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Sickle cell cerebral vasculopathy

Angela E Rankine-Mullings, Courtney R Little, Marvin E Reid, Deanne P Soares, Carolyn Taylor-Bryan, Jennifer M Knight-Madden, Susan E Stuber, Asha V Badaloo, Karen Aldred, Margaret E Wisdom-Phipps, Teresa Latham, Russell E Ware
BACKGROUND: Cerebral vasculopathy in sickle cell anemia (SCA) begins in childhood and features intracranial arterial stenosis with high risk of ischemic stroke. Stroke risk can be reduced by transcranial doppler (TCD) screening and chronic transfusion therapy; however, this approach is impractical in many developing countries. Accumulating evidence supports the use of hydroxyurea for the prevention and treatment of cerebrovascular disease in children with SCA. Recently we reported that hydroxyurea significantly reduced the conversion from conditional TCD velocities to abnormal velocities; whether hydroxyurea can be used for children with newly diagnosed severe cerebrovascular disease in place of starting transfusion therapy remains unknown...
September 12, 2016: JMIR Research Protocols
Valérie Ghilain, Elsa Wiame, Edward Fomekong, Marie-Françoise Vincent, Dana Dumitriu, Marie-Cécile Nassogne
INTRODUCTION: Lysinuric protein intolerance (LPI) is a form of inherited aminoaciduria caused by a deficiency in the cationic amino acid transport process on the basolateral membrane of enterocytes and renal tubular cells. Clinical signs include gastrointestinal symptoms, failure to thrive, hepatosplenomegaly, osteoporosis, episodes of coma, intellectual deficiency, lung and renal involvement, bone marrow abnormalities, as well as altered immune response. Moyamoya disease is a cerebrovascular disorder predisposing sufferers to stroke through progressive stenosis of the intracranial internal carotid arteries and their proximal branches...
September 2016: European Journal of Paediatric Neurology: EJPN
Erin M Hall, Jeffrey Leonard, Jodi L Smith, Kristin P Guilliams, Michael Binkley, Robert J Fallon, Monica L Hulbert
BACKGROUND: Children with sickle cell disease (SCD) and moyamoya may benefit from indirect cerebral revascularization surgery in addition to chronic blood transfusion therapy for infarct prevention. We sought to compare overt and silent infarct recurrence rates in children with SCD undergoing revascularization. METHODS: This was a retrospective cohort study of all children with SCD and moyamoya treated at two children's hospitals. Clinical events and imaging studies were reviewed...
August 2016: Pediatric Blood & Cancer
Lori C Jordan, Melissa C Gindville, Allison O Scott, Meher R Juttukonda, Megan K Strother, Adetola A Kassim, Sheau-Chiann Chen, Hanzhang Lu, Sumit Pruthi, Yu Shyr, Manus J Donahue
Sickle cell anaemia is a monogenetic disorder with a high incidence of stroke. While stroke screening procedures exist for children with sickle cell anaemia, no accepted screening procedures exist for assessing stroke risk in adults. The purpose of this study is to use novel magnetic resonance imaging methods to evaluate physiological relationships between oxygen extraction fraction, cerebral blood flow, and clinical markers of cerebrovascular impairment in adults with sickle cell anaemia. The specific goal is to determine to what extent elevated oxygen extraction fraction may be uniquely present in patients with higher levels of clinical impairment and therefore may represent a candidate biomarker of stroke risk...
March 2016: Brain: a Journal of Neurology
Aikaterini Kanavaki, Antonis Kattamis, Polyxeni Delaporta, Ioannis Papassotiriou, Konstantinos Spengos
BACKGROUND: A high incidence of clinically-silent cerebral ischemic events has been reported in splenectomised patients with β-thalassemia intermedia (βTI). These could be due to cerebral large-vessel disease. Based on the example of sickle cell disease, we applied transcranial Doppler sonography (TCD) to evaluate cerebral vessels velocity as a possible indicator of cerebral vasculopathy. PATIENTS AND METHODS: In our study, we included 17 splenectomised and 13 non-splenectomised (control group) patients with βTI...
July 2015: In Vivo
Philippe Joly, Nathalie Garnier, Kamila Kebaili, Céline Renoux, Arthur Dony, Nathalie Cheikh, Cécile Renard, Antony Ceraulo, Daniela Cuzzubbo, Corinne Pondarré, Cyril Martin, Vincent Pialoux, Alain Francina, Yves Bertrand, Philippe Connes
The aim of this study was to test the association between hematological/genetic factors and cerebral vasculopathy in children with sickle cell anemia (SCA). A group with cerebral vasculopathy (VASC) was composed of children who had stroke (n = 6), silent infarct (n = 11), or an abnormal transcranial Doppler (n = 5). Eighty-four patients had neither positive history of stroke or silent infarct, nor abnormal transcranial Doppler (NORM group). An intermediate group (COND; n = 15) was composed of SCA children with a conditional transcranial Doppler...
