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Effects of hydroxyurea on brain function in children with sickle cell anemia
Author(s) -
Wang Winfred C.,
Zou Ping,
Hwang Scott N.,
Kang Guolian,
Ding Juan,
Heitzer Andrew M.,
Schreiber Jane E.,
Helton Kathleen,
Hankins Jane S.
Publication year - 2021
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.29254
Subject(s) - medicine , neurocognitive , transcranial doppler , cerebral blood flow , deoxygenated hemoglobin , sickle cell anemia , cardiology , anesthesia , hemoglobin , cognition , psychiatry , disease
Sickle cell anemia (SCA) results in numerous adverse effects on the brain, including neurocognitive dysfunction. Hydroxyurea has been utilized extensively for management of SCA, but its effects on brain function have not been established. Methods We examined prospectively the effects of 1 year of treatment with hydroxyurea on brain function in children with SCA (HbSS/HbSβ 0 ‐thalassemia) by baseline and exit evaluations, including comprehensive neurocognitive testing, transcranial Doppler ultrasound (TCD), and brain MRI (silent cerebral infarcts [SCI], gray matter cerebral blood flow [GM‐CBF], and blood oxygen level‐dependent [BOLD] signal from visual stimulation). Results Nineteen patients with SCA, mean age 12.4 years (range 7.2–17.8), were evaluated. At baseline, subjects had these mean values: full‐scale IQ (FSIQ) 82.8, TCD velocity 133 cm/s, GM‐CBF 64.4 ml/100 g/min, BOLD signal 2.34% increase, and frequency of SCI 47%. After 1 year of hydroxyurea, there were increases in FSIQ (+2, p = .059) and reading passage comprehension (+4, p = .033), a significant decrease in TCD velocity (−11 cm/s, p = .007), and no significant changes in GM‐CBF, BOLD, or SCI frequency. Hemoglobin F (HbF) was associated with passage comprehension, hemoglobin with lower TCD velocity, and lower GM‐CBF with greater working memory. Higher BOLD signal was associated with higher processing speed and lower TCD velocity with higher math fluency. Discussion Improvements in neurocognition and decreased TCD velocity following 1 year of treatment support hydroxyurea use for improving neurocognitive outcomes in SCA. Understanding the mechanisms of benefit, as indicated by relationships of neurocognitive function with HbF, hemoglobin, and CBF, requires further evaluation.