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sickle cell disease managment

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https://www.readbyqxmd.com/read/29764840/how-we-diagnose-and-treat-venous-thromboembolism-in-sickle-cell-disease
#1
Arun S Shet, Theodore Wun
The incidence of venous thromboembolism (VTE) in adult patients with sickle cell disease (SCD) is high. However, overlapping features between the clinical presentation of VTE and SCD complications and a low index of suspicion for thrombosis can influence patient management decisions. VTE in SCD can therefore present management challenges to the clinical hematologist. Herein, we present three distinct clinical vignettes that are representative of our clinical practice with SCD patients. These vignettes are discussed with specific reference to the hypercoagulable state in SCD patients, recent VTE diagnosis and anticoagulant therapy guidelines from the general population, and evaluation of the risk of bleeding as a result of long term exposure to anticoagulant therapy...
May 15, 2018: Blood
https://www.readbyqxmd.com/read/29755709/perioperative-management-of-sickle-cell-disease
#2
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/29751732/population-screening-for-hemoglobinopathies
#3
H W Goonasekera, C S Paththinige, V H W Dissanayake
Hemoglobinopathies are the most common single-gene disorders in the world. Their prevalence is predicted to increase in the future, and low-income hemoglobinopathy-endemic regions need to manage most of the world's affected persons. International organizations, governments, and other stakeholders have initiated national or regional prevention programs in both endemic and nonendemic countries by performing population screening for α- and β-thalassemia, HbE disease, and sickle cell disease in neonates, adolescents, reproductive-age adults (preconceptionally or in the early antenatal period), and family members of diagnosed cases...
May 11, 2018: Annual Review of Genomics and Human Genetics
https://www.readbyqxmd.com/read/29749702/management-of-severe-chronic-pain-with-methadone-in-pediatric-patients-with-sickle-cell-disease
#4
Zachary LeBlanc, Chris Vance, Jason Payne, Jie Zhang, Lee Hilliard, Jeffrey D Lebensburger, Thomas H Howard
Vasocclusive pain crises are common among pediatric patients with sickle cell disease (SCD). Some patients with repeated pain crises develop chronic pain. We performed a retrospective cohort study of pediatric patients with SCD with chronic pain treated with methadone. We identified a significant reduction in pain hospitalizations following methadone treatment (0.35 ± 0.19 vs. 0.19 ± 0.17 hospitalizations/month, P = 0.016). In addition, we did not observe overt organ toxicity nor symptoms of opioid withdrawal during methadone wean...
May 11, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29737522/interventions-for-improving-adherence-to-iron-chelation-therapy-in-people-with-sickle-cell-disease-or-thalassaemia
#5
REVIEW
Patricia M Fortin, Sheila A Fisher, Karen V Madgwick, Marialena Trivella, Sally Hopewell, Carolyn Doree, Lise J Estcourt
BACKGROUND: Regularly transfused people with sickle cell disease (SCD) and people with thalassaemia (who are transfusion-dependent or non-transfusion-dependent) are at risk of iron overload. Iron overload can lead to iron toxicity in vulnerable organs such as the heart, liver and endocrine glands; which can be prevented and treated with iron chelating agents. The intensive demands and uncomfortable side effects of therapy can have a negative impact on daily activities and well-being, which may affect adherence...
May 8, 2018: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29729787/key-components-of-pain-management-for-children-and-adults-with-sickle-cell-disease
#6
REVIEW
Amanda M Brandow, Michael R DeBaun
Sickle cell disease pain manifests as severe acute pain episodes and a debilitating chronic pain syndrome. Acute pain episodes are the most common reason for health care use; however, acute pain episodes are also frequently managed at home. Chronic pain syndrome develops in 30% to 40% of individuals with sickle cell disease, with an increasing incidence and severity with age. We review the critical aspects of pain management that are integral to the comprehensive approach to sickle cell disease pain and are rooted in the biopsychosocial model...
June 2018: Hematology/oncology Clinics of North America
https://www.readbyqxmd.com/read/29724898/how-i-safely-transfuse-patients-with-sickle-cell-disease-and-manage-delayed-hemolytic-transfusion-reactions
#7
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/29693797/mobile-health-intervention-for-youth-with-sickle-cell-disease-impact-on-adherence-disease-knowledge-and-quality-of-life
#8
Lindsay M Anderson, Sarah Leonard, Jude Jonassaint, Joseph Lunyera, Melanie Bonner, Nirmish Shah
BACKGROUND: Adherence to illness self-management among youth with sickle cell disease (SCD) positively impacts health outcomes and decreases overall healthcare costs. Despite this, children with SCD face several barriers to adherence, with adherence rates that remain moderate to low. The current feasibility study examined the Intensive Training Program (ITP), a mobile health (mHealth) intervention for youth with SCD designed to promote disease knowledge, adherence, and patient-provider communication...
