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sickle cell disease managment

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https://www.readbyqxmd.com/read/28440126/transcranial-doppler-screening-for-stroke-risk-in-children-with-sickle-cell-disease-a-systematic-review
#1
Sara Mazzucco, Marina Diomedi, Amrana Qureshi, Laura Sainati, Soundrie T Padayachee
Background Sickle cell disease (SCD) is one of the most common causes of stroke in children worldwide. Based on the results of the Stroke Prevention Trial in Sickle Cell Anemia (STOP), annual transcranial Doppler ultrasound (TCD) screening for affected children is standard practice. However, the need for TCD surveillance programs could override the accuracy of the screening, affecting the correct stratification of stroke risk and subsequent clinical management of the target population. Aims To shed light on this issue, a systematic review of the literature on TCD screening for children and adolescents with SCD was carried out (CRD42016050549), according to a list of clinically relevant questions, with a particular focus on screening practices in European countries...
January 1, 2017: International Journal of Stroke: Official Journal of the International Stroke Society
https://www.readbyqxmd.com/read/28435481/a-patient-centered-emergency-department-management-strategy-for-sickle-cell-disease-super-utilizers
#2
Grant G Simpson, Hallie R Hahn, Alex A Powel, Robert R Leverence, Linda A Morris, Lara G Thompson, Marc S Zumberg, Deepa J Borde, Joseph A Tyndall, Jonathan J Shuster, Donald M Yealy, Brandon R Allen
INTRODUCTION: A subpopulation of sickle-cell disease patients, termed super-utilizers, presents frequently to emergency departments (EDs) for vaso-occlusive events and may consume disproportionate resources without broader health benefit. To address the healthcare needs of this vulnerable patient population, we piloted a multidisciplinary intervention seeking to create and use individualized patient care plans that alter utilization through coordinated care. Our goals were to assess feasibility primarily, and to assess resource use secondarily...
April 2017: Western Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28432164/use-of-gabapentin-in-the-treatment-of-chronic-pain-in-an-adolescent-with-sickle-cell-disease
#3
Cátia R Correia, Ana Teresa Soares, Laura Azurara, Maria João Palaré
Vaso-occlusive crises are the most common manifestation of sickle cell disease (SCD) and the main cause of hospital admission in these patients. There is emerging evidence that vaso-occlusive pain has both nociceptive and neuropathic components. However, the treatment of SCD-related pain with neuropathic drugs has not yet been systematically studied, particularly in children. We describe a 14-year-old girl with SCD and multiple hospital admissions for pain management for severe acute vaso-occlusive pain episodes...
April 21, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28424766/the-need-for-hematology-nurse-education-in-low-and-middle-income-countries-a-community-case-study-in-tanzania
#4
Julie M Buser
Hematology-related diseases, such as anemia, malaria, sickle cell disease (SCD), and blood cancers, have differing rates of survival between high-income and low- and middle-income countries (LMICs). Nurses in LMICs have an unmet need for specialty training and education to address hematology and hemato-oncology disorders. A gap in the literature exists about hematology nurse education and clinical service demands in LMICs. This community case study documents a collaborative hematology and basic hemato-oncology education program to sustainably strengthen nurse capacity at a national referral hospital and university in Tanzania...
2017: Frontiers in Public Health
https://www.readbyqxmd.com/read/28417347/-serological-markers-of-viral-hepatitis-b-and-c-in-children-with-sickle-cell-disease-monitored-in-the-pediatrics-department-at-the-university-hospital-of-bobo-dioulasso-burkina-faso
#5
S A Kissou, M Koura, A Sawadogo, A-S Ouédraogo, H Traoré, E Kamboulé, W W F Zogona, B Nacro
Viral hepatitis B and C are universal public health problems. Burkina Faso is a high endemic area for hepatitis B. Patients with sickle cell disease are at risk. The aim of this study was to investigate and quantify the serological markers of viral hepatitis B and C among pediatric patients with sickle cell disease. This was a descriptive cross-sectional study, which took place from July to November 2014 at the Department of Pediatrics, University Hospital of Bobo- Dioulasso. The study included 44 SS and 26 SC sickle cell patients...
