How I manage sickle cell patients with high transcranial doppler results

Brewin, John; Kaya, Burak; Chakravorty, Subarna

doi:10.1111/bjh.14850

Cited by 8 publications

(11 citation statements)

References 93 publications

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“…The transducer orientation was optimised at every 2mm interval in each vessel to ensure that the highest, audible Doppler frequency signal was obtained. The same scanning approach and STOP classification was adopted for imaging TCD based on previous studies that have shown good agreement between velocity measured by imaging and non-imaging TCD modalities [25]. The colour flow anatomical information provided by imaging TCD was used to aid vessel localisation, after which the highest audible Doppler frequency was recorded following appropriate transducer optimisation.…”

Section: Post-training Standardised Tcd Practicementioning

confidence: 99%

An Educational Study Promoting the Delivery of Transcranial Doppler Ultrasound Screening in Paediatric Sickle Cell Disease: A European Multi-Centre Perspective

Inusa

Sainati

Macmahon

et al. 2019

JCM

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Background: Effective stroke prevention in sickle cell disease (SCD) is recommended for children with sickle cell anaemia. Effective implementation relies on the correct stratification of stroke risk using Transcranial Doppler Ultrasound (TCD), prior to committing children to long-term treatment with transfusion. Nevertheless, less than 50% of children with SCD in Europe receive annual TCD—one of the reasons being a lack of trained personnel. The present European multi-centre study was designed to promote the standardisation and delivery of effective screening. Methods: Fifty-five practitioners from differing professional backgrounds were recruited to the TCD training program. The impact of the training programme was evaluated in three European haematology clinics by comparing stroke risk classification and middle cerebral artery time-averaged maximum velocity (TAMMV) obtained from a cohort of 555 patients, before and after training. Results: 42% (23/55) of trainees successfully completed the program. The TAMMV, used to predict stroke risk at each Centre, demonstrated the highest values in Centre 3 (p < 0.0001) before training. The imaging-TCD TAMMV was also higher in Centre 3 (p < 0.001). Following training, the TAMMV showed closer agreement between centres for both imaging-TCD and non-imaging TCD. The stroke risk distribution of children at each centre varied significantly before training (p < 0.001), but improved after training (Fisher’s Exact: no treatment = 5.6, p = 0.41, treatment = 13.8, p < 0.01). The same consistency in stroke risk distribution following training was demonstrated with both non-imaging and imaging-TCD data. Conclusion: The attainment of competency in stroke screening using transcranial Doppler scanning (TCD) in sickle cell disease is more feasible for professionals with an ultrasound imaging background. A quality assurance (QA) system is required to ensure that standards are maintained. Further work is in progress to develop an achievable and reproducible QA program.

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Section: Post-training Standardised Tcd Practicementioning

confidence: 99%

An Educational Study Promoting the Delivery of Transcranial Doppler Ultrasound Screening in Paediatric Sickle Cell Disease: A European Multi-Centre Perspective

Inusa

Sainati

Macmahon

et al. 2019

JCM

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“…Over the past decade, the optimization of comprehensive care and treatment has led to some advances in the management of pediatric SCD 5,6 . Of note, the effectiveness of transcranial Doppler (TCD) ultrasonography in stratifying stroke risk in patients with SCD has been confirmed by randomized controlled trials, which has established the use of TCD screening and monitoring for stroke prevention in children with SCD 2,7,8 . Generally, abnormal (≥200 cm/s) or persistently high conditional (≥170 to <200 cm/s) cerebral artery blood flow velocities, as assessed by TCD, are an indication to start a chronic transfusion regimen and to consider disease‐curative options 2,4,8,9 .…”

Section: Introductionmentioning

confidence: 99%

“…It is well known that children with sickle cell disease (SCD) are at high risk for ischemic stroke in the first decade of life. 1,2 Of the many acute and chronic complications of SCD, stroke can be particularly devastating for pediatric patients, given its potential to cause physical and neurocognitive deficits. 3,4 Over the past decade, the optimization of comprehensive care and treatment has led to some advances in the management of pediatric SCD.…”

Section: Introductionmentioning

confidence: 99%

“…5,6 Of note, the effectiveness of transcranial Doppler (TCD) ultrasonography in stratifying stroke risk in patients with SCD has been confirmed by randomized controlled trials, which has established the use of TCD screening and monitoring for stroke prevention in children with SCD. 2,7,8 Generally, abnormal (≥200 cm/s) or persistently high conditional (≥170 to <200 cm/s) cerebral artery blood flow velocities, as assessed by TCD, are an indication to start a chronic transfusion regimen and to consider disease-curative options. 2,4,8,9 In case of stable normalization of TCD velocities after 1 year of transfusions, switching to hydroxyurea (HU) treatment can be considered in the absence of stenosis with magnetic resonance angiography (MRA).…”

