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Sickle cell diseases

Massimo Rivolo
This article introduces a simple approach to adopt in the treatment of sickle cell disease (SCD) related ulcerations based on the acronym SICKLE, standing for Skin assessment, Infection control, Compression, Keep moving keep debriding, Local strapping and Endless support. This case report explains and describes each step used in the treatment of this challenging disease in a 24-year-old patient, achieving complete healing in 6 months in long-lasting SCD ulceration previously treated without results with many other treatments...
November 8, 2018: British Journal of Nursing: BJN
Puneeth Guruprasad, Robert G Mannino, Christina Caruso, Hanqing Zhang, Cassandra D Josephson, John D Roback, Wilbur A Lam
Investigating individual red blood cells (RBCs) is critical to understanding hematologic diseases, as pathology often originates at the single-cell level. Many RBC disorders manifest in altered biophysical properties, such as deformability, of RBCs. Due to limitations in current biophysical assays, there exists a need for high-throughput analysis of RBC deformability with single-cell resolution. To that end, we present a method that pairs a simple in vitro artificial microvasculature network system with an innovative MATLAB-based automated particle tracking program, allowing for the high-throughput, single-cell deformability index (sDI) measurements of entire RBC populations...
November 12, 2018: American Journal of Hematology
Umar K Mian, Joyce Tang, Ana P M Allende, Moonseong Heo, Nicole Bernstein, Leena Vattappally, Daniel Schoenfeld, Caterina P Minniti
Among the many vascular complications of sickle cell disease (SCD), retinopathy is the most prevalent and represents a leading cause of blindness. Hydroxycarbamide therapy ameliorates many symptoms of SCD, and high fetal haemoglobin (HbF) levels have been shown to protect against the development of retinopathy in children with HbSS. Its effect on adults with SCD, who are at a much higher risk of developing retinopathy, has not been studied. We aimed to investigate the effect of hydroxycarbamide use and HbF level on sickle cell retinopathy development in adults...
November 12, 2018: British Journal of Haematology
Kavita Rana, Kimberly Pantoja, Liping Xiao
Bone loss is a common complication in individuals with sickle cell disease (SCD). The mechanism(s) of bone loss in SCD subjects has not been fully investigated, and there are no targeted therapies to prevent or treat compromised bone health in this population. Recent studies showed that depletion of gut microbiota with antibiotics significantly reduced the number of aged neutrophils, thereby dramatically improved the inflammation-related organ damages in SCD mice. Since neutrophils, abundantly present in bone marrow (BM), regulate bone cells, and BM neutrophils, induced by inflammatory cytokines, are associated with a low number of osteoblasts (OBs), we hypothesize that neutrophil aging in the BM of SCD mice impairs OB function...
December 2018: Biochemistry and Biophysics Reports
Biaoru Li, Xingguo Zhu, Christina M Ward, Athena Starlard-Davenport, Mayuko Takezaki, Amber Berry, Alexander Ward, Caroline Wilder, Cindy Neunert, Abdullah Kutlar, Betty S Pace
Inherited genetic modifiers and pharmacologic agents that enhance fetal hemoglobin (HbF) expression reverse the clinical severity of sickle cell disease (SCD). Recent efforts to develop novel strategies of HbF induction include discovery of molecular targets that regulate γ-globin gene transcription and translation. The purpose of this study was to perform genome-wide microRNA (miRNA) analysis identify genes associated with HbF expression in patients with SCD. We isolated RNA from purified reticulocytes for microarray-based miRNA expression profiling...
November 6, 2018: Experimental Hematology
Christina L Dean, Cheryl L Maier, John D Roback, Sean R Stowell
BACKGROUND: Hemolytic transfusion reactions are a rare, yet serious complication of red blood cell (RBC) transfusion. Patients with sickle cell disease (SCD) are at an increased risk for such reactions, because they are prone to make alloantibodies against transfused RBCs, complicating this integral part of their disease management. These reactions may be missed, and the patient's state may be attributed to vaso-occlusive crisis (VOC), with misguided therapy ensuing. CASE REPORT: Herein, we report the clinical course of a patient with complex SCD with a delayed hemolytic transfusion reaction, followed by multiple acute hemolytic transfusion reactions mistaken for severe VOC...
