title: How I manage cerebral vasculopathy in children with sickle cell disease.
abstract: Sickle cell disease induces specific brain alterations that involve both the macrocirculation and the microcirculation. The main overt neurovascular complications in children are infarctive stroke, transient ischaemic attack and cerebral haemorrhage. Silent cerebral infarction, cognitive dysfunction and recurrent headache are also common. Cerebrovascular disease selectively affects children with the HbSS or HbS-β(0) genotypes (i.e. sickle cell anaemia). The incidence of stroke peaks between 2 and 5 years of age (1·02/100 patient-years) and increases with the severity of the anaemia. Most strokes can be prevented by annual transcranial Doppler screening from 2 to 16 years of age and providing chronic blood transfusion when this investigation shows elevated blood-flow velocities. The role for hydroxycarbamide in children with abnormal transcranial Doppler findings is under investigation. After a stroke, chronic blood transfusion is very strongly recommended, unless haematopoietic stem cell transplantation can be performed. Routine magnetic resonance imaging shows that more than one-third of children have silent cerebral infarction, which is associated with cognitive impairments. Screening for silent infarcts seems legitimate, since their presence may lead to supportive treatments. The role for more aggressive interventions such as hydroxycarbamide or chronic blood transfusion is debated.
authors.affiliation: Paediatrics and Sickle-Cell Centre, University Hospital Necker-Enfants Malades, APHP, Paris, France.|UMR S-1134, INSERM, Paris, France.|GR-Ex, Laboratory of Excellence, Paris, France.|Paris Descartes University, Paris, France.
authors.forename: Valentine
authors.initials: V
authors.lastname: Brousse
authors.affiliation: Paediatric Neurology, French Centre for Paediatric Stroke, Necker-Enfants Malades University Hospital, APHP, Paris, France.
authors.forename: Manoelle
authors.initials: M
authors.lastname: Kossorotoff
authors.affiliation: Paediatrics and Sickle-Cell Centre, University Hospital Necker-Enfants Malades, APHP, Paris, France.|GR-Ex, Laboratory of Excellence, Paris, France.|Paris Descartes University, Paris, France.
authors.forename: Mariane
authors.initials: M
authors.lastname: de Montalembert
mesh_terms: D000293:Adolescent; D064591:Allografts; D000755:Anemia, Sickle Cell / Q000150:complications / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000503:physiopathology / Q000628:therapy*; D001803:Blood Transfusion; D020520:Brain Infarction / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D002560:Cerebrovascular Circulation; D002648:Child; D002675:Child, Preschool; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D015994:Incidence; D008297:Male; D020521:Stroke; D017585:Ultrasonography, Doppler, Transcranial
keywords: hydroxycarbamide transfusion; sickle cell anaemia; stroke; transcranial doppler