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Children with sickle cell anemia with normal transcranial Doppler ultrasounds and without silent infarcts have a low incidence of new strokes
Author(s) -
Jordan Lori C.,
Roberts Williams Dionna O.,
Rodeghier Mark J.,
Covert Greene Brittany V.,
Ponisio Maria R.,
Casella James F.,
McKinstry Robert C.,
Noetzel Michael J.,
Kirkham Fenella J.,
Meier Emily R.,
Fuh Beng,
McNaull Melissa,
Sarnaik Sharada,
Majumdar Suvankar,
McCavit Timothy L.,
DeBaun Michael R.
Publication year - 2018
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.25085
Subject(s) - medicine , transcranial doppler , stroke (engine) , sickle cell anemia , incidence (geometry) , hazard ratio , neurological examination , prospective cohort study , pediatrics , anemia , clinical endpoint , cohort , magnetic resonance imaging , cardiology , confidence interval , surgery , clinical trial , radiology , disease , optics , engineering , mechanical engineering , physics
In a prospective cohort study, we tested the hypothesis that children with sickle cell anemia (SCA) with normal transcranial Doppler ultrasound (TCD) velocities and without silent cerebral infarcts (SCIs) would have a lower incidence rate of new neurological events (strokes, seizures or transient ischemic attacks) compared to children with normal TCD measurements and SCIs, not receiving regular blood transfusions. Nonrandomized participants from the silent cerebral infarct transfusion (SIT) Trial who had screening magnetic resonance imaging (MRI) of the brain and normal TCD measurements were included. Follow‐up ended at the time of first neurological event (stroke, seizure or transient ischemic attack), start of regular blood transfusion, or loss to follow‐up, whichever came first. The primary endpoint was a new neurological event. Of 421 participants included, 68 had suspected SCIs. Mean follow‐up was 3.6 years. Incidence rates of new neurological events in nontransfused participants with normal TCD values with SCIs and without SCIs were 1.71 and 0.47 neurological events per 100 patient‐years, respectively, P = .065. The absence of SCI(s) at baseline was associated with a decreased risk of a new neurological event (hazard ratio 0.231, 95% CI 0.062‐0.858; P = .029). Local pediatric neurologists examined 67 of 68 participants with suspected SCIs and identified 2 with overt strokes classified as SCIs by local hematologists; subsequently one had a seizure and the other an ischemic stroke. Children with SCA, without SCIs, and normal TCD measurements have a significantly lower rate of new neurological events when compared to those with SCIs and normal TCD measurements. Pediatric neurology assessment may assist risk stratification.