Transcranial Doppler sonography in children with sickle cell disease and silent ischemic lesions

Farina, Filippo; Rampazzo, Patrizia; Sainati, Laura; Manara, Renzo; Onofri, Angelo; Colombatti, Raffaella; Baracchini, Claudio; Meneghetti, Giorgio

doi:10.1016/j.permed.2012.02.046

Cited by 2 publications

(6 citation statements)

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“…The majority of studies excluded patients with abnormal transcranial Doppler (TCD) flow velocities (> 170 cm/s) indicative of an increased risk of overt stroke. Most studies that concomitantly measured TCD velocities found no significant differences between the mean TCD velocities of patients with normal MRI scans and patients with detected SCIs [6,23,[57][58][59][60][61][62][63][64]. Moreover, comparison of patients who were included in both the Cooperative Study of SCD (CSSCD) and the Stroke Prevention (STOP) trial showed that patients with abnormal TCDs did not demonstrate a concurrent high prevalence of SCIs.…”

Section: Additional Neuroimaging Techniques Transcranial Dopplermentioning

confidence: 99%

Silent cerebral infarcts in patients with sickle cell disease: a systematic review and meta-analysis

Houwing

Grohssteiner

Dremmen

et al. 2020

BMC Med

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Background and purpose Silent cerebral infarcts (SCIs) are the most common neurological complication in children and adults with sickle cell disease (SCD). In this systematic review, we provide an overview of studies that have detected SCIs in patients with SCD by cerebral magnetic resonance imaging (MRI). We focus on the frequency of SCIs, the risk factors involved in their development and their clinical consequences. Methods The databases of Embase, MEDLINE ALL via Ovid, Web of Science Core Collection, Cochrane Central Register of Trials via Wiley and Google Scholar were searched from inception to June 1, 2019. Results The search yielded 651 results of which 69 studies met the eligibility criteria. The prevalence of SCIs in patients with SCD ranges from 5.6 to 80.6% with most studies reported in the 20 to 50% range. The pooled prevalence of SCIs in HbSS and HbSβ0 SCD patients is 29.5%. SCIs occur more often in patients with the HbSS and HbSβ0 genotype in comparison with other SCD genotypes, as SCIs are found in 9.2% of HbSC and HbSβ+ patients. Control subjects showed a mean pooled prevalence of SCIs of 9.8%. Data from included studies showed a statistically significant association between increasing mean age of the study population and mean SCI prevalence. Thirty-three studies examined the risk factors for SCIs. The majority of the risk factors show no clear association with prevalence, since more or less equal numbers of studies give evidence for and against the causal association. Conclusions This systematic review and meta-analysis shows SCIs are common in patients with SCD. No clear risk factors for their development were identified. Larger, prospective and controlled clinical, neuropsychological and neuroimaging studies are needed to understand how SCD and SCIs affect cognition.

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Section: Additional Neuroimaging Techniques Transcranial Dopplermentioning

confidence: 99%

Silent cerebral infarcts in patients with sickle cell disease: a systematic review and meta-analysis

Houwing

Grohssteiner

Dremmen

et al. 2020

BMC Med

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“…An autosomal recessive mutation in the haemoglobin gene leads to the expression of sickle cell haemoglobin (HbS). The sickle cell hemoglobin arises from a point mutation in the β-globin chain of hemoglobin on chromosome 11 at position six by an amino acid substitution, resulting in the synthesis of valine (GTG) instead of glutamic acid (GAG) [ 3 – 5 ]. The pathophysiology of SCD results from the polymerization of HbS in the deoxygenated state [ 4 – 12 ].…”

Section: Introductionmentioning

confidence: 99%

“…SCD is endemic in Sub-Saharan Africa with around 200,000 births annually [ 3 , 13 ]. Affected are also parts of India, Saudi Arabia, Turkey and neighboring countries in the Middle East [ 7 , 12 , 13 ].…”

