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sickle cell disease managment

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https://www.readbyqxmd.com/read/28699644/roles-of-apol1%C3%A2-g1-and-g2-variants-in-sickle-cell-disease-patients-kidney-is-the-main-target
#1
Raphaël Kormann, Anne-Sophie Jannot, Céline Narjoz, Jean-Antoine Ribeil, Sandra Manceau, Marianne Delville, Valentin Joste, Dominique Prié, Jacques Pouchot, Eric Thervet, Marie Courbebaisse, Jean-Benoît Arlet
In African-American patients with sickle cell disease (SCD), APOL1 G1 and G2 variants are associated with increased risk of sickle cell nephropathy (SCN). To determine the role of APOL1 variants in SCD patients living in Europe, we genotyped 152 SCD patients [aged 30·4 (24·3-36·4) years], mainly of Sub-Saharan African ancestry, for APOL1 G1 and G2 and for variants of four genes with kidney tropism (GSTM1, GSTT1, GSTP1, and HMOX1). Homozygous or double-heterozygous APOL G1 and G2 genotypes were strongly associated with end stage renal disease (P = 0·003) and worse Kidney Disease: Improving Global Outcomes stages (P = 0·001)...
July 12, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28698780/liver-iron-content-lic-in-adults-with-sickle-cell-disease-scd-correlation-with-serum-ferritin-and-liver-enzymes-concentrations-in-trasfusion-dependent-td-scd-and-non-transfusion-dependent-nt-scd-patients
#2
Mohamed Yassin, Ashraf Soliman, Vincenzo De Sanctis, Abdelqadir Nashwan, Sandra Abusamaan, Abbas Moustafa, Samah Kohla, Dina Soliman
INTRODUCTION: Sickle cell disease (SCD) is one of the leading causes of morbidity and mortality worldwide, causing damage and dysfunction in multiple organs. The complications of this disease are numerous, affect every organ and/or tissue in the body and vary considerably among patients over the time challenging its management. THE AIM OF OUR STUDY: To determine the iron status of 17 patients with non-transfusion-dependent sickle cell disease ( NT-SCD) patients and six patients with transfusion dependent sickle cell disease (TD- SCD) using both serum ferritin level (SF) and Ferriscan® evaluation of liver iron content (LIC)...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/28698351/subdissociative-intranasal-ketamine-plus-standard-pain-therapy-versus-standard-pain-therapy-in-the-treatment-of-paediatric-sickle-cell-disease-vaso-occlusive-crises-in-resource-limited-settings-study-protocol-for-a-randomised-controlled-trial
#3
James R Young, Hendry Robert Sawe, Juma A Mfinanga, Ernest Nshom, Ethan Helm, Charity G Moore, Michael S Runyon, Stacy L Reynolds
INTRODUCTION: Pediatric sickle cell disease, highly prevalent in sub-Saharan Africa, carries great morbidity and mortality risk. Limited resources and monitoring make management of acute vaso-occlusive crises challenging. This study aims to evaluate the efficacy and safety of subdissociative intranasal ketamine as a cheap, readily available and easily administered adjunct to standard pain therapy. We hypothesise that subdissociative, intranasal ketamine may significantly augment current approaches to pain management in resource-limited settings in a safe and cost-effective manner...
July 10, 2017: BMJ Open
https://www.readbyqxmd.com/read/28697319/renal-medullary-carcinoma-establishing-standards-in-practice
#4
Kathryn E Beckermann, Deva Sharma, Shruti Chaturvedi, Pavlos Msaouel, Miguel R Abboud, Yves Allory, Franck Bourdeaut, Julien Calderaro, Aguirre A de Cubas, Vimal K Derebail, Andrew L Hong, Rakhi P Naik, Gabriel G Malouf, Elizabeth A Mullen, Victor E Reuter, Charles W M Roberts, Cheryl L Walker, Christopher G Wood, Michael R DeBaun, Hendrik Van Poppel, Nizar M Tannir, W Kimryn Rathmell
Although renal medullary carcinoma (RMC) is a rare subtype of kidney cancer, it is particularly devastating in that it is nearly uniformly lethal. No established guidelines exist for the diagnosis and management of RMC. In April 2016, a panel of experts developed clinical guidelines on the basis of a literature review and consensus statements. The goal was to propose recommendations for standardized diagnostic and management approaches and to establish an international clinical registry and biorepository for RMC...