April 17, 2017: Bulletin de la Société de Pathologie Exotique
https://www.readbyqxmd.com/read/28413532/spontaneous-extradural-and-subgaleal-hematoma-a-rare-neurosurgical-crisis-of-sickle-cell-disease
#6
Sudhansu S Mishra, Satya B Senapati, Amiya K Gouda, Sanjay K Behera, Ashis Patnaik
Extradural hematoma (EDH) in absence of trauma is a rare entity with only few cases reported in literature. The various causes reported include: Vascular malformation of dura, coagulopathies, sinus infection, middle ear or orbital infection, and tumor. Occurrence of spontaneous EDH as a complication of sickle cell disease is even much rarer. We report a case with sickle cell disease who presented with spontaneous extradural and subgaleal hematomas following an episode of vaso-oclusive crisis. He was managed successfully with surgery...
January 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/28405543/imaging-of-renal-medullary-carcinoma
#7
REVIEW
Federico Greco, Eliodoro Faiella, Domiziana Santucci, Carlo Augusto Mallio, Marco Nezzo, Carlo Cosimo Quattrocchi, Bruno Beomonte Zobel, Rosario Francesco Grasso
Renal medullary carcinoma (RMC) is a rare, highly aggressive tumor recognized as an independent pathological entity. African-descent adolescents and young adults with sickle cell hemoglobinopathy are the most affected groups. This rare subtype of renal cell carcinoma has its own morphogenetic and pathological characteristics. The major clinical manifestations include gross hematuria, abdominal or flank pain, and weight loss. The prognosis is very poor, with 95% of cases diagnosed at an advanced stage of the disease...
2017: Journal of Kidney Cancer and VHL
https://www.readbyqxmd.com/read/28390266/relationship-between-serum-25-hydroxyvitamin-d-and-inflammatory-cytokines-in-paediatric-sickle-cell-disease
#8
Samuel Ademola Adegoke, Olufemi Samuel Smith, Adekunle D Adekile, Maria Stella Figueiredo
BACKGROUND: Alteration in the concentration of inflammatory cytokines may contribute to pathogenesis in sickle cell anaemia (SCA). Vitamin D may suppress pro-inflammatory cytokines and enhance anti-inflammatory cytokines. OBJECTIVE: To compare steady state levels of pro-and anti-inflammatory cytokines of Nigerian SCA children with age- and sex-matched healthy controls, and determine the relationship with 25-hydroxyvitamin-D (25-OHD). Effects of three months of vitamin D supplementation on cytokines of SCA children with suboptimal 25-OHD were also evaluated...
April 5, 2017: Cytokine
https://www.readbyqxmd.com/read/28375958/evidence-based-practice-standard-care-for-acute-pain-management-in-adults-with-sickle-cell-disease-in-an-urgent-care-center
#9
Sunghee Kim, Ron Brathwaite, Ook Kim
BACKGROUND: Vaso-occlusive episodes (VOEs) with sickle cell disease (SCD) require opioid treatment. Despite evidence to support rapid pain management within 30 minutes, care for these patients does not consistently meet this benchmark. This quality improvement study sought to decrease the first analgesic administration time, increase patient satisfaction, and expedite patient flow. METHODS: A prospective pre-/postevaluation design was used to evaluate outcomes with patients 18 years or older with VOEs in an urgent care (UC) center after implementation of evidence-based practice standard care (EBPSC)...
April 2017: Quality Management in Health Care
https://www.readbyqxmd.com/read/28371498/chinese-guideline-for-the-diagnosis-and-treatment-of-osteonecrosis-of-the-femoral-head-in-adults
#10
(no author information available yet)
The treatment of adult osteonecrosis of the femoral head (ONFH), with 8.12 million patients in China, remains a challenge to surgeons. To standardize and improve the efficacy of the treatment of ONFH, Chinese specialists updated the experts' suggestions in March 2015, and an experts' consensus was given to provide a current basis for the diagnosis, treatment and evaluation of ONFH. The current guideline provides recommendations for ONFH with respect to epidemiology, etiology, diagnostic criteria, differential diagnosis, staging, treatment, as well as rehabilitation...
