Silent infarcts in young children with sickle cell disease

JL Kwiatkowski, RA Zimmerman… - British journal of …, 2009 - Wiley Online Library
JL Kwiatkowski, RA Zimmerman, AN Pollock, W Seto, K Smith‐Whitley, J Shults
British journal of haematology, 2009Wiley Online Library
Silent infarcts have been reported most commonly in school‐aged children with
homozygous sickle cell disease (SCD‐SS) and are associated with neurocognitive deficits.
However, the prevalence of silent infarcts in younger children with SCD‐SS is not well
defined. In this retrospective study, brain magnetic resonance imaging and angiography
(MRI/A) studies performed before 6 years of age in a cohort of children with SCD‐SS were
analysed and the prevalence of abnormalities was calculated. Clinical and laboratory …
Summary
Silent infarcts have been reported most commonly in school‐aged children with homozygous sickle cell disease (SCD‐SS) and are associated with neurocognitive deficits. However, the prevalence of silent infarcts in younger children with SCD‐SS is not well defined. In this retrospective study, brain magnetic resonance imaging and angiography (MRI/A) studies performed before 6 years of age in a cohort of children with SCD‐SS were analysed and the prevalence of abnormalities was calculated. Clinical and laboratory parameters were compared between the groups with and without silent infarcts. Sixty‐eight of 96 children in the cohort had brain MRI/A performed prior to age 6 years. Of the 65 who were neurologically asymptomatic, 18 (27·7%, 95% CI 17·3–40·2%) had silent infarcts (mean age 3·7 ± 1·1 years, range 1·3–5·9 years). Factors associated with silent infarcts included cerebral vessel stensosis by magnetic resonance angiography, lower rates of vaso‐occlusive pain and acute chest syndrome and lower haemoglobin levels. The prevalence of silent infarcts in young children with SCD‐SS is similar to that of older children and anaemia and severe vasculopathy may be risk factors.
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