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sickle cell disease managment

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https://www.readbyqxmd.com/read/29323695/lead-toxicity-in-the-pediatric-patient-with-sickle-cell-disease-unique-risks-and-management
#1
Josephine Misun Jung, Radhika Peddinti
Lead toxicity is the result of lead ingestion, one of the most common ingestions in the pediatric population. Nationwide and statewide efforts to recognize and curtail this epidemic have led to declining rates of toxicity. In patients with sickle cell disease (SCD), lead toxicity can be an elusive diagnosis due to overlapping symptom profiles, and inconsistent follow-up with a primary care physician can make the diagnosis even more difficult. In this article, two illustrative cases of lead toxicity in patients with SCD are described...
January 1, 2018: Pediatric Annals
https://www.readbyqxmd.com/read/29321025/diagnostic-challenges-of-prolonged-post-treatment-clearance-of-plasmodium-nucleic-acids-in-a-pre-transplant-autosplenectomized-patient-with-sickle-cell-disease
#2
Paul M Luethy, Sean C Murphy, Annette M Seilie, Yingda L Xie, Chuen-Yen Lau, John F Tisdale, Matthew M Hsieh, Jessica L Reinhardt, Anna F Lau, Gary A Fahle
BACKGROUND: Autosplenectomy, as a result of sickle cell disease, is an important risk factor for severe malaria. While molecular methods are helpful in providing rapid and accurate infection detection and species identification, the effect of hyposplenism on result interpretation during the course of infection should be carefully considered. CASE PRESENTATION: A 32-year old autosplenectomized Nigerian male with severe sickle cell disease was referred to the National Institutes of Health for allogenic hematopoietic stem cell transplant...
January 10, 2018: Malaria Journal
https://www.readbyqxmd.com/read/29316596/van-neck-odelberg-disease-a-3-5-year-follow-up-case-report-and-systematic-review
#3
Patrick J Mixa, Frank A Segreto, Hiram Luigi-Martinez, Bassel G Diebo, Qais Naziri, Srinivas Kolla, Aditya V Maheshwari
V an Neck-Odelberg disease (VND) is a benign skeletal overgrowth of the ischiopubic synchondrosis (IPS) in prepubescent patients. There is a paucity of long-term follow-up data and reviews on management decision-making. We report on a 15-year-old female, with a history of sickle-cell disease (HbSS), presenting with unilateral groin pain. Patient's physical examination, radiographs, and a literature-review determined a diagnosis of VND. Conservative treatment was issued. Clinical symptoms resolved at three months, followed by complete lesion resolution at three years...
December 12, 2017: Surgical Technology International
https://www.readbyqxmd.com/read/29313505/effectiveness-safety-and-cost-of-partial-exchange-transfusions-in-patients-with-sickle-cell-anemia-at-a-sickle-cell-disease-center-in-sub-saharan-africa
#4
P Boma Muteb, J F J Kaluila Mamba, P Muhau Pfutila, V Bilo, J D Panda Mulefu, D A Diallo
The partial exchange transfusions necessary for management of some sickle-cell complications raise the issue of effectiveness in the context of limited resources and inadequate blood safety. This study evaluated the effectiveness, safety, and cost of partial exchange transfusions in 39 patients with sickle-cell anemia in Lubumbashi, looking at the patients' age and gender and the tolerability and direct cost of the transfusions. Excel and SPSS 18 were used for data entry and analysis. Chi2 and Fisher exact tests were used for comparisons...
November 1, 2017: Médecine et Santé Tropicales
https://www.readbyqxmd.com/read/29313430/the-assessment-and-sustainable-management-of-sickle-cell-disease-in-the-indigenous-tharu-population-of-nepal
#5
Miles Marchand, Carson Gill, Armaan K Malhotra, Carolyn Bell, Eric Busto, Monica D McKeown, Abhiram Cherukupalli, Jordan Yeo, Brendan Arnold, Videsh Kapoor
Sickle cell disease is an inherited hemoglobinopathy associated with significant morbidity and mortality. Reports suggest a high sickle cell disease burden among the indigenous Tharu population of Nepal, who for centuries have inhabited regions where malaria is endemic. Unfortunately, health care resources are limited and often inaccessible for Tharu individuals suffering from sickle cell disease. We conducted a large-scale screening effort to estimate the prevalence of Hb S (HBB: c.20A>T) among the Tharu population and delivered community-based education sessions to increase sickle cell disease awareness...
