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https://www.readbyqxmd.com/read/27913539/prevention-of-central-nervous-system-sequelae-in-sickle-cell-disease-without-evidence-from-randomized-controlled-trials-the-case-for-a-team-based-learning-collaborative
#1
Michael R DeBaun, Allison A King
Since 1998, the National Institutes of Health has funded 5 randomized controlled trials (RCTs) for primary and secondary prevention of strokes in children with sickle cell anemia (SCA). In a systematic fashion, these trials have significantly advanced the care of children with SCA. In the absence of an RCT, clinicians are often compelled to make decisions at the bedside, based on experience, observational studies, and principles of hematology. We will provide an initial example that describes how a team-based, learning collaborative developed a multisite standard care protocol with a low budget (<$10 000 per year) to overcome the intrinsic limitations of advancing the care of neurologic complications in sickle cell disease (SCD)...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913538/sickle-cell-disease-when-and-how-to-transfuse
#2
Jo Howard
Blood transfusion remains an important therapeutic intervention in patients with sickle cell disease (SCD), aiming to both increase the oxygen carrying capacity of blood and to reduce the complications of vaso-occlusion. Simple, manual exchange and automated exchange can be effective in reducing the acute and chronic complications of SCD, and the advantages and disadvantages of each methodology mean they all have a role in different situations. Evidence for the role of emergency transfusion in the management of the acute complications of SCD, including acute pain and acute chest syndrome, comes from observational data...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27905100/the-severity-of-anaemia-depletes-cerebrovascular-dilatory-reserve-in-children-with-sickle-cell-disease-a-quantitative-magnetic-resonance-imaging-study
#3
Przemyslaw D Kosinski, Paula L Croal, Jackie Leung, Suzan Williams, Isaac Odame, Gregory M T Hare, Manohar Shroff, Andrea Kassner
Overt ischaemic stroke is one of the most devastating complications in children with sickle cell disease (SCD). The compensatory response to anaemia in SCD includes an increase in cerebral blood flow (CBF) by accessing cerebrovascular dilatory reserve. Exhaustion of dilatory reserve secondary to anaemic stress may lead to cerebral ischaemia. The purpose of this study was to investigate CBF and cerebrovascular reactivity (CVR) using magnetic resonance imaging (MRI) in children with SCD and to correlate these with haematological markers of anaemia...
December 1, 2016: British Journal of Haematology
https://www.readbyqxmd.com/read/27869039/the-role-of-rs1984112_g-at-cd36-gene-in-increasing-reticulocyte-level-among-sickle-cell-disease-patients
#4
Miniar Kalai, Marwa Dridi, Leila Chaouch, Imen Moumni, Houyem Ouragini, Imen Darragi, Imen Boudrigua, Dorra Chaouachi, Fethi Mellouli, Mohamed Bejaoui, Salem Abbes
AIMS AND BACKGROUND: Mediators of adhesion become a potential new target for pharmacological therapy to struggle the complications of sickle cell disease (SCD). Several mechanisms for increased adherence have been postulated and the well-studied are CD36 and VLA4 which encoded by ITGA4. Herein, we sought to determine whether one polymorphism of CD36 namely: rs1984112 and three exons of ITGA4 (4, 5, and 6) are implicated in hemolytic status and clinical events among SCD Tunisian patients...
November 20, 2016: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/27858986/stroke-in-sickle-cell-anaemia-is-more-than-stenosis-and-thrombosis-the-role-of-anaemia-and-hyperemia-in-ischaemia
#5
EDITORIAL
Michael M Dowling, Fenella J Kirkham
No abstract text is available yet for this article.
November 11, 2016: British Journal of Haematology
https://www.readbyqxmd.com/read/27854159/sheehan-s-syndrome-and-sickle-cell-disease-the-story-of-natasha
#6
Barbara A Wilson, Anita Rose, Gerhard Florschutz
Sheehan's syndrome (SS) is one of the pituitary disorders caused by severe blood loss during childbirth leading to necrosis of the pituitary gland. Diagnosis is made following severe haemorrhage, failure to produce milk and failure to menstruate. Rare in countries with good obstetric care, SS is still frequent in those countries with poor healthcare services. The majority of papers published on SS do not mention psychological or neuropsychological sequelae. Of those that do, mood disorders are sometimes reported and occasionally cognitive problems are noted, typically attention, memory or executive deficits...