November 9, 2018: Transfusion
Chung-Jen Chen, Sabrina Bakeera-Kitaka, Ezekiel Mupere, Philip Kasirye, Deogratias Munube, Richard Idro, Heather Hume, Betsy Pfeffer, Philip LaRussa, Nancy S Green
AIM: We aimed to assess the receipt of recommended care for young children with sickle cell disease (SCD) in a central SCD clinic in Kampala Uganda, focusing on standard vaccination and antibacterial and antimalarial prophylaxis. METHODS: A cross-sectional assessment of immunisation status and timeliness and prescribed antibacterial and antimalarial prophylaxis was performed in a sample with SCD aged ≤71 months in Mulago Hospital SCD Clinic. Government-issued immunisation cards and clinic-issued visit records for prescribed prophylaxis were reviewed...
November 9, 2018: Journal of Paediatrics and Child Health
Chinedu A Ezekekwu, Taiwo R Kotila, Titilola S Akingbola, Guillaume Lettre, Victor R Gordeuk, Richard S Cooper, Michael R DeBaun, Baba Inusa, Bamidele O Tayo
Sickle cell disease, one of the world's most common genetic disorders is prevalent in sub-Saharan Africa. The trans-Atlantic slave trade accounted for the gene movement from Africa to the Caribbean and United States of America and lately, migration has resulted in the introduction of the gene to the United Kingdom and other parts of Europe. Different haplotypes exist, however the differences in these haplotypes are not sufficient to explain the different clinical variations within the same region or different settings...
2018: Journal of Tropical Diseases & Public Health
Mohsen Saleh ElAlfy, Fatma Soliman Elsayed Ebeid, Tarek Mostafa Kamal, Deena Samir Eissa, Eman Abdel Rahman Ismail, Samar Hassan Mohamed
BACKGROUND: Cerebrovascular stroke is a common critical complication of sickle cell disease (SCD). Angiotensinogen (AGT) M235T gene polymorphism is associated with risk of ischemic stroke and cardiovascular disease. AIM: We investigated the potential association between angiotensinogen M235T gene polymorphism and susceptibility to cerebrovascular and cardiopulmonary complications in adolescents with SCD. METHODS: Forty-six patients with SCD in steady state were studied stressing on history of stroke, hydroxyurea/chelation therapy, hematological profile, and echocardiographic findings...
November 5, 2018: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
Nicholas T K D Dayie, Georgina Tetteh-Ocloo, Appiah-Korang Labi, Edeghonghon Olayemi, Hans-Christian Slotved, Margaret Lartey, Eric S Donkor
BACKGROUND: Pneumococcal carriage is the precursor for development of pneumococcal disease, and is also responsible for transmission of the organism from person-to-person. Individuals with Sickle Cell Disease (SCD) are more likely to develop invasive disease with S. pneumoniae compared to their healthy counterparts and the presentation of disease in the former is usually abrupt and severe. In Africa, little is known about the pneumococcus in relation to people with SCD Sickle Cell Disease (SCD)...
2018: PloS One
Mara Banks, James Shikle
Sickle cell disease is a genetic disease commonly affecting people of African, Indian, and Mediterranean descent. Patients with this chronic disease often require lifelong red blood cell transfusions. Formation of alloantibodies and autoantibodies are well-known complications that can arise with multiple transfusions. Another rare, but serious complication associated with transfusion is hyperhemolysis syndrome. The acquisition of new and/or rare alloantibodies can make it more difficult to find compatible blood products for patients with sickle cell disease...
November 2018: Archives of Pathology & Laboratory Medicine
Alessandro Matte, Antonio Recchiuti, Enrica Federti, Bérengère Koehl, Thomas Mintz, Wassim El Nemer, Pierre-Louis Tharaux, Valentine Brousse, Immacolata Andolfo, Alessia Lamolinara, Olga Weinberg, Angela Siciliano, Paul C Norris, Ian R Riley, Achille Iolascon, Charles N Serhan, Carlo Brugnara, Lucia De Franceschi
The endogenous lipid mediators Resolvins (Rv) play a key role in the resolution of inflammation. Sickle cell disease (SCD), a genetic disorder of hemoglobin, is characterized by inflammatory and vaso-occlusive pathologies. We document in humanized SCD mice altered pro-resolving events following hypoxia/reperfusion. We demonstrate novel protective actions of 17 R -RvD1 (7 S , 8 R , 17 R -trihydroxy-4 Z , 9 E , 11 E , 13 Z , 15 E , 19 Z -docosahexaenoic acid), in reducing ex vivo human SCD blood leukocyte recruitment by microvascular endothelial cells and in vivo neutrophil adhesion and transmigration...
