Genetic determinants and stroke in children with sickle cell disease

Rodrigues, Dow; Ribeiro, Luíz Cláudio; Sudário, Lysla Cardoso; Teixeira, Maria T.B.; Martins, Marina Lobato; Pittella, Anuska M.O.L.; Fernandes, Irtis de O.

doi:10.1016/j.jpedp.2016.06.003

Cited by 1 publication

(1 citation statement)

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“…In addition, there is an independent haplotype in India and Saudi Arabia [ 211 ]. However, the majority of the available data suggests that the β-haplotypes are not associated with stroke [ 93 , 212 , 213 , 214 , 215 ] and, although there have been few studies in Africa, there is currently no evidence that there are large difference in prevalence of stroke between populations [ 4 , 47 ]. Stroke risk is reduced in the presence of co-inherited alpha-thalassemia [ 85 , 215 , 216 ].…”

Section: Risk Factors For Neurological Cognitive and Neuroradiological Abnormalitiesmentioning

confidence: 99%

Epidemiology of Stroke in Sickle Cell Disease

Kirkham

Lagunju

2021

JCM

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Sickle cell disease is the most common cause of stroke in childhood, both ischaemic and haemorrhagic, and it also affects adults with the condition. Without any screening or preventative treatment, the incidence appears to fall within the range 0.5 to 0.9 per 100 patient years of observation. Newborn screening with Penicillin prophylaxis and vaccination leading to reduced bacterial infection may have reduced the incidence, alongside increasing hydroxyurea prescription. Transcranial Doppler screening and prophylactic chronic transfusion for at least an initial year has reduced the incidence of stroke by up to 10-fold in children with time averaged mean of the maximum velocity >200 cm/s. Hydroxyurea also appears to reduce the incidence of first stroke to a similar extent in the same group but the optimal dose remains controversial. The prevention of haemorrhagic stroke at all ages and ischaemic stroke in adults has not yet received the same degree of attention. Although there are fewer studies, silent cerebral infarction on magnetic resonance imaging (MRI), and other neurological conditions, including headache, epilepsy and cognitive dysfunction, are also more prevalent in sickle cell disease compared with age matched controls. Clinical, neuropsychological and quantitative MRI screening may prove useful for understanding epidemiology and aetiology.

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