Open AccessBrain injury pathophysiology study by a multimodal approach in children with sickle cell anemia with no intra or extra cranial arteriopathy
Author(s) -
Valentine Brousse,
Corinne Pondarré,
Manoëlle Kossorotoff,
Cécile Arnaud,
Annie Kamdem,
Mariane de Montalembert,
Bénédicte Boutonnat-Faucher,
Slimane Allali,
H. Bourdeau,
Key Ne Charlot,
S. Bertil,
Lydie Da Costa,
Philippe Connes,
D. Grévent,
Suzanne Verlhac
Publication year - 2021
Publication title -
haematologica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.782
H-Index - 142
eISSN - 1592-8721
pISSN - 0390-6078
DOI - 10.3324/haematol.2020.278226
Subject(s) - medicine , pathophysiology , sickle cell anemia , perfusion , anemia , microcirculation , magnetic resonance imaging , perfusion scanning , endothelial dysfunction , cardiology , cerebral perfusion pressure , pathology , radiology , disease
Despite its high prevalence in children with sickle cell anemia (SCA), the pathophysiology of silent cerebral infarcts (SCIs) remains elusive. The main objective of this study was to explore the respective roles of major determinants of brain perfusion in SCA children with no past or current history of intracranial or extracranial vasculopathy. We used a multimodal approach based notably on perfusion imaging Arterial spin labelling (ASL) MRI and Near Infra-Red Spectroscopy (NIRS), as well as biomarkers reflecting blood rheology and endothelial activation.Out of 59 SCA patients (mean age 11.4} 3.9 yrs), 8 (13%) had a total of 12 SCIs. Children with SCIs had a distinctive profile characterized by decreased blood pressure, impaired blood rheology, increased P-selectin levels, and marked anemia. Although ASL perfusion and oximetry values did not differ between groups, comparison of biological and clinical parameters according to the level of perfusion categorized in terciles showed an independent association between high perfusion and increased sP-selectin, decreased RBC deformability, low HbF level, increased blood viscosity and no alpha-thalassemia deletion. NIRS measurements did not yield additional novel results. Altogether, these findings argue for early MRI detection of SCIs in children with no identified vasculopathy and suggest a potential role for ASL as an additional screening tool. Early treatment targeting hemolysis, anemia and endothelial dysfunction should reduce the risk of this under diagnosed and serious complication.