November 17, 2016: Neuropsychological Rehabilitation
https://www.readbyqxmd.com/read/27823116/targeting-sickle-cell-disease
#7
Sekayi Tangayi
Sickle cell disease is one of the most common global genetic disorders. It is a debilitating condition and children with sickle cell disease are more likely to have a stroke, and experience lower quality of life and shorter life expectancy.
October 5, 2016: Nursing Standard
https://www.readbyqxmd.com/read/27814292/hemorheological-alterations-in-sickle-cell-anemia-and-their-clinical-consequences-the-role-of-genetic-modulators
#8
Marisa Silva, Sofia Vargas, Andreia Coelho, Alexandra Dias, Teresa Ferreira, Anabela Morais, Raquel Maia, Paula Kjöllerström, João Lavinha, Paula Faustino
Sickle cell anemia (SCA) is an autosomal recessive disease caused by the HBB:c.20A>T mutation that leads to hemoglobin S synthesis. The disease presents with high clinical heterogeneity characterized by chronic hemolysis, recurrent episodes of vaso-oclusion and infection. This work aimed to characterize by in silico studies some genetic modulators of severe hemolysis and stroke risk in children with SCA, and understand their consequences at the hemorheological level.Association studies were performed between hemolysis biomarkers as well as the degree of cerebral vasculopathy and the inheritance of several polymorphic regions in genes related with vascular cell adhesion and vascular tonus in pediatric SCA patients...
November 4, 2016: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/27804209/national-registry-of-hemoglobinopathies-in-spain-rephem
#9
Elena Cela, José M Bellón, María de la Cruz, Cristina Beléndez, Rubén Berrueco, Anna Ruiz, Izaskun Elorza, Cristina Díaz de Heredia, Aurea Cervera, Griselda Vallés, J Antonio Salinas, M Teresa Coll, Mar Bermúdez, Marta Prudencio, Bienvenida Argilés, Cruz Vecilla
BACKGROUND: Although highly prevalent throughout the world, the accurate prevalence of hemoglobinopathies in Spain is unknown. PROCEDURE: This study presents data on the national registry of hemoglobinopathies of patients with thalassemia major (TM), thalassemia intermedia (TI), and sickle cell disease (SCD) in Spain created in 2014. Fifty centers reported cases retrospectively. Data were registered from neonatal screening or from the first contact at diagnosis until last follow-up or death...
November 2, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27785921/multimodality-ultrasound-imaging-in-stroke-current-concepts-and-future-focus
#10
Yumei Liu, Yang Hua, Wuwei Feng, Bruce Ovbiagele
Stroke is a leading cause of disability and mortality worldwide. Ultrasound is a real-time imaging technique that is inexpensive, portable, non-invasive, and safe, with high diagnostic accuracy. Ultrasonic imaging can provide useful direct and indirect information about the characteristics of various vessels in the both intracranial and extracranial segments. Areas covered: In this review, we will discuss multimodal applications of ultrasonic imaging in stroke prevention and management including checking carotid intima-media thickness progression, evaluating the plaque morphology, calibrating the degree of stenosis, detecting the presence of patent foramen ovale, monitoring microembolization, and screening for stroke risk in patients with sickle cell disease...
December 2016: Expert Review of Cardiovascular Therapy
https://www.readbyqxmd.com/read/27775898/managing-acute-complications-of-sickle-cell-disease-in-pediatric-patients
#11
Sathyaseelan Subramaniam, Jennifer H Chao
Sickle cell disease is a chronic hematologic disease with a variety of acute, and often recurring, complications. Vaso-occlusive crisis, a unique but common presentation in sickle cell disease, can be challenging to manage. Acute chest syndrome is the leading cause of death in patients with sickle cell disease, occurring in more than half of patients who are hospitalized with a vaso-occlusive crisis. Uncommon diagnoses in children, such as stroke, priapism, and transient red cell aplasia, occur more frequently in patients with sickle cell disease and necessitate a degree of familiarity with the disease process and its management...
