keyword
MENU ▼
Read by QxMD icon Read
search

Sickle Cell Crisis

keyword
https://www.readbyqxmd.com/read/29755709/perioperative-management-of-sickle-cell-disease
#1
REVIEW
Kwame Ofori Adjepong, Folashade Otegbeye, Yaw Amoateng Adjepong
Over 30 million people worldwide have sickle cell disease (SCD). Emergent and non-emergent surgical procedures in SCD have been associated with relatively increased risks of peri-operative mortality, vaso-occlusive (painful) crisis, acute chest syndrome, post-operative infections, congestive heart failure, cerebrovascular accident and acute kidney injury. Pre-operative assessment must include a careful review of the patient's known crisis triggers, baseline hematologic profile, usual transfusion requirements, pre-existing organ dysfunction and opioid use...
2018: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/29724898/how-i-safely-transfuse-patients-with-sickle-cell-disease-and-manage-delayed-hemolytic-transfusion-reactions
#2
France Pirenne, Karina Yazdanbakhsh
Transfusions can be a life-saving treatment for patients with sickle cell disease (SCD). However, availability of matched units can be limiting due to distinctive blood group polymorphisms in patients of African descent. Development of antibodies against the transfused red blood cells (RBCs), resulting in delayed hemolytic transfusion reactions (DHTRs), can be life-threatening and pose unique challenges for this population with regard to treatment strategies and transfusion management protocols. In cases where the transfused cells as well as patient's own RBCs are destroyed, diagnosis of DHTR can be difficult because symptoms may mimic vaso-occlusive crisis, and frequently antibodies are undetectable...
May 3, 2018: Blood
https://www.readbyqxmd.com/read/29690499/levels-of-soluble-endothelium-adhesion-molecules-and-complications-among-sickle-cell-disease-patients-in-ghana
#3
Charles Antwi-Boasiako, Eric S Donkor, Fredericka Sey, Bartholomew Dzudzor, Gifty B Dankwah, Kate H Otu, Alfred Doku, Campbell A Dale, Ivy Ekem
BACKGROUND: Soluble adhesion molecules are involved in the gathering and joining of inflammatory cells to vascular endothelium. Therefore, they serve as potential markers of endothelial dysfunction in vascular diseases including sickle cell disease (SCD). In Ghana, there are scarcely any report on the levels of adhesion molecules among SCD patients. The current study aimed to determine plasma levels of ICAM-1, VCAM-1 and E-Selectin as markers of endothelial dysfunction in SCD patients in steady state, complications and controls...
April 21, 2018: Diseases (Basel)
https://www.readbyqxmd.com/read/29667721/diverse-manifestations-of-acute-sickle-cell-hepatopathy-in-pediatric-patients-with-sickle-cell-disease-a-case-series
#4
Lydia H Pecker, Nidhi Patel, Susan Creary, Anil Darbari, Emily Riehm Meier, Deepika S Darbari, Ross M Fasano
The hepatic complications of sickle cell disease (SCD) are associated with increased morbidity and mortality in adults; children usually survive but may suffer significant sequelae. Few diagnostic tools differentiate the various hepatic manifestations of SCD. Why patients exhibit one hepatic pathology versus another is unclear. We report four pediatric patients with hemoglobin SS disease with diverse manifestations of acute hepatic involvement including acute sickle hepatic crisis, hepatic sequestration, sickle cell intrahepatic cholestasis, and a non-SCD cause of hepatopathy in a patient with viral hepatitis...
April 18, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29651930/molecular-insights-into-the-irreversible-mechanical-behavior-of-sickle-hemoglobin
#5
Sumith Yesudasan, Simone A Douglas, Manu O Platt, Xianqiao Wang, Rodney D Averett
Sickle cell disease is caused by the amino acid substitution of glutamic acid to valine, which leads to the polymerization of deoxygenated sickle hemoglobin (HbS) into long strands. These strands are responsible for the sickling of red blood cells (RBCs), making blood hyper-coagulable leading to an increased chance of vaso-occlusive crisis. The conformational changes in sickled RBCs traveling through narrow blood vessels in a highly viscous fluid are critical in understanding; however, there are few studies that investigate the origins of the molecular mechanical behavior of sickled RBCs...
