Evaluation de la capacité Physique Sous Maximale au Moyen du Test de Marche de 6 Minutes Des Enfants et Adultes Jeunes Suivis Pour drépanocytose et Nouveaux paramètres d’hémolyse : Vers Une prédiction Des Complications Vasculaires ?

Study point of contact

Bernard Wenderickx
+3224773654
[email protected]
Laurence Dedeken, MD
+3224773469
[email protected]

Age

6 to 25 Years

Genotypes

HbSS, SC

Study type

Observational

Gender

All

Compensation

Unknown

About the study

The 6-minute walk (6MWT) test is used in adults and children affected by a wide range of
chronic diseases to evaluate their sub-maximal exercise capacity. It reflects the global
response of various physiological systems (respiratory, cardio-vascular, neurologic,
metabolic and musculosquelettic) in a situation simulating a daily life activity. In children
with sickle cell disease, the 6MWT is correlated with a low level of hemoglobin, a low level
of fetal hemoglobin and low red cell deformability. Our team previously reported that in a
population of children with sickle cell disease, highly treated with hydroxyurea, the sole
factor which was independently linked to the 6MWT was the presence of silence infarct.

As the cardio-vascular and cerebro-vascular injury in sickle cell disease are directly
correlated with hemolysis, the investigators aim to evaluate a) the clinical relevance of
endothelial and inflammation parameters and new hemolysis markers and b) if the presence of
silent infarct and the 6MWT are correlated with this biological markers.

This cross-sectional study will include sickle cell disease patients regularly followed for
more than 5 years at Hôpital Universitaire des Enfants Reine Fabiola, Centre Hospitalier
Universitaire (CHU)-Brugmann, Centre Hospitalier Etterbeek-Ixelles, CHU Saint-Pierre,
Cliniques Universitaires Saint-Luc (Bruxelles, Belgium). Inclusion criteria are: sickle cell
disease (SS, Sbeta°, SC, Sbeta+), age range : 6 to 25 years, signed informed consent.
Exclusion criteria are: transplanted patients, inability to perform the 6MWT (severe
cognitive disability, femoral osteonecrosis with functional impairment), hospitalization and/
or transfusion in the last 3 months for acute event.

Demographic data and clinical data will be retrospectively recorded. Blood test and 6MWT will
be performed in steady state. Studied analysis will be: coagulation factors, free hemoglobin,
Pro-B type natriuretic peptide (Pro-BNP), High sensitivity C reactive protein (HS-CRP),
Intercellular Adhesion Molecule (ICAM), Vascular Cell Adhesion Molecule (VCAM) and Selectins.

With this study, the investigators expect to validate new predictive markers for
cardio-vascular or cerebrovascular injury and to identify patients at high risk to develop
these complications.

participation requirements

– Sickle Cell Disease (HbSS, S beta°, S beta+, SC)

– Regular follow-up from more than 5 years

– Written informed consent

participation restrictions

– Transplanted patients

– Hospitalisation for an acute event within 3 months

– Acute transfusion within 3 months

– Unable to performed 6-minute walk test