July 2017: Journal of Oncology Practice
https://www.readbyqxmd.com/read/28697028/sciatic-popliteal-fossa-catheter-for-pediatric-pain-management-of-sickle-cell-crisis-a-case-report
#5
Garret Weber, Sherry Liao, Micah Alexander Burns
Sickle cell crisis, or vaso-occlusive crisis (VOC), is a major cause of hospitalizations for adults and children with sickle cell disease, and is associated with increased morbidity and mortality. Despite prompt pharmacological treatment and multimodal pain management, acute pain during a VOC is often not adequately controlled in the pediatric population. We placed a continuous popliteal sciatic nerve block under ultrasound guidance in a pediatric patient for localized refractory pain during a VOC, resulting in improved pain control with preserved sensorimotor function...
July 10, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28687959/stem-cell-therapy-for-fanconi-anemia
#6
Qing-Shuo Zhang
Stem cell therapy is the administration of stem cells to a patient to treat or prevent a disease. Since stem cells possess the long-term self-renewal capacity and provide daughter cells that differentiate into the specialized cells of each tissue, stem cell therapy will theoretically improve the disease condition for the lifetime of the patient. As the most widely used stem cell therapy, bone marrow transplantation is the treatment of choice for many kinds of blood disorders, including anemias, leukemias, lymphomas, and rare immunodeficiency diseases...
July 8, 2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28686127/effectiveness-of-surgical-revascularization-for-stroke-prevention-in-pediatric-patients-with-sickle-cell-disease-and-moyamoya-syndrome
#7
Wuyang Yang, Risheng Xu, Jose L Porras, Clifford M Takemoto, Syed Khalid, Tomas Garzon-Muvdi, Justin M Caplan, Geoffrey P Colby, Alexander L Coon, Rafael J Tamargo, Judy Huang, Edward S Ahn
OBJECTIVE Sickle cell disease (SCD) in combination with moyamoya syndrome (MMS) represents a rare complication of SCD, with potentially devastating neurological outcomes. The effectiveness of surgical revascularization in this patient population is currently unclear. The authors' aim was to determine the effectiveness of surgical intervention in their series of SCD-MMS patients by comparing stroke recurrence in those undergoing revascularization and those undergoing conservative transfusion therapy. METHODS The authors performed a retrospective chart review of patients with MMS who were seen at the Johns Hopkins Medical Institution between 1990 and 2013...
July 7, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28686043/the-role-of-nutrition-in-the-pathophysiology-and-management-of-sickle-cell-disease-among-children-a-review-of-literature
#8
Agartha Ohemeng, Isaac Boadu
Sickle cell disease (SCD) is one of the common inherited blood disorders in humans and has been associated with decreased dietary intake which results in poor nutritional status and impaired growth. Nutrition is one of the most important but often forgotten aspect of care of patients with chronic disorders and there have been emerging concern in literature on increased nutritional needs of SCD patients. This paper sought to review the available literature on the roles of individual nutrients in the pathophysiology and management of SCD among children...
July 7, 2017: Critical Reviews in Food Science and Nutrition
https://www.readbyqxmd.com/read/28673501/management-of-iron-overload-in-hemoglobinopathies
#9
S Allali, M de Montalembert, V Brousse, M Chalumeau, Z Karim
Hemoglobinopathies, thalassemia and sickle cell disease are among the most frequent monogenic diseases in the world. Transfusion has improved dramatically their prognosis, but provokes iron overload, which induces multiple organ damages. Iron overload is related to accumulation of iron released from hemolysis and transfused red cell, but also, in thalassemic patients, secondary to ineffective erythropoiesis, which increases intestinal iron absorption via decreased hepcidin production. Transfusion-related cardiac iron overload remains a main cause of death in thalassemia in well-resourced countries, and is responsible for severe hepatic damages in sickle cell disease...
June 30, 2017: Transfusion Clinique et Biologique: Journal de la Société Française de Transfusion Sanguine
https://www.readbyqxmd.com/read/28672087/interventions-for-chronic-kidney-disease-in-people-with-sickle-cell-disease
#10
REVIEW
Noemi Ba Roy, Patricia M Fortin, Katherine R Bull, Carolyn Doree, Marialena Trivella, Sally Hopewell, Lise J Estcourt
BACKGROUND: Sickle cell disease (SCD) is one of the commonest severe monogenic disorders in the world, due to the inheritance of two abnormal haemoglobin (beta-globin) genes. SCD can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Kidney disease is a frequent and potentially severe complication in people with SCD.Chronic kidney disease is defined as abnormalities of kidney structure or function, present for more than three months. Sickle cell nephropathy refers to the spectrum of kidney complications in SCD...
