Neurologic complications in children under five years with sickle cell disease

Abstract

Introduction: Sickle Cell Disease (SCD) is one of the most common genetic diseases in the world affecting every organ. The major challenge in the medical care of children with SCD is preventing end-organ dysfunction, particularly the brain. Major neurologic complications in children less than five years with SCD include, but are not limited to, Silent cerebral infarct, cerebral sinus thrombosis, epilepsy, reversible encephalopathy syndrome, and ischemic and hemorrhagic stroke. Recurrent headaches and migraine are not rare in children under five years with SCD. This review will focus on the neurologic complications and the description of the modifiable risk factors in children less than 5 years of age with emphasis on differences between high and low resource settings.Areas covered: Neurologic complications of children under 5 years of age and the modifiable risk factors. The PUBMED database was searched using medical subject headings (MeSH) and keywords for articles regarding neurologic complications in children under 5 years of age. Conclusion:Neurologic complications in children under five years of age with SCD may be more frequent than currently reported, among which Silent cerebral infarct and cognitive impairment are the most common.

Transcranial Doppler screening in Nigerian children with sickle cell disease: A 10‐year longitudinal study on the SPPIBA cohort

“…Stroke remains one of the most severe and most disabling complications of SCD 26,27 . In high‐income countries, the institution of routine TCD screening in children with SCD plus the administration of chronic transfusions in those at high risk of stroke has been described as an unprecedented breakthrough in the field of sickle cell‐related brain injury, with more than 10‐fold decline in the incidence of stroke in SCD 28,29 .…”

Silent Strokes May Be Involved in Neurocognitive Impairment in Children With Sickle Cell Disease

“…Moreover, seizures and neuropsychomotor developmental delay were rare ndings (11.76%). This is interesting data since neuropsychomotor developmental delays in children with SCD are widely reported in the literature [10,11].…”

“…Nonetheless, another factor should be considered as a potential confounder, in uencing the statistical results: patients who had small brain infarcts that could not be identi ed by TDU examinations. Although the stroke detection protocol used in this study has shown to be effective according to the literature, there are more advanced techniques, such as computed tomography angiography, that are highly sensitive for identifying small brain infarcts [10]. Therefore, cognitive impairments may be associated with silent infarcts, as they cause brain micro-injuries [12].…”

Matched sibling donor stem cell transplantation for sickle cell disease: Results from the Spanish group for bone marrow transplantation in children

“…Increasing age at transplantation has been associated with increased risk for alloimmunization, iron overload, and irreversible complications such as stroke, pulmonary hypertension, nephropathy, or osteonecrosis, and loss of school performance. 38 Furthermore, older patients present an increased transplant-related mortality due to higher risk of rejection, chronic GvHD, 24,26 and endothelial complications. But Vermyeln et al, when back in 1998, demonstrated that the best results were obtained in the younger group of patients as they were at an early stage of the disease.…”