February 2017: Orthopaedic Surgery
https://www.readbyqxmd.com/read/28353009/acute-kidney-injury-in-children-with-sickle-cell-disease-compounding-a-chronic-problem
#11
EDITORIAL
Cherry Mammen, Mei Lin Bissonnette, Douglas G Matsell
In an article recently published in Pediatric Nephrology, Baddam and colleagues discuss the relatively underreported clinical problem of repeated episodes of acute kidney injury (AKI) in children with sickle cell disease (SCD). Their report is a cautionary note about the importance of repeated kidney injury on the background of underlying chronic kidney injury and its potential implications on long-term kidney outcome. In children and adults with SCD, this includes the effects of repeated vaso-occlusive crises and the management of these painful episodes with non-steroidal anti-inflammatory drugs...
March 28, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28341428/renal-papillary-necrosis-in-patients-with-sickle-cell-disease-how-to-recognize-this-forgotten-diagnosis
#12
REVIEW
Michaël M E L Henderickx, Tim Brits, Karen De Baets, Mattias Seghers, Philip Maes, Dominique Trouet, Stefan De Wachter, Gunter De Win
INTRODUCTION: Renal papillary necrosis is not commonly seen in daily practice, but can have severe consequences when it is not diagnosed in time. It is known to be associated with sickle cell hemoglobinopathies; however a wide range of etiologies are possible, and it is therefore not the first diagnosis clinicians consider in patients with sickle cell disease who present with hematuria. METHODS: A literature search was performed to summarize the current knowledge about renal papillary necrosis associated with sickle cell disease...
March 7, 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28334590/a-prospective-emergency-department-quality-improvement-project-to-improve-the-treatment-of-vaso-occlusive-crisis-in-sickle-cell-disease-lessons-learned
#13
Paula Tanabe, Caroline E Freiermuth, David M Cline, Susan Silva
BACKGROUND: Guidelines recommend rapid, aggressive management of vaso-occlusive crisis (VOC) for patients with sickle cell disease (SCD). A large prospective research and quality improvement (QI) project was conducted to measure changes in clinical outcomes in two EDs-academic medical centers with emergency medicine residency programs and Level 1 trauma centers-during a 2.5-year time period (October 2011-March 2014). METHODS: A QI team used a Plan-Do-Study-Act approach to modify and implement changes to opioid analgesic protocols for the emergency department (ED) treatment of VOC...
March 2017: Joint Commission Journal on Quality and Patient Safety
https://www.readbyqxmd.com/read/28331267/laparoscopic-management-of-a-proximal-jejunal-gallstone-ileus-with-patulous-ampulla-and-choledochal-cyst-a-report-of-unusual-presentation-and-a-review
#14
REVIEW
Rajvilas Anil Narkhede, Vijaykumar C Bada, Lakshmi Kumari Kona
Gallstone ileus is a diagnosis of rarity, and a proximal site of obstruction in a young patient is even rare. Of the three cases in our experience, we found two cases of gallstone ileus (GSI) with typical epidemiology and presentation, one had combination of multiple rare associations. We report such a case, suspected to have gallstone ileus on ultrasound and confirmed diagnosis on computed tomography. Presence of biliary-enteric fistula, old age, and obstructive features, as in typical cases, was a bigger asset for diagnosis, but it was difficult to entertain diagnosis of GSI in young girl in absence of a demonstrable biliary-enteric fistula, with uncommon association of choledochal cyst and sickle cell disease...