April 2016: European Journal of Haematology
Valentine Brousse, Manoelle Kossorotoff, Mariane de Montalembert
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-β(0) 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...
September 2015: British Journal of Haematology
Lena N Naffaa, Yasmeen K Tandon, Neville Irani
AIM: To compare time average maximum mean velocity (TAMV) and peak systolic velocity (PSV) criteria of Trans Cranial Doppler (TCD) in their ability to predict abnormalities on magnetic resonance imaging (MRI)/magnetic resonance angiogram (MRA) in patients with sickle cell disease. METHODS: A retrospective evaluation was performed of the outcomes in all patients with a Transcranial Doppler examination at our institution since the implementation of the hospital picture archiving and communication system (PACS) system in January 2003 through December 2012...
February 28, 2015: World Journal of Radiology
A Dorie, A Guindo, Y S Saro, B A Touré, B Fané, A K Dembelé, D A Diallo
Cerebral vasculopathy exposes patients to a high risk of stroke, a major complication of sickle cell disease (SCD) associated with a high risk of death and disability. Transcranial doppler (TCD) ultrasonography used to identify SCD patients at risk of stroke may contribute to significantly reducing morbidity and mortality in these patients by indicating appropriate treatment. From March 2008 to February 2013, we conducted systematic screening for cerebral vasculopathy using TCD in 572 SCD patients (including 375 SS, 144 SC, 26 S/β(0), and 27 S/β(+) thalassemia patients) aged 1-17 years in a comprehensive center for follow-up and research on sickle cell disease in Bamako, Mali...
March 2015: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
Pablo Bartolucci, François Lionnet
Chronic complications of sickle cell disease take an increasing role in the management of patients due to their morbidity and mortality impact. The prevalence of chronic organ damages increases as the age of patients followed in France. Few organs seem unaffected by the disease. The natural history of chronic complication is highly variable from one patient to another, and the distribution of those manifestations throughout life, is different depending on their nature and pathophysiology. Thus we can, for example, distinguish SS patients presenting a "hyperhemolytic" phenotype associated with dense red blood cells that have a high risk of vascular complications including kidney disease, pulmonary hypertension, leg ulcers and priapism, from SC patients with high hemoglobin levels, who have a higher risk of retinopathy, osteonecrosis and sensory syndrome, probably related to hyperviscosity...
October 2014: La Revue du Praticien
Baba Inusa, Maddalena Casale, Caroline Booth, Sebastian Lucas
Stroke in sickle cell anaemia (SCA) is either infarctive or haemorrhagic in nature. In childhood, over 75% of strokes in SCA are infarctive. We present an adolescent with SCA who developed hypertension at the age of 13, and was treated with lisinopril. Sixteen months later she was found in cardiorespiratory arrest and died on arrival in hospital. The last transcranial Doppler scan performed 6 months before her death and a brain MRI were reported normal. The autopsy discovered massive subarachnoid haemorrhage in association with vascular damage in the circle of Willis arteries...
2014: BMJ Case Reports
Ross M Fasano, Emily R Meier, Monica L Hulbert
Sickle cell anemia (SCA)-associated cerebral vasculopathy and moyamoya is a unique entity reflecting the abnormal interactions between sickled red blood cells (RBCs) and the cerebral arterial endothelium. Endothelial injury, coagulation activation, and the inflammatory response generated by sickled RBCs are implicated in the development of cerebral vasculopathy, but the pathophysiology remains incompletely understood. SCA-specific screening and treatment guidelines have successfully reduced the incidence of overt strokes in this high-risk population...
January 2015: Blood Cells, Molecules & Diseases
Azza Abdel Gawad Tantawy, Amira Abdel Moneam Adly, Eman Abdel Rahman Ismail, Shereen Hussiny Aly
BACKGROUND: Impaired NO bioavailability represents the central feature of endothelial dysfunction, and is a common denominator in the pathogenesis of vasculopathy in sickle cell disease (SCD). Evidence indicates the contribution of 4a allele of endothelial NO synthase (eNOS) gene to cardiac and renal diseases. We studied the 27-base pair tandem repeat polymorphism in intron 4 of eNOS gene in 51 patients with SCD compared with 55 healthy controls and evaluated its role in disease severity and hemolysis-associated complications...
