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Sickle Cell Crisis

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https://www.readbyqxmd.com/read/29323695/lead-toxicity-in-the-pediatric-patient-with-sickle-cell-disease-unique-risks-and-management
#1
Josephine Misun Jung, Radhika Peddinti
Lead toxicity is the result of lead ingestion, one of the most common ingestions in the pediatric population. Nationwide and statewide efforts to recognize and curtail this epidemic have led to declining rates of toxicity. In patients with sickle cell disease (SCD), lead toxicity can be an elusive diagnosis due to overlapping symptom profiles, and inconsistent follow-up with a primary care physician can make the diagnosis even more difficult. In this article, two illustrative cases of lead toxicity in patients with SCD are described...
January 1, 2018: Pediatric Annals
https://www.readbyqxmd.com/read/29311011/-screening-for-nephropathy-in-major-sickle-cell-syndromes-in-patients-monitored-at-the-national-reference-center-for-sickle-cell-disease-in-niamey-niger
#2
Moumouni Garba, Zeinabou Maiga Moussa Tondi, Hassan Diongoule, Samaila Aboubacar, Abdou Ide, Nadège Ruddy Biyao-Nelson, Illiassou Soumaila, Soumana Alido
BACKGROUND: Sickle cell anemia is the most common hereditary hemopathy in the world. It is a disease that attacks all the systems of the organism. The kidneys are among the most sensitive organs of this disease. The main objective of this study is to detect sickle cell nephropathy in patients followed at the National Reference Center for Sickle Cell Disease in Niamey. METHODS: It is a prospective study carried out over a period of one year (January to December 2016)...
January 5, 2018: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/29296811/randomized-phase-2-trial-of-regadenoson-for-treatment-of-acute-vaso-occlusive-crises-in-sickle-cell-disease
#3
Joshua J Field, Elaine Majerus, Victor R Gordeuk, Michel Gowhari, Carolyn Hoppe, Matthew M Heeney, Maureen Achebe, Alex George, Hillary Chu, Brian Sheehan, Maneka Puligandla, Donna Neuberg, Gene Lin, Joel Linden, David G Nathan
Adenosine A2A receptor (A2AR) agonists have been shown to decrease tissue inflammation induced by hypoxia/reoxygenation in mice with sickle cell disease (SCD). The key mediator of the A2AR agonist's anti-inflammatory effects is a minor lymphocyte subset, invariant natural killer T (iNKT) cells. We tested the hypothesis that administration of an A2AR agonist in patients with SCD would decrease iNKT cell activation and dampen the severity of vaso-occlusive (VO) crises. In a phase 2, randomized, placebo-controlled trial, we administered a 48-hour infusion of the A2AR agonist regadenoson (1...
September 12, 2017: Blood Advances
https://www.readbyqxmd.com/read/29259931/bone-marrow-necrosis-and-fat-embolism-syndrome-a-dreadful-complication-of-hemoglobin-sickle-cell-disease
#4
Eduardo Pelegrineti Targueta, André Carramenha de Góes Hirano, Fernando Peixoto Ferraz de Campos, João Augusto Dos Santos Martines, Silvana Maria Lovisolo, Aloisio Felipe-Silva
Sickle cell disease encompasses a wide range of genotypic presentation with particular clinical features. The entity affects millions of people, particularly those whose ancestors came from sub-Saharan Africa and other countries in the Western Hemisphere, Saudi Arabia, and India. Currently, the high frequency of S and C genes reflects natural selection through the protection of heterozygotes against severe malaria, the high frequency of consanguineous marriages, improvement of some public health policies and the nutritional standards in the poorer countries where newborns are now living long enough to present for diagnosis and management...
October 2017: Autopsy & Case Reports
https://www.readbyqxmd.com/read/29236859/mentha-pulegium-crude-extracts-induce-thiol-oxidation-and-potentiate-hemolysis-when-associated-to-t-butyl-hydroperoxide-in-human-s-erythrocytes
#5
Matheus C Bianchini, Dennyura O Galvão, Tatiana Tamborena, Claudia O Alves, Robson L Puntel
Mentha pulegium (Lamiaceae) tea has been used as a traditional medicine; however, the modulatory effect of M. pulegium extracts on damage to human erythrocytes associated to t-butyl hydroperoxide (t-BHP) exposure remains to be investigated. Accordingly, we perform this study in order to test the hypothesis that aqueous and ethanolic extracts of M. pulegium could modulate the hemolysis associated to t-BHP exposure, non-protein thiol (NPSH) oxidation and lipid peroxidation (measured as thiobarbituric acid reactive substances - TBARS) in human erythrocytes...