Section: Introductionmentioning

confidence: 99%

“…Transcranial Doppler sonography (TCD) is the gold standard to assess blood flow velocities in the vessels of the Circle of Willis in these patients [ 4 , 6 , 16 , 20 ]. In a landmark study, the STOP trial (stroke prevention trial in sickle cell anemia), the risk of stroke was found to be predictable measuring time-averaged maximum mean velocity (TAMMV) in the basal brain supplying arteries [ 3 , 18 ]. A variety of factors in SCD seem to contribute to the development of steno-occlusive lesions in the macro- and, to a lesser degree, microcirculation: altered blood rheology leading to decreased nitric oxide production, endothelial dysfunction and inflammation, hypoxia with upregulation of several endothelial adhesion molecules, severity of hypoxia increasing blood flow and post-stenotic shear stress, and G6PD deficiency all of which leading to endothelial hyperplasia and increase of media thickness [ 21 ].…”

Section: Introductionmentioning

confidence: 99%

“…Children with blood flow velocities > 200 cm/s in the MCA and distal ICA have an increased risk of approximately 40% of developing a stroke within the following three years [ 16 , 18 , 22 , 23 ]. Thus, TCD of the Circle of Willis is highly recommended as a routine screening tool and should be applied annually from the second to the 16th year of life [ 3 , 7 , 16 , 23 ]. Abnormal TCD resulted in inclusion to exchange transfusions programs and reduced the incidence of stroke by up to 95% [ 3 , 8 , 17 , 18 ].…”

Section: Introductionmentioning

confidence: 99%

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Transcranial Doppler sonography and the effect of haematopoietic stem cell transplantation in sickle cell disease

Thurn

Kleinschmidt

Kovacic

et al. 2022

Neurol. Res. Pract.

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Background Sickle cell disease (SCD) is one of the most prevalent monogenetic diseases worldwide and one of the most serious complications is stroke. Transcranial Doppler (TCD) demonstrated to be highly predictive for an imminent stroke by measuring blood flow velocities in the basal cerebral arteries. Currently, the only curative therapy for SCD is hematopoietic stem cell transplantation (HSCT). The aim of this study is to verify the correlation between blood flow velocities and stroke including the effect of HSCT. Methods In our retrospective single-center study a total of 26 sickle cell patients (HbSS, HbSß+-thalassemia, HbSSα-thalassemia minima, HbSSα-thalassemia minor and HbSC) were analyzed between 2010 and 2016. The highest time averaged maximum mean blood flow velocity (TAMMV) measured was documented and evaluated with respect to SCD genotype and effect of HSCT. Acute and symptomatic as well as silent strokes were recorded as separate parameters. Results In our study, ten patients had normal blood flow velocities before HSCT (six HbSS and four HbSß+-thalassemia patients) and 13 patients presented with abnormal TCD (eight HbSS, three HbSSα-thalassemia minima, one HbSSα-thalassemia minor and one HbSC). Thirteen of 26 study participants (ten HbSS and three HbSß+-thalassemia patients) received HSCT. In two patients, TAMMV in basal cerebral arteries remained “normal”, in one they remained conditional and in one TAMMV was reduced to normal. Four of 26 study participants (15.4%), including all patients with HbSS genotype, presented with a stroke, but none had “abnormal” TAMMV with TCD performed after the onset of stroke in each case. At the time we performed the TCD, the patients had already suffered the stroke. Conclusion In our study, none of the patients with stroke displayed abnormal blood flow velocities in TCD. Yet, HSCT at this stage of the disease still had a positive effect on TAMMV. Further studies are needed whether this effect converts into reduced stroke risk at all or only selected SCD patients undergoing HSCT.

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Transcranial Doppler sonography in children with sickle cell disease and silent ischemic lesions

Cited by 2 publications

References 8 publications

Silent cerebral infarcts in patients with sickle cell disease: a systematic review and meta-analysis

Silent cerebral infarcts in patients with sickle cell disease: a systematic review and meta-analysis

Transcranial Doppler sonography and the effect of haematopoietic stem cell transplantation in sickle cell disease

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