-9
references.pmid: 19694721
references.citation: Clin Hemorheol Microcirc. 2011;49(1-4):151-63
references.pmid: 22214686
references.citation: Blood. 2006 Mar 15;107(6):2279-85
references.pmid: 16291595
references.citation: Am J Respir Crit Care Med. 2002 Jul 1;166(1):111-7
references.pmid: 12091180
references.citation: Br J Haematol. 2008 Feb;140(3):336-9
references.pmid: 18042265
references.citation: Int J Sports Med. 2004 Nov;25(8):569-74
references.pmid: 15531998
references.citation: J Clin Invest. 1973 Feb;52(2):422-32
references.pmid: 4683881
references.citation: Clin Hemorheol Microcirc. 2012;52(1):15-26
references.pmid: 22414551
references.citation: Am J Respir Crit Care Med. 2002 May 1;165(9):1309-16
references.pmid: 11991885
references.citation: Pediatr Pulmonol. 1999 Dec;28(6):423-8
references.pmid: 10587417
references.citation: Haematologica. 2012 Nov;97(11):1641-7
references.pmid: 22689686
references.citation: Haematologica. 2009 Mar;94(3):340-7
references.pmid: 19211639
references.citation: Blood Rev. 2007 Jan;21(1):37-47
references.pmid: 17084951
references.citation: Microcirculation. 2008 Oct;15(7):585-90
references.pmid: 18608991
references.citation: Pediatr Pulmonol. 1999 Dec;28(6):418-22
references.pmid: 10587416
references.citation: Clin Hemorheol Microcirc. 2002;26(3):175-81
references.pmid: 12082248
references.citation: Clin Hemorheol Microcirc. 2008;40(1):73-5
references.pmid: 18791268
references.citation: Am J Hematol. 2011 Feb;86(2):123-54
references.pmid: 21264896
references.citation: Crit Care Med. 2001 Sep;29(9):1803-6
references.pmid: 11546990
references.citation: J Pediatr. 2007 Apr;150(4):395-9, 399.e1-2
references.pmid: 17382117
references.citation: Haematologica. 2013 Mar;98(3):464-72
references.pmid: 22983573
references.citation: Blood. 1992 Apr 15;79(8):2154-63
references.pmid: 1562742
references.citation: Blood. 2013 Jan 3;121(1):243-4
references.pmid: 23287628
references.citation: Arch Intern Med. 1974 Apr;133(4):565-72
references.pmid: 4594394
references.citation: Br J Haematol. 2013 May;161(4):484-98
references.pmid: 23496688