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sickle cell disease managment

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https://www.readbyqxmd.com/read/28099398/utilizing-a-novel-mobile-health-selfie-application-to-improve-compliance-to-iron-chelation-in-pediatric-patients-receiving-chronic-transfusions
#1
Sarah Leonard, Lindsay M Anderson, Jude Jonassaint, Charles Jonassaint, Nirmish Shah
Iron chelation therapy can prevent iron overload for pediatric patients with sickle cell disease and β-thalassemia major; however, adherence is suboptimal. Therefore, we developed an intensive training program (ITP), to improve medication management and disease knowledge. The objectives were to determine feasibility of the ITP and its preliminary impact on adherence, disease knowledge, and health outcomes. Pediatric patients were recruited to participate in the ITP over a 90-day period and were followed for 6 months...
January 17, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28094851/blood-transfusion-for-preventing-primary-and-secondary-stroke-in-people-with-sickle-cell-disease
#2
REVIEW
Lise J Estcourt, Patricia M Fortin, Sally Hopewell, Marialena Trivella, Winfred C Wang
BACKGROUND: Sickle cell disease is one of the commonest severe monogenic disorders in the world, due to the inheritance of two abnormal haemoglobin (beta globin) genes. Sickle cell disease can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Stroke affects around 10% of children with sickle cell anaemia (HbSS). Chronic blood transfusions may reduce the risk of vaso-occlusion and stroke by diluting the proportion of sickled cells in the circulation...
January 17, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28094443/validity-of-simple-clinical-and-biological-parameters-as-screening-tool-for-sickle-cell-anemia-for-referral-to-tertiary-center-in-highly-resource-constraints
#3
Bertin Tshimanga Kadima, Jean Lambert Gini-Ehungu, Fiston Ikwa Ndol Mbutiwi, John Tunda Bahati, Michel Ntetani Aloni
BACKGROUND: In the Democratic Republic of Congo, the incidence of sickle cell anemia (SCA) is estimated around 40 000 neonates per year. However, it is notoriously difficult to perform conventional electrophoresis in all hospitals and laboratories, especially at peripheral levels and rural area. A panel of multiple clinical and laboratory features that would enhance sickle cell disease were assessed for the detection of the disease in highly resource-scarce settings. METHODS: A prospective study was conducted in Kinshasa...
January 17, 2017: Journal of Clinical Laboratory Analysis
https://www.readbyqxmd.com/read/28093814/management-of-sickle-cell-disease-in-patients-undergoing-cardiac-surgery
#4
REVIEW
Todd C Crawford, Michael V Carter, Rina K Patel, Alejandro Suarez-Pierre, Sophie Z Lin, Jonathan Trent Magruder, Joshua C Grimm, Duke E Cameron, William A Baumgartner, Kaushik Mandal
Sickle cell disease is a life-limiting inherited hemoglobinopathy that poses inherent risk for surgical complications following cardiac operations. In this review, we discuss preoperative considerations, intraoperative decision-making, and postoperative strategies to optimize the care of a patient with sickle cell disease undergoing cardiac surgery.
January 16, 2017: Journal of Cardiac Surgery
https://www.readbyqxmd.com/read/28065813/aapt-diagnostic-criteria-for-chronic-sickle-cell-disease-pain
#5
REVIEW
Carlton Dampier, Tonya M Palermo, Deepika S Darbari, Kathryn Hassell, Wally Smith, William Zempsky
: Pain in sickle cell disease (SCD) is associated with increased morbidity, mortality, and high healthcare costs. While episodic acute pain is the hallmark of this disorder, there is an increasing awareness that chronic pain is part of the pain experience of many older adolescents and adults. A common set of criteria for classifying chronic pain associated with SCD would enhance SCD pain research efforts in epidemiology, pain mechanisms, and clinical trials of pain management interventions, and ultimately improve clinical assessment and management...
January 5, 2017: Journal of Pain: Official Journal of the American Pain Society
https://www.readbyqxmd.com/read/28050373/histopathologic-findings-in-autopsies-with-emphasis-on-interesting-and-incidental-findings-a-pathologist-s-perspective
#6
Sapna Patel, B R Rajalakshmi, G V Manjunath
INTRODUCTION: Autopsy aids to the knowledge of pathology by unveiling the rare lesions which are a source of learning from a pathologist's perspective Some of them are only diagnosed at autopsy as they do not cause any functional derangement. This study emphasizes the various incidental lesions which otherwise would have been unnoticed during a person's life. AIM: The aim of this study was to determine the spectrum of histopathological findings including neoplastic lesions related or unrelated to the cause of death...
November 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28043127/potential-of-three-ethnomedicinal-plants-as-antisickling-agents
#7
Ismaila O Nurain, Clement O Bewaji, Jarrett S Johnson, Robertson D Davenport, Yang Zhang
Sickle cell disease (SCD) is a genetic blood disorder that affects the shape and transportation of red blood cells (RBCs) in blood vessels, leading to various clinical complications. Many drugs that are available for treating the disease are insufficiently effective, toxic, or too expensive. Therefore, there is a pressing need for safe, effective, and inexpensive therapeutic agents from indigenous plants used in ethnomedicines. The potential of aqueous extracts of Cajanus cajan leaf and seed, Zanthoxylum zanthoxyloides leaf, and Carica papaya leaf in sickle cell disease management was investigated in vitro using freshly prepared 2% sodium metabisulfite for sickling induction...