April 13, 2018: Journal of Biomolecular Structure & Dynamics
https://www.readbyqxmd.com/read/29622872/beneficial-effect-of-low-fixed-dose-of-hydroxyurea-in-vaso-occlusive-crisis-and-transfusion-requirements-in-adult-hbss-patients-a-prospective-study-in-a-tertiary-care-center
#6
Sudha Sethy, Tribikram Panda, Rabindra Kumar Jena
Significant reduction in morbidity and mortality have been documented in patients with sickle cell disease (HbSS) by most of the studies using hydroxyurea at a dose of 25-35 mg/kg/day or maximum tolerated dose. But toxicities, need for frequent monitoring, compliance and cost are important hurdles particularly in Indian set up. We undertook this study to find out the efficacy, safety compliance rate of low fixed dose of hydroxyurea (10 mg/kg/day) in patients presenting to our hospital and its impact on clinical profile and laboratory parameters...
April 2018: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/29620547/vitamin-d-deficiency-in-sickle-cell-disease-patients-in-the-eastern-province-of-saudi-arabia
#7
Ali AlJama, Mohammed AlKhalifah, Ibrahim Abdulla Al-Dabbous, Ghada Alqudaihi
BACKGROUND: Vitamin D deficiency (VDD) is a major global health problem. In sickle cell disease (SCD), VDD is highly prevalent, reaching up to 96% of populations. VDD may contribute to many of the complications of SCD. OBJECTIVE: Estimate the 25-hydroxyvitamin D [25(OH)D] level and the frequency of VDD and insufficiency among among SCD patients by age group and disease status. DESIGN: Analytical cross-sectional. SETTING: Ministry of Health (MOH) secondary care hospital...
March 2018: Annals of Saudi Medicine
https://www.readbyqxmd.com/read/29620434/transient-receptor-potential-polymorphism-and-haplotype-associate-with-crisis-pain-in-sickle-cell-disease
#8
Ellie H Jhun, Xiaoyu Hu, Nilanjana Sadhu, Yingwei Yao, Ying He, Diana J Wilkie, Robert E Molokie, Zaijie J Wang
AIM: Episodes of acute pain crisis contribute to considerable morbidity and mortality in sickle cell disease (SCD). Incomprehensive understanding of the underlying pain heterogeneity results in inadequate pain management. The transient receptor potential (TRP) family of voltage-gated ion channels acts as sensory transducers of diverse noxious stimuli. We performed an association study of polymorphisms in candidate genes TRPV1 and TRPA1 with pain in SCD patients. METHODS: Utilization rate, in other words, number of emergency department/acute care center admissions over 12 months as a result of pain crisis, served as a marker for acute pain...
April 5, 2018: Pharmacogenomics
https://www.readbyqxmd.com/read/29614636/autonomic-nervous-system-involvement-in-sickle-cell-disease
#9
Thomas D Coates, Patjanaporn Chalacheva, Lonnie Zeltzer, Michael C K Khoo
Sickle cell disease (SCD) is a genetic disorder of hemoglobin producing hemoglobin-S (HbS) and resulting in recurrent severe episodes of pain, organ damage and premature death due to vaso- occlusion. Deoxy HbS polymerizes, causing red cells to become rigid and lodge in the microvasculature if they do not escape into larger vessels before this transformation occurs. The mechanism that triggers this transition from steady state to vaso-occlusive crisis (VOC) is not known. Patients state that cold, emotional stress, and pain itself can trigger these events...
2018: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/29607863/total-hip-replacement-in-sickle-cell-disorder-a-preliminary-report-of-challenges-and-early-outcome-of-21-consecutive-patients
#10
A U Katchy, U E Anyaehie, C U Nwadinigwe, G O Eyichukwu
Objective: The aim of this study is to describe the pattern of presentation osteoarthritic patients with sickle cell disorder (SCD) in our environment, determine the implant sizes taking the peculiar nature of the pathology and our operating environment into consideration, highlight the challenges and technical difficulties encountered during the procedure, measure the functional outcome and observe complications of treatment, recommend ways of improving outcome. Patients and Methods: Between November 2008 and November 2012, 29 consecutive primary total hip replacements (THRs) were performed on 21 patients with avascular necrosis of the head of femur secondary to SCD...
