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Source: British Journal of Haematology

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Total 34 results found since Jan 2013.

Outcome of overt stroke in sickle cell anaemia, a single institution's experience
Summary Stroke is a traumatic complication in sickle cell anaemia (SCA) that is associated with significant morbidity and a risk of recurrent overt stroke of 2·2–6·4 events per 100 patient‐years. A retrospective study was performed on all paediatric SCA patients diagnosed with a history of overt stroke between 1997 and 2010. A total of 31 children with SCA had new onset overt stroke. The mean age of the active patients (n = 27) was 17·9 years (range 6·8–27·6 years) with a total period of observation of 305 patient‐years. Twenty‐two of 27 (81%) were receiving long term red blood cell transfusions and 16 (...
Source: British Journal of Haematology - February 27, 2014 Category: Hematology Authors: Suvankar Majumdar, Morgan Miller, Majid Khan, Catherine Gordon, Amy Forsythe, Mary G. Smith, Gail Megason, Rathi Iyer Tags: Research Paper Source Type: research

Advances in understanding stroke risk in children – a geneticist's view
Summary The advent of the genomic era has provided novel insights into the genetic architecture of common complex diseases, such as thrombophilia and stroke. Since 2006, a growing number of genome wide association studies (GWAS) for common complex diseases have revealed new candidate loci and genomic regions that play an important role in disease aetiology and progression. While GWAS on the above mentioned traits are abundant in adults, similar studies in paediatric study cohorts are lagging behind. However, genetic research in this important clinical area has gained momentum and starts to provide us with exciting insights...
Source: British Journal of Haematology - December 21, 2013 Category: Hematology Authors: Monika Stoll, Frank Rühle, Ulrike Nowak‐Göttl Tags: State of the Art Review Source Type: research

Silent cerebral infarcts in very young children with sickle cell anaemia are associated with a higher risk of stroke
We report clinical, neuroradiological, psychometric and academic follow‐up over an average period of 14 years in 37 children with SCA who had magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) of the brain between ages 7 and 48 months. Ten patients (27%) younger than age 5 years (Group I) had SCI, as did 12 (32%) older than 5 years (Group II). Fifteen (41%) had no lesions (Group III). Overt stroke or transient ischaemic attack occurred in 5/9 (56%) in Group I. Most Group I patients had progressive MRI abnormalities, concurrent stenosis, decreased cognitive ability, attention/executive function ...
Source: British Journal of Haematology - June 7, 2015 Category: Hematology Authors: Maria I. Cancio, Kathleen J. Helton, Jane E. Schreiber, Matthew P. Smeltzer, Guolian Kang, Winfred C. Wang Tags: Research Paper Source Type: research

Pregnancy loss and risk of ischaemic stroke and myocardial infarction
We investigated whether pregnancy loss increases the risk of arterial thrombosis in young women. Women (age 18–50 years) with ischaemic stroke (IS) or myocardial infarction (MI) and at least one pregnancy were compared for pregnancy loss in a control group. Odds ratios (OR) with 95% confidence intervals (CI), adjusted for matching variables, cardiovascular risk factors, cardiovascular family history and the presence of antiphospholipid antibodies, were calculated for the number of pregnancy losses as well as the type of unsuccessful pregnancy (early miscarriage, late miscarriage and stillbirth). 165 IS cases, 218 MI cas...
Source: British Journal of Haematology - April 6, 2016 Category: Hematology Authors: Alberto Maino, Bob Siegerink, Ale Algra, Ida Martinelli, Flora Peyvandi, Frits R. Rosendaal Tags: Research Paper 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

How I manage cerebral vasculopathy in children with sickle cell disease
Summary Sickle cell disease induces specific brain alterations that involve both the macrocirculation and the microcirculation. The main overt neurovascular complications in children are infarctive stroke, transient ischaemic attack and cerebral haemorrhage. Silent cerebral infarction, cognitive dysfunction and recurrent headache are also common. Cerebrovascular disease selectively affects children with the HbSS or HbS‐β0 genotypes (i.e. sickle cell anaemia). The incidence of stroke peaks between 2 and 5 years of age (1·02/100 patient‐years) and increases with the severity of the anaemia. Most strokes can be prevent...
Source: British Journal of Haematology - May 5, 2015 Category: Hematology Authors: Valentine Brousse, Manoelle Kossorotoff, Mariane Montalembert Tags: State of the Art Review Source Type: research

Haptoglobin, alpha‐thalassaemia and glucose‐6‐phosphate dehydrogenase polymorphisms and risk of abnormal transcranial Doppler among patients with sickle cell anaemia in Tanzania
Summary Transcranial Doppler ultrasonography measures cerebral blood flow velocity (CBFv) of basal intracranial vessels and is used clinically to detect stroke risk in children with sickle cell anaemia (SCA). Co‐inheritance in SCA of alpha‐thalassaemia and glucose‐6‐phosphate dehydrogenase (G6PD) polymorphisms is reported to associate with high CBFv and/or risk of stroke. The effect of a common functional polymorphism of haptoglobin (HP) is unknown. We investigated the effect of co‐inheritance of these polymorphisms on CBFv in 601 stroke‐free Tanzanian SCA patients aged <24 years. Homozygosity for alpha‐t...
Source: British Journal of Haematology - February 22, 2014 Category: Hematology Authors: Sharon E. Cox, Julie Makani, Deogratias Soka, Veline S. L'Esperence, Edward Kija, Paula Dominguez‐Salas, Charles R. J. Newton, Anthony A. Birch, Andrew M. Prentice, Fenella J. Kirkham Tags: Research Paper Source Type: research

