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sickle cell disease managment

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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
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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 sickle cell disease include hemolytic anemia and vaso-occlusive phenomena resulting in ischemic tissue injury and organ damage. Chronic sequelae of the anemia and vaso-occlusive processes involving the musculoskeletal system include complications related to extramedullary hematopoiesis, osteonecrosis, myonecrosis, and osteomyelitis...
March 10, 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
#7
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
#8
Noémie Gaudre, Pierre Cougoul, Pablo Bartolucci, Gaëlle Dörr, Alessandra Bura-Riviere, Nassim Kamar, Arnaud Del Bello
Fetal hemoglobin induction is a key-point in the management of sickle-cell disease (SCD). Herein, we report on a kidney-transplant recipient with SCD that was treated with everolimus, a mammalian target of rapamycin inhibitor. At 10 months after initiating therapy, HbF level was dramatically increased (from 4.8% to 15%) and there was excellent tolerance to treatment. This article is protected by copyright. All rights reserved.
March 9, 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/28249579/assessing-the-impact-of-differences-in-malaria-transmission-intensity-on-clinical-and-haematological-indices-in-children-with-malaria
#9
Henrietta E Mensah-Brown, James Abugri, Kwaku P Asante, Duah Dwomoh, David Dosoo, Frank Atuguba, David J Conway, Gordon A Awandare
BACKGROUND: Malaria control interventions have led to a decline in transmission intensity in many endemic areas, and resulted in elimination in some areas. This decline, however, will lead to delayed acquisition of protective immunity and thus impact disease manifestation and outcomes. Therefore, the variation in clinical and haematological parameters in children with malaria was assessed across three areas in Ghana with varying transmission intensities. METHODS: A total of 568 children between the ages of 2 and 14 years with confirmed malaria were recruited in hospitals in three areas with varying transmission intensities (Kintampo > Navrongo > Accra) and a comprehensive analysis of parasitological, clinical, haematological and socio-economic parameters was performed...
March 1, 2017: Malaria Journal
https://www.readbyqxmd.com/read/28214498/vasculitic-and-autoimmune-wounds
#10
REVIEW
Victoria K Shanmugam, Divya Angra, Hamza Rahimi, Sean McNish
OBJECTIVE: Chronic wounds are a major cause of morbidity and mortality. Approximately 20% to 23% of nonhealing wounds that are refractory to vascular intervention have other causes, including vasculitis, pyoderma gangrenosum, and other autoimmune diseases. The purpose of this article was to review the literature across medical and surgical specialties with regard to refractory chronic wounds associated with vasculitis and autoimmune diseases and to delineate clinical outcomes of these wounds in response to vascular and other interventions...
March 2017: Journal of Vascular Surgery. Venous and Lymphatic Disorders
https://www.readbyqxmd.com/read/28210360/management-of-sickle-cell-disease-super-utilizers
#11
EDITORIAL
Gary A Johnson
No abstract text is available yet for this article.
February 2017: Western Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28192225/a-randomized-trial-of-yoga-for-children-hospitalized-with-sickle-cell-vaso-occlusive-crisis
#12
Karen Moody, Bess Abrahams, Rebecca Baker, Ruth Santizo, Deepa Manwani, Veronica Carullo, Doris Eugenio, Aaron Carroll
CONTEXT: Sickle cell disease (SCD) vaso-occlusive crisis (VOC) remains an important cause of acute pain in pediatrics and the most common SCD complication. Pain management recommendations in SCD include nonpharmacological interventions. Yoga is one nonpharmacological intervention that has been shown to reduce pain in some populations, however, evidence is lacking in children with VOC. OBJECTIVES: The primary objective of this study was to compare the effect of yoga versus an attention control on pain in children with VOC...
February 9, 2017: Journal of Pain and Symptom Management
https://www.readbyqxmd.com/read/28159390/sickle-cell-disease
#13
REVIEW
Russell E Ware, Mariane de Montalembert, Léon Tshilolo, Miguel R Abboud
Sickle cell disease is a common and life-threatening haematological disorder that affects millions of people worldwide. Abnormal sickle-shaped erythrocytes disrupt blood flow in small vessels, and this vaso-occlusion leads to distal tissue ischaemia and inflammation, with symptoms defining the acute painful sickle-cell crisis. Repeated sickling and ongoing haemolytic anaemia, even when subclinical, lead to parenchymal injury and chronic organ damage, causing substantial morbidity and early mortality. Currently available treatments are limited to transfusions and hydroxycarbamide, although stem cell transplantation might be a potentially curative therapy...
