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sickle cell disease managment

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https://www.readbyqxmd.com/read/28616936/encephaloduroateriosynangiosis-edas-in-the-management-of-moyamoya-syndrome-in-children-with-sickle-cell-disease
#1
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
#2
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
#3
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
#4
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...
June 5, 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
#5
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
#6
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
https://www.readbyqxmd.com/read/28555355/laparoscopic-cholecystectomy-for-cholelithiasis-in-children-with-sickle-cell-disease
#7
REVIEW
Yousef Al Talhi, Bader Hamza Shirah, Muteb Altowairqi, Yasmin Yousef
Patients with sickle cell disease (SCD) suffer from an increased incidence of gallstone formation due to hemolysis of sickled red blood cells; this leads to an increased level of bilirubin in secreted bile that becomes a nidus for pigment stone formation. Laparoscopic cholecystectomy (LC) is considered a standard operative procedure for gallstone disease mainly due to lower postoperative wound complaints, faster recovery, better postoperative cosmetic results, shorter hospital stay, and earlier return to work...
May 29, 2017: Clinical Journal of Gastroenterology
https://www.readbyqxmd.com/read/28538513/attention-deficit-hyperactivity-disorder-in-children-with-sickle-cell-disease-referred-for-an-evaluation
#8
Melissa A Acquazzino, Meghan Miller, Matthew Myrvik, Robert Newby, John Paul Scott
Neuropsychological deficits, including difficulties with attention, are well described in children with sickle cell disease (SCD). Very little is known about attention deficit hyperactivity disorder (ADHD) in children with SCD. The objective of this study was to determine the proportion of ADHD in children with SCD referred for neuropsychological evaluation. This prospective, cross-sectional study included patients (age, 4 to 18 y) with SCD and completion of a neuropsychological evaluation between December 2013 and March 2016...
May 22, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28538505/thalassemia-major-and-priapism-a-case-report-of-an-adolescent
#9
Sibel Öz, Serhan Küpeli, Gülay Sezgin, İbrahim Bayram
Priapism is defined as a prolonged pathologic penile erection without sexual stimulation. In children, priapism secondary to sickle cell disease or hematological malignancy is a frequent condition. Appropriate treatment of priapism varies; the treatment is primarily etiological, conservative management. In the present report, we aimed to present a case of asplenic thalassemia major who developed priapism, improved with hydration and ibuprofen treatment. Clinicians should take into account that priapism can be encountered in patients with thalassemia major...
May 22, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28532265/encephaloduroarteriosynangiosis-edas-in-young-patients-with-cerebrovascular-complications-of-sickle-cell-disease-single-institution-experience
#10
Michael Winstead, Peter P Sun, Kenneth Martin, Janice Earl, Lynne Neumayr, Carolyn Hoppe, Elliott Vichinsky
Moyamoya syndrome occurs in sickle cell disease (SCD) as a secondary complication of large-artery stenosis. Moyamoya increases the risk of stroke, but its optimal management in SCD is not established.  Encephaloduroarteriosynangiosis (EDAS) is a neurosurgical revascularization procedure for moyamoya whose use has been reported in SCD patients. We report the outcomes of 11 patients with SCD systematically evaluated for EDAS by a multidisciplinary team and compare the rate of stroke in patients who received EDAS to those who did not...
May 22, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/28512745/implementation-of-multi-disciplinary-care-reduces-maternal-mortality-in-women-with-sickle-cell-disease-living-in-low-resource-setting
#11
Eugenia Vicky Asare, Edeghonghon Olayemi, Theodore Boafor, Yvonne Dei-Adomakoh, Enoch Mensah, Harriet Ghansah, Yvonne Osei-Bonsu, Selina Crabbe, Latif Musah, Charles Hayfron-Benjamin, Brittany Covert, Adetola A Kassim, Andra James, Mark Rodeghier, Michael R DeBaun, Samuel A Oppong
Sickle cell disease (SCD) is associated with adverse pregnancy outcome. In women with SCD living in low-resource settings, pregnancy is associated with significantly increased maternal and perinatal mortality rates. We tested the hypothesis that implementing a multidisciplinary obstetric and hematology care team in a low-resource setting would significantly reduce maternal and perinatal mortality rates. We conducted a before-and-after study, at the Korle-Bu Teaching Hospital in Accra, Ghana, to evaluate the effect of a multidisciplinary obstetric-hematology care team for women with SCD in a combined SCD-Obstetric Clinic...
May 16, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28506281/a-telephonic-mindfulness-based-intervention-for-persons-with-sickle-cell-disease-study-protocol-for-a-randomized-controlled-trial
#12
Hants Williams, Susan Silva, Leigh Ann Simmons, Paula Tanabe
BACKGROUND: One of the most difficult symptoms for persons with sickle cell disease (SCD) to manage is chronic pain. Chronic pain impacts approximately one-third of persons with SCD and is associated with increased pain intensity, pain behavior, and frequency and duration of hospital visits. A promising category of nonpharmacological interventions for managing both physical and affective components of pain are mindfulness-based interventions (MBIs). METHODS/DESIGN: The primary aim of this study is to conduct a randomized controlled study to evaluate the acceptability and feasibility, as well as to determine the preliminary efficacy, of a telephonic MBI for adults with SCD who have chronic pain...