Therapeutic phlebotomy is safe in children with sickle cell anaemia and can be effective treatment for transfusional iron overload
Summary Serial phlebotomy was performed on sixty children with sickle cell anaemia, stroke and transfusional iron overload randomized to hydroxycarbamide in the Stroke With Transfusions Changing to Hydroxyurea trial. There were 927 phlebotomy procedures with only 33 adverse events, all of which were grade 2. Among 23 children completing 30 months of study treatment, the net iron balance was favourable (−8·7 mg Fe/kg) with significant decrease in ferritin, although liver iron concentration remained unchanged. Therapeutic phlebotomy was safe and well‐tolerated, with net iron removal in most children who completed 30...
Source: British Journal of Haematology - January 22, 2015 Category: Hematology Authors: Banu Aygun, Nicole A. Mortier, Karen Kesler, Alexandre Lockhart, William H. Schultz, Alan R. Cohen, Ofelia Alvarez, Zora R. Rogers, Janet L. Kwiatkowski, Scott T. Miller, Pamela Sylvestre, Rathi Iyer, Peter A. Lane, Russell E. Ware, Tags: Short Report Source Type: research

Clinical and haematological risk factors for cerebral macrovasculopathy in a sickle cell disease newborn cohort: a prospective study
This study aimed to identify risk factors for CV using longitudinal biological and clinical data in a SCD newborn cohort followed at the Robert Debre Reference centre (n = 375 SS/Sβ0). Median follow‐up was 6·8 years (2677 patient‐years). Among the 59 children presenting with CV, seven had a stroke. Overall, the incidence of CV was 2·20/100 patient‐years [95% confidence interval (95%CI): 1·64–2·76] and the incidence of stroke was 0·26/100 patient‐years (95%CI: 0·07–0·46). The cumulative risk of CV by age 14 years was 26·0% (95%CI: 20·0‐33·3%). Risk factors for CV were assessed by a Cox model enc...
Source: British Journal of Haematology - January 5, 2016 Category: Hematology Authors: Julie Sommet, Corinne Alberti, Nathalie Couque, Suzanne Verlhac, Zinedine Haouari, Damir Mohamed, Martine François, Florence Missud, Laurent Holvoet, Monique Elmaleh, Ghislaine Ithier, André Denjean, Jacques Elion, André Baruchel, Malika Benkerrou Tags: Research Paper 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

Advances in understanding the pathogenesis of cerebrovascular vasculopathy in sickle cell anaemia
Summary Cerebral vasculopathy is the most severe complication to affect children with sickle cell anaemia and its pathophysiology is complex. Traditionally, small‐vessel occlusion by intravascular sickling and sludging was considered to underlie the strokes but, in the last 20 years, progressive major cerebral vessel involvement has become recognized as the principal responsible factor. Macrovasculopathy is well detected by abnormally high velocities on transcranial Doppler and with magnetic resonance angiography (MRA), and is responsible for the majority of overt strokes. Silent infarcts are ischaemic lesions detected ...
Source: British Journal of Haematology - March 18, 2013 Category: Hematology Authors: Philippe Connes, Suzanne Verlhac, Françoise Bernaudin Tags: Review Source Type: research

Paradoxical protection from atherosclerosis and thrombosis in a mouse model of sickle cell disease
Summary Sickle cell disease (SCD) is associated with vascular complications including premature stroke. The role of atherothrombosis in these vascular complications is unclear. To determine the effect of SCD on atherosclerosis and thrombosis, mice with SCD along with controls were generated by transplantation of bone marrow from mice carrying the homozygous sickle cell mutation (Hbbhβs/hβs) or wild‐type mice (Hbb+/+) into C57BL6/J or apolipoprotein E deficient (Apoe−/−) recipient mice. At the time of sacrifice, 23–28 weeks following bone marrow transplantation, anaemia, reticulocytosis, and splenomegaly were pre...
Source: British Journal of Haematology - April 17, 2013 Category: Hematology Authors: Hui Wang, Wei Luo, Jintao Wang, Chiao Guo, Stephanie L. Wolffe, Julia Wang, Eddy B. Sun, Kori N. Bradley, Andrew D. Campbell, Daniel T. Eitzman Tags: Research Paper Source Type: research

Sickle cell disease: time for a closer look at treatment options?
Summary Tremendous progress has been made in the care of individuals with sickle cell over the past several decades. Major successes have been comprehensive infection prophylaxis, prediction and prevention of stroke, and better transfusion care, the latter including both prevention of alloimmunization and treatment of iron overload. However, definitive therapies remain limited to hydroxycarbamide (hydroxyurea) and stem cell transplantation, both of which have been in use for at least two decades. Despite knowing the progressive natural history of the disease with organ dysfunction, failure, and ultimately death at a young ...
Source: British Journal of Haematology - June 15, 2013 Category: Hematology Authors: Sujit Sheth, Maureen Licursi, Monica Bhatia Tags: Review Source Type: research