January 3, 2017: Molecular Pharmaceutics
https://www.readbyqxmd.com/read/28005272/prophylactic-versus-selective-blood-transfusion-for-sickle-cell-disease-in-pregnancy
#8
REVIEW
Babasola O Okusanya, Olufemi T Oladapo
BACKGROUND: Pregnant women with sickle cell disease (HbSS, HbSC and HbSβThal) may require blood transfusion to prevent severe anaemia or to manage potential medical complications. Preventive blood transfusion in the absence of complications starting from the early weeks of pregnancy or blood transfusion only for medical or obstetric indications have been used as management policies. There is currently no consensus on the blood transfusion policy that guarantees optimal clinical benefits with minimal risks for such women and their babies...
22, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28000341/heart-failure-in-haemoglobinopathies-pathophysiology-clinical-phenotypes-and%C3%A2-management
#9
REVIEW
Dimitrios Farmakis, Filippos Triposkiadis, John Lekakis, John Parissis
Hereditary haemoglobinopathies, mainly beta-thalassemia and sickle cell disease, constitute the most common monogenic disorders in humans, and although once geographically confined, they are currently globally distributed. They are demanding clinical entities that require multidisciplinary medical management. Despite their genotypic and phenotypic heterogeneity, the haemoglobinopathies share several similarities in pathophysiology, clinical manifestations, therapeutic requirements, and complications, among which heart failure (HF) represents a leading cause of mortality and morbidity...
December 20, 2016: European Journal of Heart Failure
https://www.readbyqxmd.com/read/27994488/an-unfortunate-case-of-acquired-hemochromatosis-a-case-report-review-of-the-clinical-presentation-diagnosis-management-and-prognosis
#10
Anam Tariq, Kevin Westra, Arben Santo
BACKGROUND: While blood transfusions are commonly used for prophylaxis and treatment for acute chest syndromes and strokes in sickle cell patients, accumulation of excess iron resulting in secondary hemochromatosis remains a rare disease. Chelation is the mainstay for preventing and treating iron overload to deter potential end-organ damages; it is rare when therapy fails. CASE REPORT: A 52-year-old African American woman with chronic anemia secondary to sickle cell anemia and history of multiple blood transfusions presented with elevated serum ferritin (8000 ng/mL) and bilirubin (16...
2016: International Medical Case Reports Journal
https://www.readbyqxmd.com/read/27982424/knowledge-insufficient-the-management-of-haemoglobin-sc-disease
#11
REVIEW
Lydia H Pecker, Beverly A Schaefer, Lori Luchtman-Jones
Although haemoglobin SC (HbSC) accounts for 30% of sickle cell disease (SCD) in the United States and United Kingdom, evidence-based guidelines for genotype specific management are lacking. The unique pathology of HbSC disease is complex, characterized by erythrocyte dehydration, intracellular sickling and increased blood viscosity. The evaluation and treatment of patients with HbSC is largely inferred from studies of SCD consisting mostly of haemoglobin SS (HbSS) patients. These studies are underpowered to allow definitive conclusions about HbSC...
December 16, 2016: British Journal of Haematology
https://www.readbyqxmd.com/read/27924667/contraceptive-practices-in-women-with-sickle-cell-disease
#12
Natália S Carvalho, Josefina Pellegrini Braga, Márcia Barbieri, Maria R Torloni, Maria S Figueiredo, Cristina A F Guazzelli
This was a cross-sectional study of sexually active women with sickle-cell disease (SCD) managed at São Paulo Federal University during a one-year period. A total of 54 women were included. Mean age was 32.0 (11.2, standard deviation) years and almost 95% were black or of mixed racial ancestry. Almost 80% reported a history of multiple blood transfusions, 50% had kidney disease and 17% had a history of thrombosis. Over 80% of them had used some form of contraception, mostly combined hormonal contraceptive (52%) or progestin-only contraceptives (46%)...