May 15, 2017: Trials
https://www.readbyqxmd.com/read/28486096/key-pharmacogenomic-considerations-for-sickle-cell-disease-patients
#13
Alexandra Kolliopoulou, Apostolos Stratopoulos, Stavroula Siamoglou, Argyro Sgourou, Bassam R Ali, Adamantia Papachatzopoulou, Theodora Katsila, George P Patrinos
Sickle cell disease (SCD), although a monogenic disease, exhibits a complex clinical phenotype that hampers optimum patient stratification and disease management, especially on hydroxyurea treatment. Moreover, theranostics, the combination of diagnostics to individualize and optimize therapeutic interventions, has not been firmly on the forefront of SCD research and clinical management to date. We suggest that if tailor-made theranostics in SCD is envisaged, pharmacogenomics is anticipated to be the way forward...
June 2017: Omics: a Journal of Integrative Biology
https://www.readbyqxmd.com/read/28484512/developing-new-pharmacotherapeutic-approaches-to-treating-sickle-cell-disease
#14
Marilyn J Telen
Survival for patients with SCD has been prolonged by improvements in supportive care, including vaccinations, antibiotic prophylaxis, and overall medical management, including tra nsfusion. However, there remains only one approved, partially effective drug for sickle cell disease-hydroxyurea (hydroxycarbamide). The world desperately needs better ways of both treating and preventing the recurrent painful vaso-occlusive episodes pathognomonic of sickle cell disease as well as the end-organ damage that still leads inexorably to severely shortened life expectancies throughout the world...
February 2017: ISBT Science Series
https://www.readbyqxmd.com/read/28470719/from-total-blood-exchange-to-erythrocytapheresis-and-back-to-treat-complications-of-sickle-cell-disease
#15
Samir K Ballas
Erythrocytapheresis is an important procedure in the management of certain complications of sickle cell disease, including acute stroke, stroke prevention, acute chest syndrome, and multiorgan failure. Erythrocytapheresis in sickle cell disease simply entails the removal of the patient's red blood cells containing the abnormal sickle hemoglobin and replacing them with normal red blood cells carrying normal hemoglobin. In these procedures, the patient's plasma is not exchanged but is returned to the patient...
May 3, 2017: Transfusion
https://www.readbyqxmd.com/read/28464177/hospital-readmissions-from-patients-perspectives
#16
Beril Cakir, Stephanie Kaltsounis, Katherine D' Jernes, Sara Kopf, Julea Steiner
OBJECTIVES: Healthcare expenditures in the United States have increased exponentially and hospital care accounts for one-third of these costs. Approximately 18% of hospitalized Medicare beneficiaries are being readmitted to the hospital within 30 days. Engaging patients in the discharge process can help better identify patients' postdischarge needs and implement more effective readmission prevention strategies. The objective of our study was to identify the factors that contribute to hospital readmission as seen from patients' perspectives in a large urban community hospital...
May 2017: Southern Medical Journal
https://www.readbyqxmd.com/read/28460533/a-multidisciplinary-approach-to-impact-acute-care-utilization-in-sickle-cell-disease
#17
Rhea E Powell, Paris B Lovett, Albert Crawford, John McAna, David Axelrod, Lawrence Ward, Dianne Pulte
Sickle cell disease (SCD), an inherited red blood cell disorder, is characterized by anemia, end-organ damage, unpredictable episodes of pain, and early mortality. Emergency department (ED) visits and hospitalizations are frequent, leading to increased burden on patients and increased health care costs. This study assessed the effects of a multidisciplinary care team intervention on acute care utilization among adults with SCD. The multidisciplinary care team intervention included monthly team meetings and development of individualized care plans...
April 1, 2017: American Journal of Medical Quality: the Official Journal of the American College of Medical Quality
https://www.readbyqxmd.com/read/28455727/perioperative-considerations-for-patients-with-sickle-cell-disease-a-narrative-review
#18
Narjeet Khurmi, Andrew Gorlin, Lopa Misra
PURPOSE: Approximately 200,000 individuals worldwide are born annually with sickle cell disease (SCD). Regions with the highest rates of SCD include Africa, the Mediterranean, and Asia, where its prevalence is estimated to be 2-6% of the population. An estimated 70,000-100,000 people in the United States have SCD. Due to enhanced newborn screening, a better understanding of this disease, and more aggressive therapy, many sickle cell patients survive into their adult years and present more frequently for surgery...
April 28, 2017: Canadian Journal of Anaesthesia, Journal Canadien D'anesthésie
https://www.readbyqxmd.com/read/28444215/behavioral-and-pharmacological-adherence-in-pediatric-sickle-cell-disease-parent-child-agreement-and-family-factors-associated-with-adherence
#19
Page H Klitzman, Julia K Carmody, Mary H Belkin, David M Janicke
This study aimed to evaluate agreement between children and parents on a measure of behavioral and pharmacological adherence in children with sickle cell disease (SCD), and the associations among family factors (i.e., problem-solving skills, routines, communication) and adherence behaviors. In all, 85 children (aged 8-18 years) with SCD and their parents completed questionnaires assessing individual and family factors. Overall parent-child agreement on an adherence measure was poor, particularly for boys and older children...
April 21, 2017: Journal of Pediatric Psychology
https://www.readbyqxmd.com/read/28440126/transcranial-doppler-screening-for-stroke-risk-in-children-with-sickle-cell-disease-a-systematic-review
#20
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
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