July 2017: Hemoglobin
https://www.readbyqxmd.com/read/29310823/systemic-and-oral-health-considerations-in-managing-sickle-cell-disease-patients
#6
EDITORIAL
Jerrold B Leikin
No abstract text is available yet for this article.
January 5, 2018: Disease-a-month: DM
https://www.readbyqxmd.com/read/29300776/prevalence-of-relative-systemic-hypertension-in-adults-with-sickle-cell-disease-in-ghana
#7
Amma Benneh-Akwasi Kuma, Amma Twumwa Owusu-Ansah, Mary Akua Ampomah, Fredericka Sey, Edeghonghon Olayemi, Mehdi Nouraie, Solomon Fiifi Ofori-Acquah
Individuals with sickle cell disease particularly with the homozygous (SS) genotype historically have relatively low blood pressure. Nonetheless, they develop vasculopathy-associated organ dysfunction and the risk of organ dysfunction increases at blood pressures that are normal in the general population. This phenomenon is termed relative systemic hypertension (RSH) with a systolic blood pressure range of 120-139 mmHg, and diastolic blood pressure range of 70-89 mmHg. The significance of RSH lies in its association with renal insufficiency, pulmonary hypertension, stroke and propensity to progress to systemic hypertension...
2018: PloS One
https://www.readbyqxmd.com/read/29294650/interpersonal-violence-exposure-and-chronic-pain-in-adult-sickle-cell-patients
#8
Julian D Ford, Damion J Grasso, Sasia Jones, Teresa Works, Biree Andemariam
Almost half of sickle cell disease (SCD) patients develop chronic, debilitating physical pain with uncertain genesis for which they primarily receive opiate-based palliative treatment. Psychological trauma exposure, especially interpersonal victimization, has been linked to the perception of pain in several medical diseases, but has yet to be examined in SCD patients. This study examines self-reported chronicity of pain and use of prescribed opiates in 50 adult SCD patients with and without a history of interpersonal violence exposure...
February 1, 2017: Journal of Interpersonal Violence
https://www.readbyqxmd.com/read/29279789/asthma-screening-in-pediatric-sickle-cell-disease-a-clinic-based-program-using-questionnaires-and-spirometry
#9
Sara C Sadreameli, Rachel O Alade, Peter J Mogayzel, Sharon McGrath-Morrow, John J Strouse
A clinician diagnosis of asthma is associated with increased morbidity and mortality in people with sickle cell disease (SCD). We hypothesized that a screening program would help identify children with asthma needing referral to pulmonary clinic. We conducted a single-center project to screen patients with SCD for asthma using a previously validated questionnaire (Breathmobile) and for pulmonary function abnormalities with portable spirometry. Participants with a positive questionnaire and/or abnormal spirometry were referred to pediatric pulmonary clinic...
December 1, 2017: Pediatric Allergy, Immunology, and Pulmonology
https://www.readbyqxmd.com/read/29279787/acute-chest-syndrome-in-children-with-sickle-cell-disease
#10
REVIEW
Shilpa Jain, Nitya Bakshi, Lakshmanan Krishnamurti
Acute chest syndrome (ACS) is a frequent cause of acute lung disease in children with sickle cell disease (SCD). Patients may present with ACS or may develop this complication during the course of a hospitalization for acute vaso-occlusive crises (VOC). ACS is associated with prolonged hospitalization, increased risk of respiratory failure, and the potential for developing chronic lung disease. ACS in SCD is defined as the presence of fever and/or new respiratory symptoms accompanied by the presence of a new pulmonary infiltrate on chest X-ray...
