Defining stroke risk in children with sickle cell anaemia

Abstract

SummarySickle cell anaemia (SCA) is the most common cause of childhood stroke, occurring with the highest frequency before the age of 6 years. Despite the relative frequency of stroke in SCA, few predictors of risk exist. Anaemia, leucocytosis, hypertension, silent infarction, and history of acute chest syndrome are well-documented risk factors for ischaemic stroke in SCA. Recent data suggest that other environmental and genetic factors, many unrelated to SCA, influence the development of cerebrovascular disease. Non-invasive assessment of individual stroke risk using transcranial Doppler ultrasonography has provided a means of selecting and prophylactically treating SCA children at highest risk. With the ultimate goal of preventing stroke, the information gained from the studies reviewed here may lead to improved prediction of stroke so that clinical trials to assess risk-based therapy may be carried out on selected children with SCA.

Stroke Prevention Trial in Sickle Cell Anemia (STOP): extended follow-up and final results

“…23,24 Further improvement in risk stratification using TCD and/or other imaging modalities, or perhaps other clinical or genetic factors, could optimize future preventive therapies for stroke in SCD. 25,26 BLOOD, 1 AUGUST 2006 ⅐ VOLUME 108, NUMBER 3 For personal use only. on May 11, 2018.…”

Neurological Soft Signs as the Stroke Risk in Sickle Cell Disease

“…Another study conducted in SCD patients using PET studies have shown that the presence of metabolic abnormalities particularly in the frontal lobes and areas of low perfusion are undetectable by conventional MRI (Powars et al 1999). Previous MRI-based studies have shown that the affected areas in case of silent infarction are the same as the overt stroke in patients with SCD (Hoppe 2005). The most affected lobe was frontal lobe (81%), followed by the parietal lobe (45%), the basal ganglia or thalamus (16%), and the temporal lobe (9%) of SCD patients with a history of overt stroke (Ohene-Frempong et al 1998;Pegelow et al 2001Pegelow et al , 2002.…”

“…These patients with silent lesions predispose to the development of new or progressive neurological silent lesions (Wang et al 1998). Furthermore, among the various clinical and laboratory parameters, the presence of silent infarctions on MRI was the strongest independent risk factor for stroke and was associated with a 14 fold increased risk factor of developing overt stroke (Kugler et al 1993;Pegelow et al 2002;Hoppe 2005). Kral et al (2003) have reported that the frontal lobes of the brain were most vulnerable to cerebro-vascular injury in SCD patients.…”

Acidente vascular cerebral e outras complicações do sistema nervoso central nas doenças falciformes

“…Os programas de recuperação neurofuncional são aparentemente bem sucedidos. 7 O quadro 1 (Hoppe, 2004) 8 compara as duas entidades.…”