November 2016: Pediatric Emergency Medicine Practice
https://www.readbyqxmd.com/read/27766637/increased-prevalence-of-potential-right-to-left-shunting-in-children-with-sickle-cell-anaemia-and-stroke
#12
Michael M Dowling, Charles T Quinn, Claudio Ramaciotti, Julie Kanter, Ifeyinwa Osunkwo, Baba Inusa, Rathi Iyer, Janet L Kwiatkowski, Clarissa Johnson, Melissa Rhodes, William Owen, John J Strouse, Julie A Panepinto, Lynne Neumayr, Sharada Sarnaik, Patricia A Plumb, Nomazulu Dlamini, Fenella Kirkham, Linda S Hynan
'Paradoxical' embolization via intracardiac or intrapulmonary right-to-left shunts (RLS) is an established cause of stroke. Hypercoagulable states and increased right heart pressure, which both occur in sickle cell anaemia (SCA), predispose to paradoxical embolization. We hypothesized that children with SCA and overt stroke (SCA + stroke) have an increased prevalence of potential RLS. We performed contrasted transthoracic echocardiograms on 147 children (aged 2-19 years) with SCA + stroke) mean age 12·7 ± 4·8 years, 54·4% male) and a control group without SCA or stroke (n = 123; mean age 12·1 ± 4·9 years, 53·3% male)...
October 21, 2016: British Journal of Haematology
https://www.readbyqxmd.com/read/27759170/erythrocytapheresis-for-chronic-transfusion-therapy-in-sickle-cell-disease-survey-of-current-practices-and-review-of-the-literature
#13
Shannon Kelly, Keith Quirolo, Anne Marsh, Lynne Neumayr, Alicia Garcia, Brian Custer
BACKGROUND: Chronic red blood cell (RBC) transfusion therapy (CTT) is an integral component of the management of severe sickle cell disease (SCD) and can prevent complications, such as stroke. RBC units can be administered via simple transfusion or exchange transfusion, and erythrocytapheresis (automated RBC exchange transfusion [aRBX]), is increasingly used for CTT. Comparisons of simple and aRBX transfusions are limited, and the current scope of aRBX use is not known. STUDY DESIGN AND METHODS: We administered a survey to define current transfusion practices for CTT and performed a review of the erythrocytapheresis literature...
October 19, 2016: Transfusion
https://www.readbyqxmd.com/read/27740993/ultrasound-in-neurology
#14
Georgios Tsivgoulis, Andrei V Alexandrov
PURPOSE OF REVIEW: Low cost, avoidance of irradiation, and high temporal resolution are inherent advantages of ultrasound imaging that translate into multiple clinical uses in many domains of neurology. This article presents clinical uses of ultrasound examination in cerebrovascular, neurodegenerative, and peripheral nervous system diseases. RECENT FINDINGS: Modern treatment and prevention of ischemic stroke rely on prompt diagnosis. Ultrasonography has found a place as a noninvasive screening test and bedside technique that provides estimates of the degree of stenosis as well as hemodynamic and structural information about intracranial and extracranial vessels in real time...
October 2016: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/27726639/genetics-of-sickle-cell-associated-cardiovascular-disease-an-expert-review-with-lessons-learned-in-africa
#15
Amy Geard, Gift D Pule, David Chelo, Valentina Josiane Ngo Bitoungui, Ambroise Wonkam
Sickle cell disease (SCD) vastly impacts the African continent and is associated with cardiovascular diseases. Stroke, kidney disease, and pulmonary hypertension are considered as proxies of severity in SCD with several genomic loci implicated in their heritability. The present expert review examined the current data on epidemiology and genetic risk factors of stroke, pulmonary hypertension, and kidney disease associated with SCD, as indexed in PubMed(®) and Google Scholar(®). Studies collectively show that stroke and kidney disease each affect ∼10% of SCD patients, with pulmonary hypertension displaying a higher prevalence of 30% among adults with SCD...