December 11, 2017: Anais da Academia Brasileira de Ciências
https://www.readbyqxmd.com/read/29223211/perinatal-and-neonatal-implications-of-sickle-cell-disease
#6
Cathi Phillips, Margaret Peggy Boyd
Sickle cell disease is the genetic disorder most commonly detected with state-mandated newborn screening. Women with sickle cell disease struggle with psychosocial, emotional, and physical challenges throughout their lives. Pregnancy for women with sickle cell disease brings greater risk for maternal and fetal morbidity and mortality and increased likelihood of hospitalization for complications, including sickle cell pain crisis. Chronic maternal opioid use for pain can place newborns at risk for neonatal abstinence syndrome...
December 2017: Nursing for Women's Health
https://www.readbyqxmd.com/read/29222301/optimizing-the-care-model-for-an-uncomplicated-acute-pain-episode-in-sickle-cell-disease
#7
REVIEW
Paul Telfer, Banu Kaya
The pathophysiology, clinical presentation, and natural history of acute pain in sickle cell disease are unique and require a disease-centered approach that also applies general principles of acute and chronic pain management. The majority of acute pain episodes are managed at home without the need to access health care. The long-term consequences of poorly treated acute pain include chronic pain, adverse effects of chronic opioid usage, psychological maladjustment, poor quality of life, and excessive health care utilization...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29222291/evolving-treatment-paradigms-in-sickle-cell-disease
#8
REVIEW
Ramasamy Jagadeeswaran, Angela Rivers
Sickle cell disease (SCD) is an inheritable hemoglobinopathy characterized by polymerization of hemoglobin S in red blood cells resulting in chronic hemolytic anemia, vaso-occlusive painful crisis, and multiorgan damage. In SCD, an increased reactive oxygen species (ROS) generation occurs both inside the red blood cells and inside the vascular lumen, which augment hemolysis and cellular adhesion. This review discusses the evolving body of literature on the role of ROS in the pathophysiology of SCD as well as some emerging therapeutic approaches to SCD with a focus on the reduction of ROS...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29183008/correlates-of-pulmonary-function-in-children-with-sickle-cell-disease-and-elevated-fetal-hemoglobin
#9
Adekunle D Adekile, Asmaa Azab, Abdullah Owayed, Mousa Khadadah
OBJECTIVE: The current study was carried out to compare pulmonary function tests (PFT) in pediatric Kuwaiti sickle cell disease (SCD) patients to age-matched normal controls and to investigate the association of PFTS to selected clinical and laboratory parameters. SUBJECTS AND METHODS: There were 38 patients with SCD and 36 controls in the study. The patients were recruited from the Pediatric Hematology Clinics of Mubarak Al-Kabeer and Al-Amiri Hospitals, Kuwait and were studied in steady state...
November 28, 2017: Medical Principles and Practice: International Journal of the Kuwait University, Health Science Centre
https://www.readbyqxmd.com/read/29182088/perinatal-maternal-mortality-in-sickle-cell-anemia-two-case-reports-and-review-of-the-literature
#10
Sanaa Rizk, Elizabeth D Pulte, David Axelrod, Samir K Ballas
As outcomes of patients with sickle cell anemia improve and survival into adulthood with good quality of life and expectation of long-term survival becomes more common, challenges have developed, including issues related to reproduction. Pregnancy is frequently complicated in patients with sickle cell anemia with mortality up to 4.0%. Here we report maternal perinatal mortality in two women with sickle cell anemia who died post-partum due to acute chest syndrome (ACS), caused by bone marrow fat embolism and review the literature pertinent to this subject...
November 28, 2017: Hemoglobin
https://www.readbyqxmd.com/read/29174516/retracted-delayed-hemolytic-transfusion-reaction-and-hyperhemolysis-syndrome-without-detectable-alloantibodies-or-autoantibodies-in-a-patient-with-sickle-cell-disease-a-fatal-case-report-and-literature-review
#11
Basile Nsimba
L'éditeur a le regret de vous informer que cet article a déjà été publié dans: Journal of Blood Disorders & Transfusion, 2017, 8:4. DOI: 10.4172/2155-9864.1000388. Cette seconde publication faite par erreur a été retirée.
