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https://www.readbyqxmd.com/read/29127678/allogeneic-matched-related-transplantation-for-%C3%AE-thalassemia-and-sickle-cell-anemia
#1
Françoise Bernaudin, Corinne Pondarré, Claire Galambrun, Isabelle Thuret
Allogeneic hematopoietic stem cell transplantation (HSCT) can cure single gene disorders such as thalassemia and sickle cell anemia (SCA). These non-malignant diseases have in common severe hemolytic anemia and high proliferative bone marrow, requiring frequent transfusions. The risk of rejection is high and graft-vs-host disease is not desirable. Important progress has been made in the management of these diseases, including leukocyte depletion of blood products, and chelation therapy, for both diseases, and erythrocytapheresis and hydroxycarbamide for SCA...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/29127677/current-standards-of-care-and-long-term-outcomes-for-thalassemia-and-sickle-cell-disease
#2
Satheesh Chonat, Charles T Quinn
Thalassemia and sickle cell disease (SCD) are disorders of hemoglobin that affect millions of people worldwide. The carrier states for these diseases arose as common, balanced polymorphisms during human history because they afforded protection against severe forms of malaria. These complex, multisystem diseases are reviewed here with a focus on current standards of clinical management and recent research findings. The importance of a comprehensive, multidisciplinary and lifelong system of care is also emphasized...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/29127676/genetic-basis-and-genetic-modifiers-of-%C3%AE-thalassemia-and-sickle-cell-disease
#3
Swee Lay Thein
β-thalassemia and sickle cell disease (SCD) are prototypical Mendelian single gene disorders, both caused by mutations affecting the adult β-globin gene. Despite the apparent genetic simplicity, both disorders display a remarkable spectrum of phenotypic severity and share two major genetic modifiers-α-globin genotype and innate ability to produce fetal hemoglobin (HbF, α2γ2).This article provides an overview of the genetic basis for SCD and β-thalassemia, and genetic modifiers identified through phenotype correlation studies...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/29112240/splenectomy-versus-conservative-management-for-acute-sequestration-crises-in-people-with-sickle-cell-disease
#4
REVIEW
Shirley Owusu-Ofori, Tracey Remmington
BACKGROUND: Acute splenic sequestration crises are a complication of sickle cell disease, with high mortality rates and frequent recurrence in survivors of first attacks. Splenectomy and blood transfusion, with their consequences, are the mainstay of long-term management used in different parts of the world. This is a 2017 update of a Cochrane Review first published in 2002, and previously updated, most recently in 2015. OBJECTIVES: To assess whether splenectomy (total or partial), to prevent acute splenic sequestration crises in people with sickle cell disease, improved survival and decreased morbidity in people with sickle cell disease, as compared with regular blood transfusions...
November 7, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29100558/immunohaematological-complications-in-patients-with-sickle-cell-disease-after-haemopoietic-progenitor-cell-transplantation-a-prospective-single-centre-observational-study
#5
Elizabeth S Allen, Kshitij Srivastava, Matthew M Hsieh, Courtney D Fitzhugh, Harvey G Klein, John F Tisdale, Willy A Flegel
BACKGROUND: Haemopoietic progenitor cell (HPC) transplantation can cure sickle cell disease. Non-myeloablative conditioning typically results in donor-derived erythrocytes and stable mixed chimerism of recipient-derived and donor-derived leucocytes. Exposure to donor antigens from the HPC graft and new red cell antibodies induced by transfusion can lead to immunohaematological complications. We assessed the incidence of such complications among HPC transplant recipients with sickle cell disease...
November 2017: Lancet Haematology
https://www.readbyqxmd.com/read/29094643/parent-perspectives-on-pain-management-in-preschool-age-children-with-sickle-cell-disease
#6
Kelsey Smith, Laura Reinman, Jeffrey Schatz, Carla W Roberts
Pain episodes occur for many preschoolers with sickle cell disease (SCD), but little is known about parent perceptions of managing pain episodes in young children. We surveyed parents of young children with SCD who had managed pain episodes in the past year to assess their management and satisfaction with their strategies, challenges of pain management, and interest in additional education. Parents were recruited from health maintenance visits at a SCD specialty clinic. Forty-two of 51 parents (82%) of 2- to-6-year-olds reported managing pain over the past year...
November 1, 2017: Journal of Pediatric Oncology Nursing: Official Journal of the Association of Pediatric Oncology Nurses
https://www.readbyqxmd.com/read/29078924/sickle-cell-disease-in-the-emergency-department
#7
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/29048948/the-spectrum-of-sickle-hemoglobin-related-nephropathy-from-sickle-cell-disease-to-sickle-trait
#8
Rakhi P Naik, Vimal K Derebail
Renal dysfunction is among the most common complication of sickle cell disease (SCD), from hyposthenuria in children to progression to overt chronic kidney disease (CKD) in young adults. Emerging evidence now suggests that sickle hemoglobin-related nephropathy extends to individuals with sickle cell trait (SCT). Areas covered: This review will highlight the pathophysiology, epidemiology, and management recommendations for sickle nephropathy in both SCD and SCT. In addition, it will focus on the major demographic and genetic modifiers of renal disease in sickling hemoglobinopathies...