January 31, 2017: Lancet
https://www.readbyqxmd.com/read/28154185/heterochromatin-protein-1%C3%AE-is-a-novel-epigenetic-repressor-of-human-embryonic-%C3%AF%C2%B5-globin-gene-expression
#14
Yadong Wang, Ying Wang, Lingling Ma, Min Nie, Junyi Ju, Ming Liu, Yexuan Deng, Bing Yao, Tao Gui, Xinyu Li, Chan Guo, Chi Ma, Renxiang Tan, Quan Zhao
Production of hemoglobin during development is tightly regulated. For example, expression from the human β-globin gene locus, comprising β-, δ-, ϵ-, and γ-globin genes, switches from ϵ-globin to γ-globin during embryonic development and then from γ-globin to β-globin after birth. Expression of human ϵ-globin in mice has been shown to ameliorate anemia caused by β-globin mutations, including those causing β-thalassemia and sickle cell disease (SCD), raising the prospect that reactivation of ϵ-globin expression could be used in managing these conditions in humans...
February 1, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28143586/socio-demographic-characteristics-and-psychosocial-consequences-of-sickle-cell-disease-the-case-of-patients-in-a-public-hospital-in-ghana
#15
Vincent A Adzika, Franklin N Glozah, Desmond Ayim-Aboagye, Collins S K Ahorlu
BACKGROUND: Sickle cell disease (SCD) is of major public health concern globally, with majority of patients living in Africa. Despite its relevance, there is a dearth of research to determine the socio-demographic distribution and psychosocial impact of SCD in Ghana. The objective of this study was to examine the socio-demographic distribution and psychosocial consequences of SCD among patients in Ghana and to assess their quality of life and coping mechanisms. METHODS: A cross-sectional research design was used that involved the completion of questionnaires on socio-demographic characteristics, quality of life, coping mechanisms, anxiety and depression...
January 31, 2017: Journal of Health, Population, and Nutrition
https://www.readbyqxmd.com/read/28133359/myonecrosis-in-sickle-cell-anemia-case-study
#16
Lalita Prabha Turaga, Prajwal Boddu, Steve Kipferl, Anupam Basu, Martin Yorath
BACKGROUND Myonecrosis is one of the more poorly studied, painful manifestations of sickle cell crisis. Medical literature is sparse detailing the manifestations and management of such symptoms. In myonecrosis, red cells containing sickle hemoglobin become rigid, resulting in reduced blood flow and myonecrosis. CASE REPORT We present a case study of a patient in sickle cell crisis with an episode of acute pain and swelling to the intrinsic muscles of the foot as a prominent feature of the crises. Although muscle biopsy is considered the gold standard for the diagnosis of myositis or myonecrosis, a low intensity signal on T1 and high intensity signal on T2 at the affected muscle belly can be as conclusive as imaging studies...
January 30, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28110486/spectra-optia-%C3%A2-for-automated-red-blood-cell-exchange-in-patients-with-sickle-cell-disease-a-nice-medical-technology-guidance
#17
REVIEW
Iain Willits, Helen Cole, Roseanne Jones, Kimberley Carter, Mick Arber, Michelle Jenks, Joyce Craig, Andrew Sims
The Spectra Optia(®) automated apheresis system, indicated for red blood cell exchange in people with sickle cell disease, underwent evaluation by the National Institute for Health and Care Excellence, which uses its Medical Technologies Advisory Committee to make recommendations. The company (Terumo Medical Corporation) produced a submission making a case for adoption of its technology, which was critiqued by the Newcastle and York external assessment centre. Thirty retrospective observational studies were identified in their clinical submission...
January 21, 2017: Applied Health Economics and Health Policy
https://www.readbyqxmd.com/read/28109314/sickle-cell-disease-and-pregnancy-outcomes-a-study-of-the-community-based-hospital-in-a-tribal-block-of-gujarat-india
#18
Gayatri Desai, Ankit Anand, Pankaj Shah, Shobha Shah, Kapilkumar Dave, Hardik Bhatt, Shrey Desai, Dhiren Modi
BACKGROUND: Sickle cell disease (SCD) is a hereditary blood disorder prevalent in tribal regions of India. SCD can increase complications during pregnancy and in turn negatively influence pregnancy outcomes. This study reports the analysis of tribal maternal admissions in the community-based hospital of SEWA Rural (Kasturba Maternity Hospital) in Jhagadia block, Gujarat. The objective of the study is to compare the pregnancy outcomes among SCD, sickle cell trait and non-SCD admissions...
January 21, 2017: Journal of Health, Population, and Nutrition
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
#19
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...
April 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
#20
REVIEW
Lise J Estcourt, Patricia M Fortin, Sally Hopewell, Marialena Trivella, Winfred C Wang
BACKROUND: 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
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