November 22, 2017: Transfusion Clinique et Biologique: Journal de la Société Française de Transfusion Sanguine
https://www.readbyqxmd.com/read/29170352/55%C3%A2-mini-sim-innovative-bite-sized-simulation-teaching-in-a-busy-children-s-emergency-department
#12
Rachel Sunley, Karen Moloney, Jessica Parker, Christopher Arrowsmith, Kirsty Brown, Alex Wilson
: Emergency Medicine requires a highly skilled workforce who are passionate about delivering excellent patient care. Shift patterns linked with the ever increasing numbers of patients who attend Emergency Departments puts strain on educating the workforce and fostering team togetherness.Our objective in devising and instigating the 'Mini Sim' programme was to embed regular in-situ simulation training to enhance the learning of all staff within our Emergency Department team, building a highly trained workforce to deliver excellent care within the remit of our busy department...
December 2017: Emergency Medicine Journal: EMJ
https://www.readbyqxmd.com/read/29152307/coagulation-profile-of-sudanese-children-with-homozygous-sickle-cell-disease-and-the-effect-of-treatment-with-omega-3-fatty-acid-on-the-coagulation-parameters
#13
Shiekh Awoda, Ahmed A Daak, Nazik Elmalaika Husain, Kebreab Ghebremeskel, Mustafa I Elbashir
Background: It has been reported that patients with SCD do have an abnormal coagulation profile. Coagulopathy is thought to be one of the key factors that contribute to the vaso-occlusive crisis that characterises sickle cell disease (SCD). In this study, we investigated whether Sudanese sickle cell patients have an abnormal coagulation profile. In addition, the effect of treatment with either omega-3 fatty acids or hydroxyurea on coagulation profile was assessed. Methods: Homozygous SCD patients untreated (n = 52), omega-3 treated (n = 44), hydroxyurea (HU) treated (n = 8) and healthy (HbAA) controls (n = 52) matched for age (4-20 years), gender and socioeconomic status were enrolled...
2017: BMC Hematology
https://www.readbyqxmd.com/read/29134776/acute-chest-syndrome-among-children-hospitalized-with-vaso-occlusive-crisis-a-nationwide-study-in-the-united-states
#14
Takuto Takahashi, Yusuke Okubo, Atsuhiko Handa
PURPOSE: Acute chest syndrome (ACS) is a common complication among pediatric inpatients with sickle cell disease and vaso-occlusive crisis (VOC). However, little is known about the factors associated with ACS complication. The present study assessed the epidemiological features of children hospitalized with VOC and ascertained factors associated with ACS complication. METHODS: Hospital discharge records of patients with VOC aged <20 years were obtained for the years 2003, 2006, 2009, and 2012 from the Kids' Inpatient Database...
November 14, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29107441/real-life-experience-with-hydroxyurea-in-sickle-cell-disease-a-multicenter-study-in-a-cohort-of-patients-with-heterogeneous-descent
#15
Paolo Rigano, Lucia De Franceschi, Laura Sainati, Antonio Piga, Frédéric B Piel, Maria Domenica Cappellini, Carmelo Fidone, Nicoletta Masera, Giovanni Palazzi, Barbara Gianesin, Gian Luca Forni
We conducted the first nation-wide cohort study of sickle cell disease (SCD) in Italy, a Southern European country exposed to intense recent flux migration from endemic areas for SCD. We evaluate the impact of hydroxyurea on a total of 652 pediatric and adult patients from 33 Reference Centers for SCD (mean age 24.5±15years, 51.4% males). Hydroxyurea median treatment duration was 7years (range: <1year to 29years) at a mean therapeutic dose of 18±4.7mg/kg/day. Hydroxyurea was associated with a significant increase in mean total and fetal hemoglobin and a significant decrease in mean hemoglobin S, white blood and platelet counts, and lactate dehydrogenase levels...