December 7, 2016: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/27913540/sickle-cell-disease-an-inherited-thrombophilia
#13
Ted Wun, Ann Brunson
Activation of the hemostatic system occurs in patients with sickle cell disease. The extent to which this activation contributes to sickle cell pathophysiology is uncertain. Clinical trials of anticoagulants or platelet inhibitors have demonstrated the ability to decrease biomarkers of hemostatic activation, but this has generally not resulted in improvement in clinically relevant outcomes. Venous thromboembolism (VTE: deep venous thrombosis and pulmonary embolism) has been until recently an underappreciated complication of sickle cell disease, with incident event and recurrence rates consistent with a strong thrombophilia...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913539/prevention-of-central-nervous-system-sequelae-in-sickle-cell-disease-without-evidence-from-randomized-controlled-trials-the-case-for-a-team-based-learning-collaborative
#14
Michael R DeBaun, Allison A King
Since 1998, the National Institutes of Health has funded 5 randomized controlled trials (RCTs) for primary and secondary prevention of strokes in children with sickle cell anemia (SCA). In a systematic fashion, these trials have significantly advanced the care of children with SCA. In the absence of an RCT, clinicians are often compelled to make decisions at the bedside, based on experience, observational studies, and principles of hematology. We will provide an initial example that describes how a team-based, learning collaborative developed a multisite standard care protocol with a low budget (<$10 000 per year) to overcome the intrinsic limitations of advancing the care of neurologic complications in sickle cell disease (SCD)...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913538/sickle-cell-disease-when-and-how-to-transfuse
#15
Jo Howard
Blood transfusion remains an important therapeutic intervention in patients with sickle cell disease (SCD), aiming to both increase the oxygen carrying capacity of blood and to reduce the complications of vaso-occlusion. Simple, manual exchange and automated exchange can be effective in reducing the acute and chronic complications of SCD, and the advantages and disadvantages of each methodology mean they all have a role in different situations. Evidence for the role of emergency transfusion in the management of the acute complications of SCD, including acute pain and acute chest syndrome, comes from observational data...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27880985/daily-pain-in-adults-with-sickle-cell-disease-a-different-perspective
#16
Charlotte F J van Tuijn, Joep W R Sins, Karin Fijnvandraat, Bart J Biemond
Previous reports demonstrated that patients with sickle cell disease (SCD) experience pain on more than half of the observed days. Yet, these high incidences do not seem to match observations in our population. In this prospective cohort study we aimed to assess the frequency and characteristics of daily, self-reported pain among adult SCD patients in the Netherlands. Consecutive patients were enrolled during routine outpatient visits and followed up to 6 months. A total of 55 patients completed 5 982 diary observation days...
November 23, 2016: American Journal of Hematology
https://www.readbyqxmd.com/read/27869662/the-cell-killing-mechanisms-of-hydroxyurea
#17
REVIEW
Amanpreet Singh, Yong-Jie Xu
Hydroxyurea is a well-established inhibitor of ribonucleotide reductase that has a long history of scientific interest and clinical use for the treatment of neoplastic and non-neoplastic diseases. It is currently the staple drug for the management of sickle cell anemia and chronic myeloproliferative disorders. Due to its reversible inhibitory effect on DNA replication in various organisms, hydroxyurea is also commonly used in laboratories for cell cycle synchronization or generating replication stress. However, incubation with high concentrations or prolonged treatment with low doses of hydroxyurea can result in cell death and the DNA damage generated at arrested replication forks is generally believed to be the direct cause...
November 17, 2016: Genes
https://www.readbyqxmd.com/read/27869039/the-role-of-rs1984112_g-at-cd36-gene-in-increasing-reticulocyte-level-among-sickle-cell-disease-patients
#18
Miniar Kalai, Marwa Dridi, Leila Chaouch, Imen Moumni, Houyem Ouragini, Imen Darragi, Imen Boudrigua, Dorra Chaouachi, Fethi Mellouli, Mohamed Bejaoui, Salem Abbes
AIMS AND BACKGROUND: Mediators of adhesion become a potential new target for pharmacological therapy to struggle the complications of sickle cell disease (SCD). Several mechanisms for increased adherence have been postulated and the well-studied are CD36 and VLA4 which encoded by ITGA4. Herein, we sought to determine whether one polymorphism of CD36 namely: rs1984112 and three exons of ITGA4 (4, 5, and 6) are implicated in hemolytic status and clinical events among SCD Tunisian patients...
November 20, 2016: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/27866856/free-tissue-transfer-in-patients-with-sickle-cell-disease-considerations-for-multi-disciplinary-peri-operative-management
#19
REVIEW
Lilli Cooper, Rohit Seth, Elizabeth Rhodes, Mohammed Alousi, Bran Sivakumar
INTRODUCTION AND AIMS: Sickle cell disease (SCD) is an increasingly common condition in the UK. The safety of free tissue transfer in these patients is controversial, and no specific guidelines exist. The aim of this paper is to create recommendations for the plastic surgical multidisciplinary team for use in the assessment and management of SCD patients undergoing free tissue transfer and reconstruction. METHOD: A literature review was performed in PubMed of 'sickle [TiAb] AND plast* adj3 surg*...
January 2017: Journal of Plastic, Reconstructive & Aesthetic Surgery: JPRAS
https://www.readbyqxmd.com/read/27800652/opioid-prescription-practices-at-discharge-and-30-day-returns-in-children-with-sickle-cell-disease-and-pain
#20
Leslie M Okorji, Devin S Muntz, Robert I Liem
BACKGROUND: Acute pain episodes in children with sickle cell disease (SCD) represent a leading cause of readmissions. We examined prescription practices at the time of discharge in children with SCD presenting with acute pain to determine their impact on 30-day emergency department (ED) revisits and readmissions. METHODS: In this single-institution, 5-year retrospective study, we reviewed 290 encounters of patients with SCD aged 7-21 years hospitalized or discharged from the ED with acute pain...
November 1, 2016: Pediatric Blood & Cancer
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