October 19, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/29048590/family-functioning-medical-self-management-and-health-outcomes-among-school-aged-children-with-sickle-cell-disease-a-mediation-model
#9
Alexandra M Psihogios, Lauren C Daniel, Reem Tarazi, Kim Smith-Whitley, Chavis A Patterson, Lamia P Barakat
Background: Informed by the Pediatric Self-Management Model, the present study tested relationships between parent and family functioning, sickle cell disease (SCD) self-management, and health outcomes for children with SCD. Method: 83 children with SCD and a parent completed baseline data as part of a larger investigation of a family-based, problem-solving intervention for children with SCD (M age = 8.47). Youth and parents completed a measure of child health-related quality of life (HRQOL), and parents completed measures of family efficacy, parenting stress, and SCD self-management...
September 26, 2017: Journal of Pediatric Psychology
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
#10
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
https://www.readbyqxmd.com/read/29033725/assessment-of-patient-controlled-analgesia-versus-intermittent-opioid-therapy-to-manage-sickle-cell-disease-vaso-occlusive-crisis-in-adult-patients
#11
Alaa Al-Anazi, Lowloa Al-Swaidan, Maha Al-Ammari, Tariq Al-Debasi, Abdulmalik M Alkatheri, Shmeylan Al-Harbi, Aiman A Obaidat, Abdulkareem M Al-Bekairy
BACKGROUND: Vaso-occlusive crisis (VOC) is one of the acute complications of sickle-cell disease (SCD). Treatment mainly relies on hydration and pain control by analgesics. The specific aim of this study was to assess potential health outcomes within the first 72 h of admission between intermittent and patient-controlled analgesia (PCA) by opioids among VOC patients. METHODS: A retrospective chart review study was conducted to determine SCD patients with VOC. Using the hospital electronic system, the following data were collected: patient's age, gender, blood pressure, heart rate, respiratory rate, oxygen saturation, and pain score on admission and daily for 3 days as well as the cumulative opioid analgesic dose for 72 h which is reported as morphine equivalent...
October 2017: Saudi Journal of Anaesthesia
https://www.readbyqxmd.com/read/29026400/hydroxyurea-therapy-in-adult-nigerian-sickle-cell-disease-a-monocentric-survey-on-pattern-of-use-clinical-effects-and-patient-s-compliance
#12
Ademola Samson Adewoyin, Omokiniovo Sunday Oghuvwu, Omolade Augustina Awodu
BACKGROUND: The clinical prospects of hydroxyurea therapy in the management of sickle cell disease (SCD) require evaluation in the Nigerian setting to develop indigenous guidelines. This survey examines the pattern of hydroxyurea therapy, its clinico-haematologic benefits and safety profile in Nigerian SCD subjects. METHODS: A cross sectional pilot survey was carried out among 60 adult SCD subjects over 3 months. Data on clinical phenotypes, relevant haematological parameters and details of hydroxyurea therapy were obtained using a structured questionnaire through an interview process and case file review...
March 2017: African Health Sciences
https://www.readbyqxmd.com/read/29023896/predicting-changes-in-hemoglobin-s-after-simple-transfusion-using-complete-blood-counts
#13
Gagan Mathur, Patrick Ten Eyck, C Michael Knudson
BACKGROUND: Hemoglobin S percentages are used in the management of patients who have sickle cell disease. However, hemoglobin S measurements often are not routinely or rapidly performed. Rapid and accurate methods to estimate hemoglobin S levels after simple transfusion may improve the care of patients with sickle cell disease. STUDY DESIGN AND METHODS: A comprehensive review of the electronic medical record identified 24 stable patients with sickle cell disease who received simple red blood cell transfusions and had hemoglobin S measurements before and after the transfusion that were less than 72 hours apart...
October 11, 2017: Transfusion
https://www.readbyqxmd.com/read/28988427/hydroxyurea-therapy-in-uk-children-with-sickle-cell-anaemia-a-single-centre-experience
#14
Kate Phillips, Laura Healy, Louise Smith, Russell Keenan
INTRODUCTION: Despite the demonstrated efficacy of hydroxyurea therapy, children with sickle cell anaemia in the UK are preferentially managed with supportive care or transfusion. Hydroxyurea is reserved for children with severe disease phenotype. This is in contrast to North America and other countries where hydroxyurea is widely used for children of all clinical phenotypes. The conservative UK practice may in part be due to concerns about toxicity, in particular marrow suppression with high doses, and growth in children...