October 9, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/29099347/the-morbidity-pattern-of-children-with-sickle-cell-disorders-admitted-to-the-queen-elizabeth-hospital-barbados-2009-2013
#16
D Grannum, Paula M Lashley
A retrospective study was conducted by examining all the medical files of all the children with sickle cell anaemia (Hb SS) admitted from 1 January 2009 to 31 December 2013. A total of 220 admission notes (59 patients aged 5 months-16 years) were analysed. Of these, 53.2% were boys; 85.8% of the patients had Hb SS. The most common reason for admission was vaso-occlusive crisis. Blood transfusions were used in 39.7% of admissions There were no deaths during the study period. We note the number of sickle cell-related admissions and morbidity has increased, along with an increase in the number of blood transfusions...
January 1, 2017: Tropical Doctor
https://www.readbyqxmd.com/read/29078924/sickle-cell-disease-in-the-emergency-department
#17
REVIEW
Paris B Lovett, Harsh P Sule, Bernard L Lopez
Acute painful episodes are the most common reason for emergency department visits among patients with sickle cell disease (SCD). Early and aggressive pain management is a priority. Emergency providers (EPs) must also diagnose other emergent diagnoses in patients with SCD and differentiate them from vaso-occlusive crisis. EPs should be aware of cognitive biases that may misdirect the diagnostic process. Administration of intravenous fluids should be used judiciously. Blood transfusion may be considered. Coordination of care with hematology is an important part of the effective emergency department and long-term management of patients with SCD...
December 2017: Hematology/oncology Clinics of North America
https://www.readbyqxmd.com/read/29076161/use-of-the-blood-substitute-hboc-201-in-critically-ill-patients-during-sickle-crisis-a-three-case-series
#18
Jonathan M Davis, Nura El-Haj, Nimish N Shah, Garry Schwartz, Margaret Block, James Wall, Mark Tidswell, Ernest DiNino
BACKGROUND: Red blood cell (RBC) transfusion is an important treatment modality during severe sickle cell crisis (SCC). SCC patients who refuse, or cannot accept, RBCs present a unique challenge. Acellular hemoglobin (Hb)-based oxygen carriers (HBOCs) might be an alternative for critically ill patients in SCC with multiorgan failure due to life-threatening anemia. HBOC-201 (HbO2 Therapeutics) has been administered to more than 800 anemic patients in 22 clinical trials, but use of any HBOCs in critically ill sickle cell patients with organ failure is exceedingly rare...
October 26, 2017: Transfusion
https://www.readbyqxmd.com/read/29051184/novel-use-of-hydroxyurea-in-an-african-region-with-malaria-noharm-a-trial-for-children-with-sickle-cell-anemia
#19
Robert O Opoka, Christopher M Ndugwa, Teresa S Latham, Adam Lane, Heather A Hume, Phillip Kasirye, James S Hodges, Russell E Ware, Chandy C John
Hydroxyurea treatment is recommended for children with sickle cell anemia (SCA) living in high-resource malaria-free regions, but its safety and efficacy in malaria-endemic sub-Saharan Africa, where the greatest sickle cell burden exists, remain unknown. In vitro studies suggest hydroxyurea could increase malaria severity, and hydroxyurea-associated neutropenia could worsen infections. NOHARM was a randomized, double-blinded, placebo-controlled trial conducted in malaria-endemic Uganda, comparing hydroxyurea to placebo at 20 ± 2...
October 19, 2017: Blood
https://www.readbyqxmd.com/read/29039292/an-innovative-short-stay-health-care-model-for-treatment-of-uncomplicated-vaso-occlusive-crisis-in-adult-sickle-cell-disease-patients-in-canada-to-reduce-emergency-department-utilization
#20
Andrew Binding, Richard Ward, Chai Phua, Veronique Naessens, Tara O'Brien, Sacha Bhatia, Hayley Baranek, Husayn Marani, Geetha Mukerji
OBJECTIVES: Patients with sickle cell disease (SCD) with vaso-occlusive crises (VOC) often visit the emergency department (ED) for management of painful episodes. The primary objective of this pilot study was to evaluate the acceptability of a short-stay model for treatment of VOC in SCD outside of the ED in Toronto, Canada. Secondary objectives were to assess patient satisfaction of this model, barriers to its use and comparison of clinical outcomes to a historical control. METHODS: Adult SCD patients with symptoms of an uncomplicated VOC between October 2014 to July 2016 were managed according to best practice recommendations in a short-stay unit as an alternative to the local emergency room...
October 17, 2017: CJEM
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