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Transcranial Doppler Screening in a Regional Care Network for Sickle Cell Disease in the United Kingdom
The risk of stroke in children screened with transcranial Doppler ultrasound in the United Kingdom is not known. We evaluated a clinician-led program using a risk assessment modified from the STOP protocol. High-risk classification included abnormal velocities in the anterior cerebral artery, and single abnormal scan if initial velocity>220 cm/s (high abnormal) or if preceded by at least 2 conditional scans. In total, 1653 scans were performed in 542 children, followed for 2235 patient-years. Fifty-eight (10.7%) high-risk subjects were identified, including 18 (31%) with high abnormal, and 15 (26%) with previous conditiona...
Source: Journal of Pediatric Hematology Oncology - September 30, 2016 Category: Hematology Tags: Original Articles Source Type: research

How I manage sickle cell patients with high transcranial doppler results
Summary Stroke is one of the most severe complications to affect children with sickle cell anaemia (SCA). Transcranial doppler (TCD) is an accurate and non‐invasive method to determine stroke risk. Randomised controlled trials have demonstrated the efficacy of chronic transfusion therapy in stroke prevention based on risk stratification determined by TCD velocities. This has led to the regular use of TCD monitoring for children with SCA in order to determine stroke risk. Significant resource allocation is necessary to facilitate training, quality assurance and failsafe arrangements for non‐attenders. In a subgroup of p...
Source: British Journal of Haematology - August 2, 2017 Category: Hematology Authors: John Brewin, Banu Kaya, Subarna Chakravorty Tags: Review Source Type: research

Intracranial 4D flow magnetic resonance imaging reveals altered haemodynamics in sickle cell disease
Summary Stroke risk in children with sickle cell disease (SCD) is currently assessed with routine transcranial Doppler ultrasound (TCD) measurements of blood velocity in the Circle of Willis (CoW). However, there is currently no biomarker with proven prognostic value in adult patients. Four‐dimensional (4D) flow magnetic resonance imaging (MRI) may improve risk profiling based on intracranial haemodynamics. We conducted neurovascular 4D flow MRI and blood sampling in 69 SCD patients [median age 15 years (interquartile range, IQR: 12–50)] and 14 healthy controls [median age 21 years (IQR: 18–43)]. We measured veloci...
Source: British Journal of Haematology - December 21, 2017 Category: Hematology Authors: Lena V áclavů, Zelonna A. V. Baldew, Sanna Gevers, Henri J. M. M. Mutsaerts, Karin Fijnvandraat, Marjon H. Cnossen, Charles B. Majoie, John C. Wood, Ed VanBavel, Bart J. Biemond, Pim Ooij, Aart J. Nederveen Tags: Research Paper Source Type: research

How I manage red cell transfusions in patients with sickle cell disease
Summary Sickle cell disease is one of the commonest serious inherited diseases in the world, and red cell transfusion is still one of the few effective treatments for acute and chronic complications. Transfusion corrects anaemia and dilutes out the number of red cells able to cause vaso‐occlusion and vascular damage. Urgent red cell transfusions are used to correct acute anaemia, treat acute chest syndrome and patients with acute neurological symptoms. We use elective transfusions preoperatively for moderate risk surgery, and in some pregnant women. There is good evidence for the use of long‐term regular transfusions i...
Source: British Journal of Haematology - January 29, 2018 Category: Hematology Authors: David C. Rees, Susan Robinson, Jo Howard Tags: Annotation Source Type: research

Transcranial Doppler velocity among Jamaican children with sickle cell anaemia: determining the significance of haematological values and nutrition
This study investigated the association of nutritional and haematological variables with maximum time‐averaged mean velocity (TAMV) measured by transcranial Doppler (TCD) velocity and the agreement of classification between two protocols. TCD categories included: normal (<170 cm/s), conditional (170–199 cm/s) and abnormal (≥200 cm/s) based on TAMV in distal internal carotid artery (dICA), middle cerebral artery (MCA), internal carotid bifurcation, anterior and posterior cerebral arteries. Of 358 children with sickle cell anaemia (SCA) examined, the mean age (±standard deviation) was 7·4 ± 2·7 years; 13·...
Source: British Journal of Haematology - March 1, 2018 Category: Hematology Authors: Angela E. Rankine ‐Mullings, Nadine Morrison‐Levy, Deanne Soares, Karen Aldred, Lesley King, Susanna Ali, Jennifer M. Knight‐Madden, Margaret Wisdom‐Phipps, Robert J. Adams, Russell E. Ware, Marvin Reid Tags: Research Paper Source Type: research

Interleukin ‐6 G‐174C polymorphism predicts higher risk of stroke in sickle cell anaemia
British Journal of Haematology, EarlyView.
Source: British Journal of Haematology - May 23, 2017 Category: Hematology Source Type: research

Hydroxyurea Therapy Does Not Impact Current Survival Estimates in the East London Sickle Cell Newborn Cohort Study
Conclusions: The study is the first to evaluate survival in a newborn cohort past the age of 20 years. We have confirmed a low mortality rate in childhood, but increased number of deaths in young adults including those with HbSC. This study provides evidence of the benefit of newborn screening and comprehensive care which is accessible at all ages, free of charge. The uptake of HU, particularly at a young age, has been low and a positive effect of HU on survival as described in other studies has not yet been observed in this cohort. Earlier initiation of disease-modifying treatment and longer-term follow-up will be require...
Source: Blood - November 21, 2018 Category: Hematology Authors: Telfer, P., Kaya, B., Tsitsikas, D. A., Barroso, F., Sangarappillai, C. Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Poster III Source Type: research