April 2018: Nigerian Journal of Clinical Practice
https://www.readbyqxmd.com/read/29590553/aps-sickle-cell-disease-conference-mini-review-targeting-pain-at-its-source-in-sickle-cell-disease
#11
Kanika Gupta, Om Jahagirdar, Kalpna Gupta
Sickle cell disease (SCD) is a genetic disorder associated with hemolytic anemia, end-organ damage, reduced survival and pain. One of the unique features of SCD is, recurrent and unpredictable episodes of acute pain due to vaso-occlusive crisis, requiring hospitalization. Additionally, SCD patients often develop chronic persistent pain. Currently, sickle pain is treated with opioids, an approach limited by adverse effects. Because pain can start at infancy and continue throughout life, preventing the genesis of pain may be relatively better than treating the pain once it has been evoked...
March 28, 2018: American Journal of Physiology. Regulatory, Integrative and Comparative Physiology
https://www.readbyqxmd.com/read/29579313/sickle-cell-disease-in-southwestern-nigeria-assessment-of-knowledge-of-primary-health-care-workers-and-available-facilities
#12
Samuel A Adegoke, Morenike A Akinlosotu, Olaronke B Adediji, Oyeku A Oyelami, Oluwagbemiga O Adeodu, Adekunle D Adekile
Background: Patients with sickle cell disease (SCD) benefit optimally from comprehensive care. In Nigeria, despite the huge burden, involvement of community health workers (CHWs) in the management of SCD is poor. Methods: This community-based study assessed SCD-related knowledge of 182 CHWs from the 46 primary health care (PHC) centres in Ilesa, southwestern Nigeria. Available facilities and management practices for SCD care at these centres were also evaluated using pretested self-administered questionnaires and observational checklists...
March 22, 2018: Transactions of the Royal Society of Tropical Medicine and Hygiene
https://www.readbyqxmd.com/read/29560059/emergency-department-ed-ed-observation-day-hospital-and-hospital-admissions-for-adults-with-sickle-cell-disease
#13
David M Cline, Susan Silva, Caroline E Freiermuth, Victoria Thornton, Paula Tanabe
Introduction: Use of alternative venues to manage uncomplicated vaso-occlusive crisis (VOC), such as a day hospital (DH) or ED observation unit, for patients with sickle cell anemia, may significantly reduce admission rates, which may subsequently reduce 30-day readmission rates. Methods: In the context of a two-institution quality improvement project to implement best practices for management of patients with sickle cell disease (SCD) VOC, we prospectively compared acute care encounters for utilization of 1) emergency department (ED); 2) ED observation unit; 3) DH, and 4) hospital admission, of two different patient cohorts with SCD presenting to our two study sites...
March 2018: Western Journal of Emergency Medicine
https://www.readbyqxmd.com/read/29533092/inherited-blood-disorders-genetic-risk-and-global-public-health-framing-birth-defects-as-preventable-in-india
#14
Sangeeta Chattoo
This paper engages critically with the global assemblage framing sickle cell and thalassaemia disorders as a 'global health crisis'; and the promise of genomics, largely DNA-based carrier/pre-conceptual screening, prenatal diagnosis with a view to terminations, deployed in framing a solution to these historically racialised spectrum of diseases as essentially preventable. Sickle cell and thalassaemia are recessively inherited, potentially life-threatening haemoglobin disorders with significant variation of severity, often needing life-long treatment...
April 2018: Anthropology & Medicine
https://www.readbyqxmd.com/read/29531654/adult-sickle-cell-anaemia-patients-in-bone-pain-crisis-have-elevated-pro-inflammatory-cytokines
#15
Adekunle Emmanuel Alagbe, John Ayodele Olaniyi, Oladapo Wale Aworanti
Background and Objectives: Inflammatory markers that influence bone pain crisis (BPC) and other complications of sickle cell anaemia (SCA) are numerous and play various roles. This study determined the plasma levels of tumour necrosis factor (TNF) - α, interleukin - 8 (IL-8), and endothelin - 1 (ET-1) in adult SCA patients during BPC and in steady state. In addition, the plasma levels of these cytokines were correlated with the severity of BPC of the patients. Methods and Materials: Sixty adult SCA patients (30 during BPC and 30 during steady state) and 30 haemoglobin A controls were enrolled for this cross-sectional study...
