TY - JOUR
T1 - Cerebral arterial lumens are enlarged in children and young adults with sickle cell disease compared to peers
AU - Lewis, Josiah B.
AU - Fields, Melanie E.
AU - Binkley, Michael M.
AU - Zhou, Anita
AU - Mirro, Amy
AU - Ouyang, Amy
AU - Gupta, Niket
AU - Chen, Yasheng
AU - Fellah, Slim
AU - Smith, Alyssa E.
AU - Dedkov, Igor
AU - Hulbert, Monica L.
AU - Ford, Andria L.
AU - An, Hongyu
AU - Lee, Jin Moo
AU - Goyal, Manu S.
AU - Guilliams, Kristin P.
N1 - Publisher Copyright:
© 2025 The Authors
PY - 2025/6
Y1 - 2025/6
N2 - Children with sickle cell disease (SCD) may develop large vessel narrowing, but studies suggest vessels may also be enlarged, possibly related to increased cerebral blood flow (CBF). We used MRI to investigate whether the cross-sectional total inflow vessel luminal area (TIVLA) proximal to the circle of Willis (carotid arteries + basilar artery) would be increased in SCD compared to age- and sex-matched peers after adjusting for CBF. Across 36 children with SCD (19 female, median age 10.7 [8.0–14.5] years and 43 controls (26 female, median age 12.7 [9.2–18.2] years) matched by age (p = 0.13) and sex (p = 0.50), the median TIVLA in the SCD group (35.9 mm2 [30.7, 39.5]) was larger than controls (30.5 mm2 [27.8, 35.4], p = 0.002). In a mixed model including age, sex, hemoglobin, CBF, SCD status, and an interaction between hemoglobin and SCD status, CBF (β = 0.11, CI 0.02–0.20, p = 0.02), SCD (β = 28.02, CI 5.62–50.42, p = 0.015), and the interaction between SCD and hemoglobin (β = −2.48, CI −4.49 to −0.47, p = 0.018) were all significantly associated with increased TIVLA. Notably, TIVLA as a measure of arterial lumens is larger in children with SCD, even after adjusting for CBF in the mixed model. This implies disease-specific normative values may be needed to detect early vasculopathy.
AB - Children with sickle cell disease (SCD) may develop large vessel narrowing, but studies suggest vessels may also be enlarged, possibly related to increased cerebral blood flow (CBF). We used MRI to investigate whether the cross-sectional total inflow vessel luminal area (TIVLA) proximal to the circle of Willis (carotid arteries + basilar artery) would be increased in SCD compared to age- and sex-matched peers after adjusting for CBF. Across 36 children with SCD (19 female, median age 10.7 [8.0–14.5] years and 43 controls (26 female, median age 12.7 [9.2–18.2] years) matched by age (p = 0.13) and sex (p = 0.50), the median TIVLA in the SCD group (35.9 mm2 [30.7, 39.5]) was larger than controls (30.5 mm2 [27.8, 35.4], p = 0.002). In a mixed model including age, sex, hemoglobin, CBF, SCD status, and an interaction between hemoglobin and SCD status, CBF (β = 0.11, CI 0.02–0.20, p = 0.02), SCD (β = 28.02, CI 5.62–50.42, p = 0.015), and the interaction between SCD and hemoglobin (β = −2.48, CI −4.49 to −0.47, p = 0.018) were all significantly associated with increased TIVLA. Notably, TIVLA as a measure of arterial lumens is larger in children with SCD, even after adjusting for CBF in the mixed model. This implies disease-specific normative values may be needed to detect early vasculopathy.
KW - Arterial spin labeling
KW - Brain development
KW - Cerebral blood flow
KW - Cerebrovascular disease
KW - Hematology
KW - MR angiography
KW - MRI
UR - http://www.scopus.com/inward/record.url?scp=105004347903&partnerID=8YFLogxK
U2 - 10.1016/j.ynirp.2025.100265
DO - 10.1016/j.ynirp.2025.100265
M3 - Article
AN - SCOPUS:105004347903
SN - 2666-9560
VL - 5
JO - Neuroimage: Reports
JF - Neuroimage: Reports
IS - 2
M1 - 100265
ER -