July 3, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28669521/management-of-delayed-hemolytic-transfusion-reaction-in-sickle-cell-disease-prevention-diagnosis-treatment
#11
F Pirenne, P Bartolucci, A Habibi
Transfusion remains a key treatment of sickle cell disease complications. However, delayed hemolytic transfusion reaction, the most serious complication of transfusion, may be life-threatening if hyperhemolysis develops. This syndrome is generally underdiagnosed because its biological and clinical features resemble those of vaso-occlusive crisis, and red blood cell antibodies are frequently absent. Further transfusions may aggravate the symptoms, leading to severe multiple organ failure and death. It is therefore essential to prevent, diagnose and treat this syndrome efficiently...
June 29, 2017: Transfusion Clinique et Biologique: Journal de la Société Française de Transfusion Sanguine
https://www.readbyqxmd.com/read/28662607/integrating-fat-embolism-syndrome-scoring-indices-in-sickle-cell-disease-a-practice-management-review
#12
Keneisha Bailey, Jagila Wesley, Adebayo Adeyinka, Louisdon Pierre
Fat embolism syndrome (FES) has been described in the literature as a rare complication of sickle cell disease (SCD). A review article published in 2005 reported 24 cases of FES associated with SCD. In many cases, a definitive diagnosis of FES in SCD is made on autopsy because of the lack of early recognition and the paucity of sensitive and specific testing for this syndrome. Patients with FES usually have a fulminant, rapidly deteriorating clinical course with mortality occurring within the first 24 hours...
January 1, 2017: Journal of Intensive Care Medicine
https://www.readbyqxmd.com/read/28648733/-is-transplantation-an-alternative-to-the-transfusional-impasse-in-sickle-cell-disease
#13
F Bernaudin, M Kuentz
Sickle cell disease is the most frequent genetic disease in France, concerning 400 newborns each year. The management of these Afro-Caribbean patients requires frequent transfusions from Caucasian donors. Due to important erythroid antigenic differences between Caucasian and African, the prevalence of allo-immunization is high in this population with a risk of transfusional impasse. Allogeneic stem cell transplantation is the only curative treatment for this disease and the replacement of red cells and lymphocytes of the sickle cell patient by those of the donor can also resolve the transfusional impasse...
June 22, 2017: Transfusion Clinique et Biologique: Journal de la Société Française de Transfusion Sanguine
https://www.readbyqxmd.com/read/28643377/randomized-feasibility-trial-to-improve-hydroxyurea-adherence-in-youth-ages-10-18-years-through-community-health-workers-the-habit-study
#14
Nancy S Green, Deepa Manwani, Sergio Matos, April Hicks, Luisa Soto, Yina Castillo, Karen Ireland, Yvonne Stennett, Sally Findley, Haomiao Jia, Arlene Smaldone
INTRODUCTION: The main therapeutic intervention for sickle cell disease (SCD) is hydroxyurea (HU). The effect of HU is largely through dose-dependent induction of fetal hemoglobin (HbF). Poor HU adherence is common among adolescents. METHODS: Our 6-month, two-site pilot intervention trial, "HABIT," was led by culturally aligned community health workers (CHWs). CHWs performed support primarily through home visits, augmented by tailored text message reminders. Dyads of youth with SCD ages 10-18 years and a parent were enrolled...
June 23, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28616936/encephaloduroateriosynangiosis-edas-in-the-management-of-moyamoya-syndrome-in-children-with-sickle-cell-disease
#15
Alexander Alamri, Pennylouise Hever, Jebet Cheserem, Catia Gradil, Sanj Bassi, Christos M Tolias
BACKGROUND: Encephalo-duro-arterio-synangiosis (EDAS) in Moyamoya syndrome (MMS) treatment has been well described in the literature, however in MMS caused by sickle cell anaemia (SCA), EDAS use remains controversial with poor long-term follow-up. We present a case-series of SCA patients who have undergone EDAS for SCA-related MMS and describe their post-operative course as well as provide a literature review of the role of EDAS in the treatment of sickle cell anaemia. METHODS: A retrospective review of all the paediatric EDAS procedures conducted in our institution for SCA from 2007 to 2015...