2018: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/29531649/association-of-the-sod2-polymorphism-val16ala-and-sod-activity-with-vaso-occlusive-crisis-and-acute-splenic-sequestration-in-children-with-sickle-cell-anemia
#16
Isabela Cristina Cordeiro Farias, Taciana Furtado Mendonça-Belmont, Andreia Soares da Silva, Kleyton Palmeira do Ó, Felipe Ferreira, Fernanda Silva Medeiros, Luydson Richardson da Silva Vasconcelos, Marcos André Cavalcanti Bezerra, Aderson da Silva Araújo, Patricia Muniz Mendes Freire de Moura, Betânia Lucena Domingues Hatzlhofer, Ana Claudia Mendonça Dos Anjos, Maria do Socorro de Mendonça Cavalcanti
The SOD2 polymorphism Val16Ala T→C influences the antioxidative response. This study investigated the association of the SOD2 polymorphism and superoxide dismutase (SOD) activity with the vaso-occlusive crisis (VOC) and acute splenic sequestration (ASS) in children with sickle cell anemia (SCA). One hundred ninety-five children with SCA aged 1-9 years old were analyzed. The TC and CC genotypes were associated with lower SOD activity compared with the TT genotype (p=0.0321; p=0.0253, respectively). Furthermore, TC and CC were more frequent in patients with VOC or ASS (p=0...
2018: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/29524230/transfusion-transmitted-malaria-masquerading-as-sickle-cell-crisis-with-multisystem-organ-failure
#17
Cheryl L Maier, Phillip J Gross, Christina L Dean, Satheesh Chonat, Andrew Ip, Morgan McLemore, Fuad El Rassi, Sean R Stowell, Cassandra D Josephson, Ross M Fasano
BACKGROUND: Fever accompanying vaso-occlusive crisis is a common presentation in patients with sickle cell disease (SCD) and carries a broad differential diagnosis. Here, we report a case of transfusion-transmitted malaria in a patient with SCD presenting with acute vaso-occlusive crisis and rapidly decompensating to multisystem organ failure (MSOF). CASE REPORT: An 18-year-old African American male with SCD was admitted after multiple days of fever and severe generalized body pain...
March 9, 2018: Transfusion
https://www.readbyqxmd.com/read/29495591/chronic-administration-of-hydroxyurea-hu-benefits-caucasian-patients-with-sickle-beta-thalassemia
#18
Rosario Di Maggio, Matthew M Hsieh, Xiongce Zhao, Giuseppina Calvaruso, Paolo Rigano, Disma Renda, John F Tisdale, Aurelio Maggio
In sickle cell disease (SCD), hydroxyurea (HU) treatment decreases the number of vaso-occlusive crisis (VOC) and acute chest syndrome (ACS) by increasing fetal hemoglobin (HbF). Data are lacking regarding the frequency of HU dose modification or whether sub-therapeutic doses (<15 mg/kg/day) are beneficial. We reviewed the medical records of 140 patients from 2010 to 2014. The laboratory parameters and SCD complications were compared between the first and last visits based on HU use. Fifty patients (36%) never took HU or suspended HU ("no HU" group)...
February 28, 2018: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29473172/ceftriaxone-induced-drug-reaction-mimicking-acute-splenic-sequestration-crisis-in-a-child-with-hemoglobin-sc-disease
#19
Nancy L Van Buren, Jed B Gorlin, Robyn C Reed, Jerome L Gottschall, Stephen C Nelson
BACKGROUND: Acute splenic sequestration crisis is a complication of sickle cell disease (SCD) occurring when intrasplenic red blood cell (RBC) sickling prevents blood from leaving the spleen, causing acute splenic enlargement. Although typically seen in young children, it has been reported in older children with hemoglobin (Hb)SC disease, eventually resulting in functional asplenia. Ceftriaxone is a frequently used antibiotic of choice for children with SCD, because of its efficacy against invasive pneumococcal disease...
April 2018: Transfusion
https://www.readbyqxmd.com/read/29465435/clinical-manifestations-of-sickle-cell-disease-in-india-misconceptions-and-reality
#20
Dipty Jain, Dipika Mohanty
PURPOSE OF REVIEW: In the past, milder clinical manifestations of sickle cell disease (SCD) have been described from India. However, recent data from some parts of India suggest that the severity of the disease can be compared to that of African phenotypes. This review therefore describes the varied clinical manifestation of SCD, the success of newborn screening programme, prenatal diagnosis and low dose hydroxyurea therapy in India. RECENT FINDINGS: The varied clinical manifestations such as anemia, vaso-occlusive crisis, acute chest syndrome, renal involvement, stroke and so on vary from one part of the country to the other and also among different communities of India...
May 2018: Current Opinion in Hematology
keyword
keyword
73939
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"