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What drives transcranial Doppler velocity improvement in paediatric sickle cell anaemia: analysis from the Sickle Cell Clinical Research and Intervention Program (SCCRIP) longitudinal cohort study
Author(s) -
Estepp Jeremie H.,
Cong Ze,
Agodoa Irene,
Kang Guolian,
Ding Juan,
McCarville M. Beth,
Hankins Jane S.,
Wang Winfred C.
Publication year - 2021
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.17620
Subject(s) - transcranial doppler , medicine , fetal hemoglobin , sickle cell anemia , observational study , stroke (engine) , cohort , cohort study , pediatrics , fetus , pregnancy , biology , disease , genetics , mechanical engineering , engineering
Summary Children with sickle cell anaemia (SCA) and conditional transcranial Doppler (TCD) flow velocities (conditional: 170–199 cm/s; normal: <170 cm/s) have an increased risk of stroke. The Sickle Cell Clinical Research and Intervention Program (SCCRIP), a lifetime observational study, assessed the influence of haematological markers on TCD velocities. In children (≤16 years) with SCA (HbSS/HbSβ 0 ‐thalassaemia) and conditional TCD velocities ( n = 32), increases in haemoglobin and in fetal haemoglobin after hydroxyurea initiation were significantly associated with decreases in TCD velocities. The benefit of pharmacological intervention to increase haemoglobin and fetal haemoglobin and normalise TCD velocities was demonstrated in this real‐world dataset.