June 15, 2017: British Journal of Neurosurgery
https://www.readbyqxmd.com/read/28584527/efficacy-and-safety-of-manual-partial-red-cell-exchange-in-the-management-of-severe-complications-of-sickle-cell-disease-in-a-developing-country
#16
B F Faye, D Sow, M Seck, N Dieng, S A Toure, M Gadji, A B Senghor, Y B Gueye, D Sy, A Sall, T N Dieye, A O Toure, S Diop
INTRODUCTION: The realization of red cell exchange (RCE) in Africa faces the lack of blood, transfusion safety, and equipment. We evaluated its efficacy and safety in severe complications of sickle cell disease. PATIENTS AND METHOD: Manual partial RCE was performed among sickle cell patients who had severe complications. Efficacy was evaluated by clinical evolution, blood count, and electrophoresis of hemoglobin. Safety was evaluated on adverse effects, infections, and alloimmunization...
2017: Advances in Hematology
https://www.readbyqxmd.com/read/28584506/prognostic-factors-and-outcome-of-management-of-ischemic-priapism-in-zaria-nigeria
#17
Muhammed Ahmed, Benjamin Augustine, Messi Matthew, Mudi Awaisu, Abdullahi Sudi, Kolapo B Hamza, Ahmad Bello, Hussaini Yusuf Maitama
OBJECTIVES: The objective of this study was to determine the nuances of management, prognostic factors, and outcome of ischemic priapism in patients seen at Ahmadu Bello University Teaching Hospital, Zaria, Nigeria. PATIENTS AND METHODS: We retrospectively studied the case notes of all patients managed for ischemic priapism in the Ahmadu Bello University Teaching Hospital, Zaria, over a period of 10 years (2006-2015). The data extracted included patients' age, occupation, duration of painful penile erection, and previous episodes...
January 2017: Nigerian Journal of Surgery: Official Publication of the Nigerian Surgical Research Society
https://www.readbyqxmd.com/read/28581814/hybrid-statistical-and-mechanistic-mathematical-model-guides-mobile-health-intervention-for-chronic-pain
#18
Sara M Clifton, Chaeryon Kang, Jingyi Jessica Li, Qi Long, Nirmish Shah, Daniel M Abrams
Nearly a quarter of visits to the emergency department are for conditions that could have been managed via outpatient treatment; improvements that allow patients to quickly recognize and receive appropriate treatment are crucial. The growing popularity of mobile technology creates new opportunities for real-time adaptive medical intervention, and the simultaneous growth of "big data" sources allows for preparation of personalized recommendations. Here we focus on the reduction of chronic suffering in the sickle cell disease (SCD) community...
July 2017: Journal of Computational Biology: a Journal of Computational Molecular Cell Biology
https://www.readbyqxmd.com/read/28579850/coexisting-sickle-cell-anemia-and-sarcoidosis-a-management-conundrum
#19
Fnu Nutan, Nagesh S Gollahalli
Sickle cell disease and Sarcoidosis are conditions that are more common in the African American population. In this report we share an unfortunate patient who had hepatic sarcoidosis but could not receive steroids since that precipitated acute liver failure. We have discussed potential therapy options but we need more options that improve mortality.
2017: Clinical Medicine Insights. Blood Disorders
https://www.readbyqxmd.com/read/28556448/non-typhoidal-salmonella-infections-in-children-review-of-literature-and-recommendations-for-management
#20
REVIEW
Sophie Ch Wen, Emma Best, Clare Nourse
Non-typhoidal Salmonellae are a major cause of infectious diarrhoea worldwide and can cause invasive diseases, including bacteraemia, meningitis and osteomyelitis. Young or immunocompromised children and those with underlying conditions such as sickle cell disease are particularly vulnerable to invasive disease. There has been an increase in the rate of resistant non-typhoidal Salmonella, which is associated with invasive disease and hospitalisation. The intracellular nature of non-typhoidal Salmonella protects against extracellular antibiotics and can facilitate disease relapse, particularly meningitis...
May 29, 2017: Journal of Paediatrics and Child Health
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