Micro- and macrovascular function in children with sickle cell anaemia and sickle cell haemoglobin C disease.
It is unclear whether vascular function is affected similarly in children with sickle cell anaemia (SS) and children with sickle haemoglobin C (SC) disease. Therefore, we compared micro and macrovascular functions in healthy (AA) children, children with SS and SC disease, and assessed their association with physical activity. Participants (24 SS, 22 SC and 16 AA), were compared in terms of 1) thermal hyperaemic response (finger pad warming to 42°C) measured by Laser Doppler techniques, 2) arterial stiffness determined by pulse wave velocity, 3) daily energy expenditure related to moderate and intense physical activities estimated by questionnaire and 4) fitness level, evaluated by the six-minute walk test. Response to heating differed between SS, SC and controls. Peripheral microvascular reactivity was lower and pulse wave velocity higher in SS compared to AA. SC had blunted microvascular reactivity in response to heating compared to AA but pulse wave velocity was not different within the two groups. Physical activity and fitness levels were markedly lower in sickle cell patients compared to healthy controls but no association was observed with vascular function. Microvasodilatory reserve is decreased in both SS and SC patients but only SS patients were also characterised by impaired macrovascular function.
Laboratory ACTES (EA 3596), French West Indies University, Pointe-à-Pitre, Guadeloupe, France; UMR Inserm U1134, French West Indies University, Pointe-à-Pitre, Guadeloupe, France; Laboratoire d'Excellence du Globule Rouge (LABEX GR-Ex), PRES Sorbonne, Paris, France.
Berenike
B
Möckesch
Laboratory ACTES (EA 3596), French West Indies University, Pointe-à-Pitre, Guadeloupe, France; UMR Inserm U1134, French West Indies University, Pointe-à-Pitre, Guadeloupe, France; Laboratoire d'Excellence du Globule Rouge (LABEX GR-Ex), PRES Sorbonne, Paris, France.
Keyne
K
Charlot
Laboratory ACTES (EA 3596), French West Indies University, Pointe-à-Pitre, Guadeloupe, France.
Stéphane
S
Jumet
UMR Inserm U1134, French West Indies University, Pointe-à-Pitre, Guadeloupe, France; Laboratoire d'Excellence du Globule Rouge (LABEX GR-Ex), PRES Sorbonne, Paris, France.
Marc
M
Romana
Sickle Cell Center, Academic Hospital of Pointe-à-Pitre, Pointe-à-Pitre, Guadeloupe, France.
Lydia
L
Divialle-Doumdo
UMR Inserm U1134, French West Indies University, Pointe-à-Pitre, Guadeloupe, France; Laboratoire d'Excellence du Globule Rouge (LABEX GR-Ex), PRES Sorbonne, Paris, France.
Marie-Dominique
MD
Hardy-Dessources
Sickle Cell Center, Academic Hospital of Pointe-à-Pitre, Pointe-à-Pitre, Guadeloupe, France.
Marie
M
Petras
Centre Investigation Clinique Antilles Guyane, 1424 Inserm, Academic Hospital of Pointe-à-Pitre, Pointe-à-Pitre, Guadeloupe, France.
Benoît
B
Tressieres
Sickle Cell Center, Academic Hospital of Pointe-à-Pitre, Pointe-à-Pitre, Guadeloupe, France.
Vanessa
V
Tarer
Laboratory ACTES (EA 3596), French West Indies University, Pointe-à-Pitre, Guadeloupe, France.
Olivier
O
Hue
Sickle Cell Center, Academic Hospital of Pointe-à-Pitre, Pointe-à-Pitre, Guadeloupe, France.
Maryse
M
Etienne-Julan
UMR Inserm U1134, French West Indies University, Pointe-à-Pitre, Guadeloupe, France; Laboratoire d'Excellence du Globule Rouge (LABEX GR-Ex), PRES Sorbonne, Paris, France; Institut Universitaire de France (IUF), Paris, France; Laboratory LIBM EA7424, Team "Vascular Biology and Red Blood Cell", University of Lyon 1, France.
Philippe
P
Connes
Laboratory ACTES (EA 3596), French West Indies University, Pointe-à-Pitre, Guadeloupe, France. Electronic address: s_antoine@ymail.com.
Sophie
S
Antoine-Jonville
D000293:Adolescent; D002648:Child; D004734:Energy Metabolism; D015444:Exercise; D005260:Female; D006450:Hemoglobin SC Disease / Q000503:physiopathology*; D006801:Humans; D008297:Male; D008833:Microcirculation; D063177:Pulse Wave Analysis
Physical activity; Sickle cell anaemia; Sickle cell haemoglobin C disease; Vascular function