April 25, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29666710/acute-chest-syndrome-progressing-to-ards-in-a-patient-of-25-week-gestation
#9
Jefferson Chambers, Nichole Smith, Matthew Sehring, Subramanyam Chittivelu
Acute chest syndrome is a complication of sickle cell disease and represents the highest cause of mortality in those afflicted with the disorder. Pregnancy represents an increased risk for complications of sickle cell disease in both the mother and fetus. We present a case of a 20-year-old patient with known sickle cell disease who was at 25-week gestation and developed acute chest syndrome refractory to conventional therapies and requiring emergency cesarean section. Following delivery, the patient developed acute respiratory distress syndrome (ARDS) requiring extracorporeal membrane oxygenation (ECMO)...
2018: Case Reports in Critical Care
https://www.readbyqxmd.com/read/29663858/assessment-of-liver-and-cardiac-iron-overload-using-mri-in-patients-with-chronic-anemias-in-latin-american-countries-results-from-asimila-study
#10
Rodolfo Cancado, Nora P Watman, Clarisse Lobo, Zulay Chona, Fernando Manzur, Fabiola Traina, Miriam Park, Guillermo Drelichman, Juan Pablo Zarate, Luis Marfil
OBJECTIVES: A multicenter, noninterventional, observational study was conducted in the Latin American countries including Argentina, Brazil, Colombia, Mexico, and Venezuela to assess the prevalence of liver and cardiac iron overload using magnetic resonance imaging (MRI) in patients with chronic anemias except thalassemia. METHODS: Patients aged >10 years with transfusion-dependent anemias, except thalassemia, either with <20 units of red blood cell (RBC) transfusions with serum ferritin (SF) levels >2000 ng/mL or with ≥20 units of RBC transfusions regardless of SF level in their lifetime, were enrolled...
April 17, 2018: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/29659914/cognitive-abilities-moderate-the-effect-of-disease-severity-on-health-related-quality-of-life-in-pediatric-sickle-cell-disease
#11
Steven J Hardy, Sarah E Bills, Shane M Wise, Kristina K Hardy
Objective: Complications that can arise from sickle cell disease (SCD) have the potential to negatively affect health-related quality of life (HRQL). SCD manifests in varying degrees of severity, but effects on HRQL are not uniform. Cognitive abilities influence HRQL in other pediatric groups, potentially through variability in treatment adherence and psychological coping. This study examined the effect of SCD severity on HRQL and explored cognitive abilities as a moderator of this relationship...
April 6, 2018: Journal of Pediatric Psychology
https://www.readbyqxmd.com/read/29653206/late-effects-screening-guidelines-after-hematopoietic-cell-transplantation-hct-for-hemoglobinopathy-consensus-statement-from-the-second-pediatric-blood-and-marrow-transplant-consortium-international-conference-on-late-effects-after-pediatric-hct
#12
Shalini Shenoy, Javid Gaziev, Emanuele Angelucci, Allison King, Monica Bhatia, Angela Smith, Dorine Bresters, Anne E Haight, Christine N Duncan, Josu de la Fuente, Andrew C Dietz, K Scott Baker, Michael A Pulsipher, Mark C Walters
Allogeneic hematopoietic cell transplantation (HCT) can halt organ damage and eliminate symptoms in hemoglobin disorders, including sickle cell disease (SCD) and thalassemia major (TM). Managing the residual manifestations of pre-HCT disease complications and the long-term effects of HCT requires systematic monitoring, follow-up, and intervention when indicated. Late complications vary with age and disease status at HCT, and with transplant variables such as preparative regimen, donor source and compatibility, and immune reconstitution...
April 10, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29652215/stigma-of-sickle-cell-disease-a-systematic-review
#13
Dominique Bulgin, Paula Tanabe, Coretta Jenerette
The aim of this systematic review was to synthesize the literature regarding health-related stigma in adolescents and adults living with sickle cell disease (SCD). Four domains were identified from 27 studies: 1) social consequences of stigma, 2) the effect of stigma on psychological well-being, 3) the effect of stigma on physiological well-being, and 4) the impact of stigma on patient-provider relationships and care-seeking behaviors. Current literature revealed that SCD stigma has detrimental consequences...
