Association between hydroxycarbamide exposure and neurocognitive function in adolescents with sickle cell disease.
Br J Haematol
; 189(6): 1192-1203, 2020 06.
Article
en En
| MEDLINE
| ID: mdl-32103506
ABSTRACT
Patients with sickle cell disease (SCD) are at increased risk for neurocognitive impairments. While disease-modifying treatment, such as hydroxycarbamide (hydroxyurea), may decrease this risk, it has not been systematically investigated in children with SCD. We screened neurocognitive functioning in 103 adolescents with SCD (16-17 years, 50% female) and compared outcomes between patients with a history of exposure to hydroxycarbamide (n = 12 HbSC/HbSß+ thalassaemia; n = 52 HbSS/HbSß0 thalassaemia) and those never treated with hydroxycarbamide (n = 31 HbSC/HbSß+ thalassaemia; n = 8 HbSS/HbSß0 thalassaemia). Demographic distributions were similar between the groups. After adjusting for socioeconomic status, the hydroxycarbamide group had significantly higher scores on nonverbal IQ (HbSC/HbSß thalassaemia P = 0·036, effect size [d] = 0·65), reaction speed (HbSS/HbSß0 thalassaemia P = 0·002, d = 1·70), sustained attention (HbSS/HbSß0 thalassaemia P = 0·014, d = 1·30), working memory (HbSC/HbSß+ thalassaemia P = 0·034, d = 0·71) and verbal memory (HbSC/HbSß+ thalassaemia P = 0·038, d = 0·84) when compared to those who did not receive hydroxycarbamide. In patients with HbSS/HbSß0 thalassaemia, longer treatment duration with hydroxycarbamide was associated with better verbal memory (P = 0·009) and reading (P = 0·002). Markers of hydroxycarbamide effect, including higher fetal haemoglobin (HbF), higher mean corpuscular volume (MCV) and lower white blood cell count (WBC), were associated with better verbal fluency (HbF P = 0·014, MCV P = 0·006, WBC P = 0·047) and reading (MCV P = 0·021, WBC P = 0·037). Cognitive impairment may be mitigated by exposure to hydroxycarbamide in adolescents with SCD.
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Texto completo:
1
Base de datos:
MEDLINE
Asunto principal:
Atención
/
Trastornos Neurocognitivos
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Anemia de Células Falciformes
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Memoria a Corto Plazo
Tipo de estudio:
Risk_factors_studies
Límite:
Adolescent
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Adult
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Child
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Child, preschool
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Female
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Humans
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Male
Idioma:
En
Revista:
Br J Haematol
Año:
2020
Tipo del documento:
Article
País de afiliación:
Estados Unidos