November 7, 2018: Blood
Yutaka Niihara, Wally R Smith, Charles W Stark
New England Journal of Medicine, Volume 379, Issue 19, Page 1879-1880, November 2018.
November 8, 2018: New England Journal of Medicine
Elizabeth S Allen, Randin C Nelson, Willy A Flegel
Multiple hematopoietic progenitor cell (HPC) transplantation options for patients with sickle cell disease (SCD) are currently under investigation. Patients with SCD have a high rate of alloimmunization to red blood cell antigens, often complicating transfusion support. Transfusion reactions, including acute and delayed hemolytic reactions, have been observed despite immunosuppressive regimens. Allogeneic donor transplants have been shown to carry a risk of prolonged reticulocytopenia and acute hemolysis with severe anemia in nonmyeloablative regimens...
November 2018: Transfusion
Rachael Hall, Kate Gardner, David C Rees, Subarna Chakravorty
Objective: To assess the prevalence of high body mass index (BMI) in children with sickle cell disease and assess correlation between BMI and disease severity. Design: Retrospective chart review followed by statistical analysis. Setting: A single tertiary paediatric clinic in inner city London. Patients: All patients with sickle cell disease, including homozygous haemoglobin (HbSS) and compound heterozygous Hb (HbSC), age 2-18 years receiving clinical care at the centre, were included in the study...
2018: BMJ Paediatrics Open
Foluso Joy Ogunsile, Shawn M Bediako, Julie Nelson, Cody Cichowitz, Tiffany Yu, C Patrick Carroll, Kerry Stewart, Rakhi Naik, Carlton Haywood, Sophie Lanzkron
Metabolic syndrome (MetS) is a key risk factor for cardiovascular disease (CVD) incidence and all-cause mortality. MetS prevalence among adults with sickle cell disease (SCD) is not well known. We report initial findings from a cross-sectional study that examined MetS risk factors within a cohort of adults living with SCD. 50 adult SCD participants (ages 21-66 years; 72% female) completed demographic and health behavior surveys, health-related family and personal histories, and anthropometric and laboratory measurements...
October 22, 2018: Blood Cells, Molecules & Diseases
Barnabas Gellen, Laurent A Messonnier, Frédéric Galactéros, Etienne Audureau, Angèle N Merlet, Thomas Rupp, Sandrine Peyrot, Cyril Martin, Léonard Féasson, Pablo Bartolucci
BACKGROUND: Exercise could be a triggering factor for vaso-occlusive crises in patients with sickle-cell disease. We aimed to investigate whether a patient-adapted training programme of moderate endurance exercise could be safe and beneficial for patients with sickle-cell disease. METHODS: We did a multicentre, prospective, open-label, randomised controlled trial at four university hospitals in France. Eligible patients were older than 18 years, with an HbSS or S/β0 -thalassaemia genotype, and with no severe chronic complications...
November 2018: Lancet Haematology
Gregory J Kato
No abstract text is available yet for this article.
November 2018: Lancet Haematology
George B Richter-Addo
No abstract text is available yet for this article.
November 1, 2018: Acta Crystallographica. Section D, Structural Biology
Amber Fearon, Anne Marsh, Jennifer Kim, Marsha Treadwell
OBJECTIVE: Current guidelines recommend high-priority treatment of severe sickle cell disease (SCD) pain with opioids; however, patients with SCD have historically been undertreated. We used mixed methods to assess pediatric residents' perceptions toward opioid use in SCD pain management. METHODS: We distributed a survey to 88 residents at an urban pediatric medical center in a cross-sectional study. Participants responded to questions about perceived barriers to acute SCD pain management and attitudes toward patients with SCD...
November 1, 2018: Pediatric Blood & Cancer
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