April 13, 2018: Issues in Mental Health Nursing
https://www.readbyqxmd.com/read/29648482/acute-chest-syndrome-in-sickle-cell-disease
#14
Sajid Farooq, Mohannad Abu Omar, Gary A Salzman
Acute chest syndrome (ACS) is a leading complication of sickle cell disease (SCD) with significant morbidity and mortality. ACS is the most common cause of death and the second most common cause of hospitalization in patients with SCD. Delineating the specific cause of ACS is often difficult, and multiple risk factors that precipitate ACS frequently coexist. The prominent risk factors include infection, hypoxia, bronchial hyperresponsiveness, the SCD genotype, and opioid use. The key to the successful treatment of ACS is early recognition and initiation of treatment without delay...
April 12, 2018: Hospital Practice (Minneapolis)
https://www.readbyqxmd.com/read/29642733/warfarin-patients-with-anemia-show-trend-of-out-of-range-international-normalized-ratio-frequency-with-point-of-care-testing-in-an-anticoagulation-clinic
#15
Christina E DeRemer, Bliss McMichael, Henry N Young
INTRODUCTION: Many factors influence international normalized ratio (INR); however, few studies have examined the impact of anemia in warfarin patients. The primary objective of this study was to explore the relationship between in-clinic anemia and the control of INR within an anticoagulation clinic. METHODS: A retrospective chart review was performed on a random sample of patients seen in an academic medical center pharmacy-managed anticoagulation clinic. Hemochron® Signature Elite machine was utilized to monitor point-of-care (POC) INR...
January 1, 2018: Journal of Pharmacy Practice
https://www.readbyqxmd.com/read/29624481/craniofacial-manifestations-of-systemic-disorders-ct-and-mr-imaging-findings-and-imaging-approach
#16
V Carlota Andreu-Arasa, Margaret N Chapman, Hirofumi Kuno, Akifumi Fujita, Osamu Sakai
Many systemic diseases or conditions can affect the maxillofacial bones; however, they are often overlooked or incidentally found at routine brain or head and neck imaging performed for other reasons. Early identification of some conditions may significantly affect patient care and alter outcomes. Early recognition of nonneoplastic hematologic disorders, such as thalassemia and sickle cell disease, may help initiate earlier treatment and prevent serious complications. The management of neoplastic diseases such as lymphoma, leukemia, or Langerhans cell histiocytosis may be different if diagnosed early, and metastases to the maxillofacial bones may be the first manifestation of an otherwise occult neoplasm...
April 6, 2018: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/29620434/transient-receptor-potential-polymorphism-and-haplotype-associate-with-crisis-pain-in-sickle-cell-disease
#17
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/29614633/vascular-complications-of-sickle-cell-disease
#18
Ashar Usmani, Roberto F Machado
Sickle cell disease (SCD) is a monogenetic disorder caused by a mutation in the β-globin gene HBB leading to polymerization of red blood cells causing damage to cell membranes, increasing its rigidity and intravascular hemolysis. Multiple lines of evidence suggest that SCD can be viewed as pan-vasculopathy associated with multiple mechanisms but driven by hemoglobin S polymerization. Here we review the pathophysiology, clinical manifestations and management strategies for cerebrovascular disease, pulmonary hypertension and renal disease associated with SCD...
2018: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/29614627/sickle-cell-disease-classification-of-clinical-complications-and-approaches-to-preventive-and-therapeutic-management
#19
Samir K Ballas
Sickle cell disease (SCD) is an inherited disorder of hemoglobin structure that has no established cure in adult patients. Cure has been achieved in selected children with sickle cell anemia (SCA) using allogeneic bone marrow transplantation or cord blood transplantation. SCD is essentially a triumvirate of (1) pain syndromes, (2) anemia and its sequelae and (3) organ failure, including infection. Pain, however, is the hallmark of SCD and dominates its clinical picture throughout the life of the patients. The prevalence of these complications varies with age from infancy through adult life...
2018: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/29609623/cross-sectional-study-on-prevalence-of-sickle-cell-alleles-s-and-c-among-patients-with-mild-malaria-in-ivory-coast
#20
Stephane Koui Tossea, Eric Gbessi Adji, Baba Coulibaly, Berenger Ako Ako, David Ngolo Coulibaly, Philippe Joly, Serge-Brice Assi, Andre Toure, Ronan Jambou
OBJECTIVES: Sickle cell anemia is due to a mutations on the betaglobin gene, inducing abnormal hemoglobin. In West Africa the main mutations lead to S or C types of hemoglobin. Patients with homozygote mutations seem protected against severe malaria, but not against mild disease. The prevalence of abnormal hemoglobin among patients attending dispensaries for mild malaria is thus unknown. A retrospective study was conducted to update data on the prevalence of S and C hemoglobin among patients attending dispensaries with mild malaria...
April 2, 2018: BMC Research Notes
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