October 8, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28988213/management-of-pneumatosis-intestinalis-in-children-over-the-age-of-6-months-a-conservative-approach
#15
Leel Nellihela, Mohamed Mutalib, David Thompson, Kammermeier Jochen, Manasvi Upadhyaya
BACKGROUND: Pneumatosis intestinalis (PI) is an uncommon and poorly understood condition. Although it can be an incidental finding in asymptomatic individuals, it can also be secondary to life-threatening bowel ischaemia and sepsis. In premature infants, it is a pathognomonic sign of necrotising enterocolitis. There is no consensus regarding management and long-term outcome of children with PI. AIM: Review of our experience of PI in children beyond the early infantile period...
October 7, 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/28978568/impact-of-extracellular-fluid-tonicity-on-sickle-red-blood-cell-deformability-and-adhesion
#16
Marcus A Carden, Meredith E Fay, Xinran Lu, Robert G Mannino, Yumiko Sakurai, Jordan C Ciciliano, Caroline E Hansen, Satheesh Chonat, Clinton H Joiner, David K Wood, Wilbur A Lam
Abnormal sickle red blood cell (sRBC) biomechanics, including pathological deformability and adhesion, correlate with clinical severity in sickle cell disease (SCD). Clinical intravenous fluids (IVFs) of various tonicities are often used during treatment of vaso-occlusive pain episodes (VOE), the major cause of morbidity in SCD. However, evidence-based guidelines are lacking and there is no consensus regarding which IVFs to use during VOE. Further, it is unknown how altering extracellular fluid tonicity with IVFs affects sRBC biomechanics in the microcirculation, where vaso-occlusion takes place...
October 4, 2017: Blood
https://www.readbyqxmd.com/read/28977106/daily-use-of-phosphodiesterase-type-5-inhibitors-as-prevention-for-recurrent-priapism
#17
Archimedes Nardozza, Marcelo Rodrigues Cabrini
OBJECTIVE: The pathogenesis of recurrent priapism is currently being investigated based on the regulation of the phosphodiesterase 5 (PDE5) enzyme. We explored the daily use of PDE5 inhibitors to treat and prevent priapism recurrences. METHOD: We administered PDE5 inhibitors using a long-term therapeutic regimen in seven men with recurrent priapism, with a mean age of 29.2 years (range 21 to 35 years). Six men (85.7%) had idiopathic priapism recurrences and one man (24...
August 2017: Revista da Associação Médica Brasileira
https://www.readbyqxmd.com/read/28946013/manifestations-of-sickle-cell-disease-on-thoracic-imaging
#18
REVIEW
Mindy X Wang, Eric W Pepin, Nupur Verma, Tan-Lucien Mohammed
Sickle cell disease is an inherited hemolytic disease with systemic complications. These complications significantly impact the patients' quality of life. There are characteristic radiological findings that can aid in the diagnosis and management of common sequelae of sickle cell disease. In this review, we will discuss the thoracic imaging findings of common complications of sickle cell disease in an organ-based approach and provide imaging examples of them.
September 8, 2017: Clinical Imaging
https://www.readbyqxmd.com/read/28941954/a-parental-report-of-youth-transition-readiness-the-parent-starx-questionnaire-starx-p-and-re-evaluation-of-the-starx-child-report
#19
Meaghan Nazareth, Laura Hart, Maria Ferris, Eniko Rak, Stephen Hooper, Miranda A L van Tilburg
PURPOSE: The STARx Questionnaire is a self-report measure of health care transition (HCT) readiness in youth with chronic diseases. We aimed to improve reliability and generalizability of the STARx and report initial reliability data on the STARx-P Questionnaire, a self-report measure of parent perspective on their child's HCT readiness. METHODS: Participants were recruited in several clinics from a large academic hospital in the southeastern USA and via the therapeutic summer camp for children with chronic disease...
September 20, 2017: Journal of Pediatric Nursing
https://www.readbyqxmd.com/read/28939451/hla-haploidentical-stem-cell-transplant-with-pretransplant-immunosuppression-for-patients-with-sickle-cell-disease
#20
Anna B Pawlowska, Jerry C Cheng, Nicole A Karras, Weili Sun, Leo D Wang, Alison D Bell, Lisa Gutierrez, Joseph Rosenthal
Allogeneic stem cell transplantation (HCT) is curative in patients with severe sickle cell disease (SCD), but a significant number of patients lack an HLA-identical sibling or matched unrelated donor. Mismatched related (haploidentical) HCT with post-transplant cyclophosphamide (PTCY) allows expansion of the donor pool but is complicated by high rates of graft failure. In this report we describe a favorable haploidentical HCT approach in a limited cohort of SCD patients with significant comorbidities. To reduce the risk of graft failure we administered the conditioning regimen of rabbit antithymocyte globulin, busulfan, and fludarabine preceded with 2 courses of pretransplant immunosuppressive therapy (PTIS) with fludarabine and dexamethasone...
September 20, 2017: Biology of Blood and Marrow Transplantation
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