March 2015: Pediatric Blood & Cancer
Annie K Ahn, Adam Honeybrook, Lori C Jordan, Robert J Singer, Dale A Tylor
IMPORTANCE: Moyamoya syndrome is a rare, occlusive cerebrovascular arteriopathy with significant risk for stroke. Populations that frequently undergo otolaryngologic procedures, including patients with Down syndrome and sickle cell disease, are particularly at risk for moyamoya. The initial presentation of moyamoya syndrome as stroke in the perioperative period of an otolaryngologic procedure has not been reported. OBSERVATIONS: A retrospective medical record review assessed the relationship of otolaryngologic operations and the onset of moyamoya symptoms...
November 2014: JAMA Otolaryngology—Head & Neck Surgery
Kathleen J Helton, Robert J Adams, Karen L Kesler, Alex Lockhart, Banu Aygun, Catherine Driscoll, Matthew M Heeney, Sherron M Jackson, Lakshmanan Krishnamurti, Scott T Miller, Sharada A Sarnaik, William H Schultz, Russell E Ware
The Stroke With Transfusions Changing to Hydroxyurea (SWiTCH) trial compared standard (transfusions/chelation) to alternative (hydroxyurea/phlebotomy) treatment to prevent recurrent stroke and manage iron overload in children chronically transfused over 7 years before enrollment. Standardized brain magnetic resonance imaging/magnetic resonance angiography (MRA) and transcranial Doppler (TCD) exams were performed at entry and exit, with a central blinded review. A novel MRA vasculopathy grading scale demonstrated frequent severe baseline left/right vessel stenosis (53%/41% ≥Grade 4); 31% had no vessel stenosis on either side...
August 7, 2014: Blood
Antoine Gueguen, Matthieu Mahevas, Ruben Nzouakou, Hassan Hosseini, Anoosha Habibi, Dora Bachir, Pierre Brugière, François Lionnet, Jean-Antoine Ribei, Bertrand Godeau, Robert Girot, Vahid Ibrahima, David Calvet, Frédéric Galactéros, Pablo Bartolucci
Strokes are one of the most severe complications of sickle-cell disease. Most studies have been restricted to children with sickle-cell disease. To better understand the characteristics and follow-up of strokes occurring from childhood to adulthood, we undertook a retrospective cohort study of 69 stroke patients among the 2,875 patients consulting at the French Adult Sickle-Cell Disease Referral Center. Between 1970 and 2008, they had experienced 104 strokes: 80 ischemic, 22 hemorrhagic, and 2 intracranial sinus thromboses...
March 2014: American Journal of Hematology
Charles Mellerio, Wassim H Farhat, David Calvet, Catherine Oppenheim, Jean Pascal Lefaucheur, Pablo Bartolucci
No abstract text is available yet for this article.
June 2014: American Journal of Hematology
M Kossorotoff, D Grevent, M de Montalembert
In children with sickle-cell anemia, cerebral vasculopathy is a frequent and severe complication. It is attributed not only to erythrocyte sickling but also to multiple physiological modifications associated with sickle-cell anemia: platelet and leukocyte activation, endothelial injury and remodeling, coagulation activation, hemolysis and subsequent chronic inflammation, impaired vasomotricity, etc. Intracranial large-vessel remodeling leads to clinical cerebral infarction, whereas microvascular injury and impaired vasoreactivity lead to so-called silent infarcts, which are actually associated with impaired cognitive development...
April 2014: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
Suzanne Verlhac, Stéphane Balandra, Isabelle Cussenot, Florence Kasbi, Manuela Vasile, Ahmed Kheniche, Monique Elmaleh-Bergès, Ghislaine Ithier, Malika Benkerrou, Françoise Bernaudin, Guy Sebag
BACKGROUND: Cerebral vasculopathy is a serious complication of sickle cell anemia. Overt strokes are largely due to intracranial arteriopathy, detected by routine transcranial Doppler and largely prevented through chronic transfusions. As extracranial internal carotid artery arteriopathy was considered rare, it has not been routinely assessed in sickle cell anemia. Recent cases of overt strokes associated with stenosis/occlusion of the extracranial portion of the internal carotid artery prompted us to include extracranial internal carotid artery assessment to our transcranial Doppler sonography protocol...
May 2014: Pediatric Radiology
René van den Berg, Astrid L Rijssenbeek, Marieke E S Sprengers, Joost C J Bot, Charles B L M Majoie, Yvo B W E M Roos, William P Vandertop
Vasculopathies, including vasculitis of the central nervous system, can lead to stenosed, cicatrized vessels and the development of arterio-arteriolar collateral vessels. Bleeding due to these vascular changes, although rare, does occur. We describe six patients (all female, age range, 21-52 years; mean age, 42 years) with steno-occlusive lesions of intracranial vessels who presented with an acute intracranial haemorrhage. All had arterial steno-occlusive changes in conjunction with extensive leptomeningeal and arterio-arteriolar collaterals...
January 2014: Interventional Neuroradiology
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