Section: Introductionmentioning

confidence: 99%

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Relationship between hemoglobin, hemolysis, and transcranial Doppler velocities in children with sickle cell disease: Results from a long‐term natural history study in Italy in the era of multimodal therapy

Reggiani

Coppadoro

Munaretto

et al. 2023

European J of Haematology

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Background Stroke and cerebral vasculopathy are leading causes of morbidity and mortality in patients with sickle cell disease (SCD). Transcranial Doppler (TCD) is a reliable and validated predictor of stroke risk. Children with conditional or abnormal TCD are at an increased risk for stroke, which can be mitigated by red blood cell transfusion or hydroxyurea. Elucidating the relationship between cerebral hemodynamics and hemolytic anemia can help identify novel therapeutic approaches to reduce stroke risk and transfusion dependence. Methods This long‐term, real‐world study was designed to evaluate the prevalence of TCD imaging (TCDi)‐assessed flow velocities in children and to interrogate their relationship with markers of anemia and hemolysis. Results In total, 155 children (median follow‐up 79.8 months, 1358.44 patient‐years) had 583 evaluable TCDi results. Only patients with HbSS or HbSβ0 had abnormal (1.6%) or conditional (10.9%) TCDi. Children with abnormal or conditional TCDi had lower hemoglobin (Hb) and higher hemolysis markers. A linear correlation was detected between TCD velocity and Hb: an Hb increase of 1 g/dL corresponded to decreases in velocity in the internal carotid and middle cerebral arteries (6.137 cm/s and 7.243 cm/s). Moreover, patients with Hb >9 g/dL presented a lower risk of TCDi‐associated events. Conclusion These results support the need to optimize disease‐modifying treatments that increase Hb and reduce hemolysis for stroke prevention in young children with SCD.

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“…In the absence of early Transcranial Doppler (TCD) screening, strokes occur in 7.4-11% of patients with SCA before age 20 (5)(6)(7). As the risk of stroke persists throughout childhood (8), TCD screening has been implemented to detect SCA children at risk, in order to initiate transfusion programs known for significantly reducing stroke incidence in SCA patients with an abnormal TCD (9)(10)(11)(12).…”

Section: Introductionmentioning

confidence: 99%

Low Stroke Risk in Children With Sickle Cell Disease in French Guiana: A Retrospective Cohort Study

Gargot

Parriault²,

Adenis³

et al. 2022

Front. Med.

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One in every 227 babies born in French Guiana has sickle cell disease, which represents the greatest incidence in France. This study aimed to determine the incidence of stroke in children with sickle cell disease and its associated risk factors. This retrospective cohort study included all children with sickle cell disease diagnosed in the neonatal period who were born in French Guiana between 01/01/1992 and 12/31/2002. Of a total of 218 records, 122 patients were included. There were 70 HbSS/Sβ0 (58%), 40 HbSC (33%), and 11 Sβ + thalassemia (9%). The number of emergency admissions was significantly different between genotypes, with a higher number in SS/Sβ0 children (p = 0.004). There were significantly more acute chest syndromes (p = 0.006) and more elevated Lactate Dehydrogenase in SS/Sβ0 patients (p = 0.003). Three of these patients had ischemic strokes at a mean age of 6.9 years, and one had a hemorrhagic stroke at the age of 9,2 years. The incidence rate of ischemic stroke for SS/Sβ0 children was 3.1 (95% CI: 1.0–9.7) per 1,000 patient-years, and the clinically apparent stroke risk by the age of 15 years and 3 months was 6,4%. The incidence of hemorrhagic stroke was 1.1 (95% CI: 0.1–7.4) per 1,000 patients-years. No patient with SC or Sβ + thalassemia genotypes experienced any stroke.

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How I manage sickle cell patients with high transcranial doppler results

Cited by 8 publications

References 93 publications

An Educational Study Promoting the Delivery of Transcranial Doppler Ultrasound Screening in Paediatric Sickle Cell Disease: A European Multi-Centre Perspective

An Educational Study Promoting the Delivery of Transcranial Doppler Ultrasound Screening in Paediatric Sickle Cell Disease: A European Multi-Centre Perspective

Relationship between hemoglobin, hemolysis, and transcranial Doppler velocities in children with sickle cell disease: Results from a long‐term natural history study in Italy in the era of multimodal therapy

Low Stroke Risk in Children With Sickle Cell Disease in French Guiana: A Retrospective Cohort Study

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