February 2017: Indian Journal of Surgery
https://www.readbyqxmd.com/read/28323673/clinical-pearls-of-maternal-critical-care-part-2-sickle-cell-disease-in-pregnancy
#15
Vinod Patil, Gamunu Ratnayake, Galina Fastovets
PURPOSE OF REVIEW: The current review outlines the challenges in managing pregnant women with sickle-cell anemia, who are at risk of becoming critically ill during pregnancy. RECENT FINDINGS: Sickle obstetric patients pose unique challenges to the anesthetist and intensivist. We discuss the role of prophylactic transfusions for specific indications like acute anemia and twin pregnancies. The management and prevention of vaso-occlusive crises and chest crisis are also outlined...
March 18, 2017: Current Opinion in Anaesthesiology
https://www.readbyqxmd.com/read/28295224/sickle-cell-disease-in-africa-an-overview-of-the-integrated-approach-to-health-research-education-and-advocacy-in-tanzania-2004-2016
#16
REVIEW
Furahini Tluway, Julie Makani
Sickle cell disease (SCD) is the single most important genetic cause of childhood mortality globally. Tanzania has one of the highest annual births of SCD individuals in the world, estimated to reach 11 000 births a year. Without intervention, 50-90% of children will die in childhood. However, cost-effective interventions have the potential to reduce childhood mortality by up to 70%. The effects of SCD are multi-dimensional, ranging from causing high morbidity and mortality, and reducing the quality of life, to imposing a high socio-economic burden on individuals, families and health systems...
March 14, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28295188/how-we-manage-iron-overload-in-sickle-cell-patients
#17
REVIEW
Thomas D Coates, John C Wood
Blood transfusion plays a prominent role in the management of patients with sickle cell disease (SCD), but causes significant iron overload. As transfusions are used to treat the severe complications of SCD, it remains difficult to distinguish whether organ damage is a consequence of iron overload or is due to the complications treated by transfusion. Better management has resulted in increased survival, but prolonged exposure to iron puts SCD patients at greater risk for iron-related complications that should be treated...
March 14, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28281830/imaging-of-musculoskeletal-manifestations-in-sickle-cell-disease-patients
#18
REVIEW
Vijaya Kosaraju, Alok Harwani, Sasan Partovi, Nicholas Bhojwani, Vasant Garg, Sabarish Ayyappan, Christos Kosmas, Mark Robbin
Sickle cell disease (SCD) is a hereditary red cell disorder with clinical manifestations secondary to sickling or crescent-shaped distortion of the red blood cells. Major clinical manifestations of SCD include haemolytic anaemia and vaso-occlusive phenomena resulting in ischaemic tissue injury and organ damage. Chronic sequelae of the anaemia and vaso-occlusive processes involving the musculoskeletal system include complications related to extramedullary haematopoiesis, osteonecrosis, myonecrosis and osteomyelitis...
May 2017: British Journal of Radiology
https://www.readbyqxmd.com/read/28279592/older-red-cell-units-are-associated-with-an-increased-incidence-of-infection-in-chronically-transfused-adults-with-sickle-cell-disease
#19
Matthew S Karafin, Erica Carpenter, Amy Pan, Pippa Simpson, Joshua J Field
BACKGROUND: In adults with sickle cell disease (SCD), the effects of the red cell storage lesion are not well defined. The objectives of this study were to: (1) describe the distribution of storage ages provided to adults with SCD, and (2) evaluate clinical outcomes associated with storage age. PATIENTS AND METHODS: We performed a retrospective cohort study of adults with SCD managed with prophylactic simple transfusion regimens. Units were universally pre-storage leukocyte reduced and CEK-matched...
February 9, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28276629/improved-fetal-hemoglobin-with-mtor-inhibitor-based-immunosuppression-in-a-kidney-transplant-recipient-with-sickle-cell-disease
#20
N Gaudre, P Cougoul, P Bartolucci, G Dörr, A Bura-Riviere, N Kamar, A Del Bello
Fetal hemoglobin induction is a key point in the management of sickle cell disease (SCD). We report the case of a kidney transplant recipient with SCD who was treated with everolimus, a mammalian target of rapamycin inhibitor. At 10 months after initiating therapy, the patient's fetal hemoglobin level was dramatically increased (from 4.8% to 15%) and there was excellent tolerance to treatment.
March 9, 2017: American Journal of Transplantation
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