October 2016: Omics: a Journal of Integrative Biology
https://www.readbyqxmd.com/read/27706129/pediatric-sickle-cell-disease-past-successes-and-future-challenges
#16
Emily Riehm Meier, Angeli Rampersad
Once a fatal disease of childhood, more than 95% of patients born today with sickle cell disease (SCD) in developed countries are expected to survive into adulthood, largely because of improvements in supportive and preventive care (newborn screening, penicillin prophylaxis, transcranial Doppler (TCD) screening). Hydroxyurea (HU) therapy, the only oral medication currently available to prevent SCD complications, has become more widespread over the past 20 years. The NHLBI recommends that HU be offered to all patients with HbSS beginning at nine months of age, and the recently published Abnormal TCD with Transfusions Changing to HU (TWiTCH) trial has shown HU as an acceptable alternative to transfusion therapy for patients at high risk of stroke...
October 5, 2016: Pediatric Research
https://www.readbyqxmd.com/read/27692991/-chronic-brain-damage-in-sickle-cell-disease-and-its-relation-with-quality-of-life
#17
Elena Cela, Ana G Vélez, Alejandra Aguado, Gabriela Medín, José M Bellón, Cristina Beléndez
BACKGROUND AND OBJECTIVE: Sickle cell anaemia causes progressive organ damage. The objective is to describe school performance of patients with sickle cell anaemia and their clinical parameters and quality of life that may have an influence. The hypothesis is that if school alterations occur without other objective data, additional factors must be present besides the disease itself. PATIENTS AND METHODS: Transversal study performed in November 2015 considering analytical variables, complications and neuroradiological images of children with sickle cell anaemia, and family survey on school performance and quality of life...
September 29, 2016: Medicina Clínica
https://www.readbyqxmd.com/read/27677923/epidemiology-and-treatment-of-relative-anemia-in-children-with-sickle-cell-disease-in-sub-saharan-africa
#18
Halima Bello-Manga, Michael R DeBaun, Adetola A Kassim
Sickle cell disease (SCD) is the most common inherited hemoglobinopathy in the world, with the majority of cases in sub-Saharan Africa. Concomitant nutritional deficiencies, infections or exposure to environmental toxins exacerbate chronic anemia in children with SCD. The resulting relative anemia is associated with increased risk of strokes, poor cognitive function and impaired growth. It may also attenuate optimal response to hydroxyurea therapy, the only effective and practical treatment option for SCD in sub-Saharan Africa...
October 17, 2016: Expert Review of Hematology
https://www.readbyqxmd.com/read/27639979/prevalence-of-stroke-in-children-admitted-with-sickle-cell-anaemia-to-mulago-hospital
#19
Deogratias Munube, Elly Katabira, Grace Ndeezi, Moses Joloba, Samden Lhatoo, Martha Sajatovic, James K Tumwine
BACKGROUND: Stroke is a major complication of sickle cell anaemia (SCA). It occurs commonly in childhood with about 10 % of children with sickle cell anaemia getting affected by this complication. In Uganda, there is paucity of data on the prevalence of stroke in children admitted in a tertiary institution. We determined the prevalence of stroke amongst children with SCA admitted to Mulago National Referral Hospital in Uganda and described the ir co-morbidities. METHODS: We conducted a retrospective record review of children with SCA admitted from August 2012 to August 2014 to the Paediatric Haematology Ward of Mulago Hospital in Kampala, Uganda...
September 17, 2016: BMC Neurology
https://www.readbyqxmd.com/read/27637966/sickle-cell-disease
#20
J Strouse
Sickle cell disease (SCD) is an inherited hemoglobinopathy caused by a mutation in the sixth amino acid of the β-globin gene (HBB). It is the most common serious genetic diseases in childhood, affecting approximately 1 in 2500 births and 100 000 individuals in the USA, in addition to 300 000 new cases globally each year. Central nervous system injury is the most debilitating frequent complication of SCD and includes stroke, silent cerebral infarct (SCI), and cognitive impairment. Among children with sickle cell anemia (HbSS), 11% had a stroke by age 18 years before the implementation of transcranial Doppler screening...
2016: Handbook of Clinical Neurology
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