Silent cerebral infarcts occur despite regular blood transfusion therapy after first strokes in children with sickle cell disease.

Children with sickle cell disease (SCD) and strokes receive blood transfusion therapy for secondary stroke prevention; despite this, approximately 20% experience second overt strokes. Given this rate of second overt strokes and the clinical significance of silent cerebral infarcts, we tested the hypothesis that silent cerebral infarcts occur among children with SCD being transfused for secondary stroke prevention. A prospective cohort enrolled children with SCD and overt strokes at 7 academic centers. Magnetic resonance imaging and magnetic resonance angiography of the brain were scheduled approximately every 1 to 2 years; studies were reviewed by a panel of neuroradiologists. Eligibility criteria included regularly scheduled blood transfusion therapy. Forty children were included; mean pretransfusion hemoglobin S concentration was 29%. Progressive cerebral infarcts occurred in 45% (18 of 40 children) while receiving chronic blood transfusion therapy; 7 had second overt strokes and 11 had new silent cerebral infarcts. Worsening cerebral vasculopathy was associated with new cerebral infarction (overt or silent; relative risk = 12.7; 95% confidence interval, 2.65-60.5, P = .001). Children with SCD and overt strokes receiving regular blood transfusion therapy experience silent cerebral infarcts at a higher rate than previously recognized. Additional therapies are needed for secondary stroke prevention in children with SCD.

Variability of pulse oximetry measurement over 1 year in children with sickle cell disease depends on initial oxygen saturation measurement.

A prospective cohort of children with sickle cell disease (SCD) was evaluated to determine the variability of daytime pulse oximetry among three measurements over approximately 1 year. Fifty-eight participants were evaluated. Asymptomatic children with initial oxygen saturation < or = 92% had a mean range over 1 year of 4.6% (2.1-7.5%). In contrast, asymptomatic children whose oxygen saturation was >92% had a mean range of 1.9% (0-5.5%). These results suggest, changes in pulse oximetry measurement of approximately 5% may not be clinically significant in otherwise, healthy children with SCD with previous pulse oximetry < or = 92%.

Stability of polysomnography for one year and longer in children with sickle cell disease.

STUDY OBJECTIVES: Serious morbidity may be linked to sleep disordered breathing (SDB) among children with sickle cell disease (SCD). We investigated the stability of polysomnography (PSG) results among children not having acute complications of SCD. METHODS: Two PSGs were performed on a subsample of 63 children 4 to 18 years of age from the Sleep and Asthma Cohort Study. All had Hb SS or HbSß(0) disease. Two PSGs were compared for 45 subjects. Excluded from comparison were 18 children who had begun transfusions or hydroxyurea, had an adenotonsillectomy between the PSGs, or had a pain crisis or the acute chest syndrome within 3 months of the second PSG. Sleep disordered breathing was identified using 2 thresholds for the apnea hypopnea index (AHI): ≥ 2 or ≥ 5 respiratory events per hour. RESULTS: Ages were 12.3 yrs ± 4.0, BMI, 18.2 ± 3.2. Interval between PSGs was 581 ± 119 days (19.1 ± 3.9 months). Ten of 45 changed from ≥ 2 events per hour to < 2; 3 of 45 from < 2 to ≥ 2; 7 of 45 had ≥ 2 on both nights. Six of 45 changed from ≥ 5 to < 5, 2 of 45 from < 5 to ≥ 5, and 1 had ≥ 5 on both nights (McNemar χ(2), p = 0.09, and p = 0.29). CONCLUSIONS: In the absence of acute SCD complications, overnight PSG usually remains stable or improves over a 12- to 30-month period. Only 6.7% subjects, or fewer, had AHI on a subsequent PSG that would re-classify the child as having SDB not identified in the earlier PSG.