December 1, 2017: Pediatric Allergy, Immunology, and Pulmonology
https://www.readbyqxmd.com/read/29259931/bone-marrow-necrosis-and-fat-embolism-syndrome-a-dreadful-complication-of-hemoglobin-sickle-cell-disease
#11
Eduardo Pelegrineti Targueta, André Carramenha de Góes Hirano, Fernando Peixoto Ferraz de Campos, João Augusto Dos Santos Martines, Silvana Maria Lovisolo, Aloisio Felipe-Silva
Sickle cell disease encompasses a wide range of genotypic presentation with particular clinical features. The entity affects millions of people, particularly those whose ancestors came from sub-Saharan Africa and other countries in the Western Hemisphere, Saudi Arabia, and India. Currently, the high frequency of S and C genes reflects natural selection through the protection of heterozygotes against severe malaria, the high frequency of consanguineous marriages, improvement of some public health policies and the nutritional standards in the poorer countries where newborns are now living long enough to present for diagnosis and management...
October 2017: Autopsy & Case Reports
https://www.readbyqxmd.com/read/29232169/dietary-nonheme-iron-is-equally-bioavailable-from-ferritin-or-ferrous-sulfate-in-thalassemia-intermedia
#12
Monica Khurana, Ellen B Fung, Elliott P Vichinsky, Elizabeth C Theil
Transfusion-independent patients with thalassemia intermedia (TI) develop fatal iron overload from excessive iron absorption triggered by ineffective erythropoiesis. More information about iron pharmacokinetics and nonheme, dietary iron absorption in such patients is needed to optimize management. To obtain more information, different forms of supplemental nonheme iron sources (ferritin and ferrous sulfate) were compared in 4 TI (hemoglobin <9 g/dL) and 6 control (hemoglobin 12-16 g/dL) patients. Serial serum iron concentrations were measured during the 24 hours following consumption of 1 mg/kg of elemental iron as ferritin or ferrous sulfate...
December 12, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29225982/sarcomatoid-renal-cell-carcinoma-in-an-adolescent-with-sickle-cell-anaemia
#13
H R Ahmad, J A Faruk, M A Bugaje, A Solomon, M O A Samaila, R M Akuse
Malignancies have been reported to occur in people with sickle cell disease. Renal medullary carcinoma (RMC), also tagged seventh sickle cell nephropathy, is an aggressive cancer seen almost exclusively in people with sickle cell disease with more than 160 cases reported worldwide, but only few cases were reported in patients with sickle cell anaemia (HBSS) and from Nigeria. Sarcomatoid renal cell carcinoma is a renal tumour of any histologic variant containing foci of high-grade malignant spindle cells. We report an adolescent girl with sickle cell anaemia (HBSS) who presented with left renal tumour, histology of which confirmed a diagnosis of sarcomatoid renal cell carcinoma (sRCC)...
2017: Case Reports in Oncological Medicine
https://www.readbyqxmd.com/read/29224750/blood-transfusion-in-children-with-sickle-cell-disease-undergoing-tonsillectomy
#14
Carlyn M Atwood, Sharon H Gnagi, Ronald J Teufel, Shaun A Nguyen, David R White
INTRODUCTION: Tonsillectomy is the second most common surgery in children with sickle cell disease. These children are at an increased risk of perioperative complications due to vaso-occlusive events. Although controversial, preoperative blood transfusions are sometimes given in an effort to prevent such complications. The purpose of this study is to analyze trends in the use of blood transfusion for management of children with sickle cell disease (SCD) undergoing tonsillectomy in a national database...
December 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29222302/pain-measurement-tools-in-sickle-cell-disease-where-are-we-now
#15
REVIEW
Deepika S Darbari, Amanda M Brandow
Pain is a complex multidimensional experience and the most common morbidity in patients with sickle cell disease (SCD). Tools to assess pain can be of use not only to guide pain treatment but also to provide insight into underlying pain neurobiology. Mechanisms of pain in SCD are multifactorial and are not completely elucidated. Although vaso-occlusion of microcirculation by sickled red cells is believed to be the underlying mechanism of acute vaso-occlusive pain, mechanisms for chronic pain and the transition from acute to chronic pain are under investigation...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29222301/optimizing-the-care-model-for-an-uncomplicated-acute-pain-episode-in-sickle-cell-disease
#16
REVIEW
Paul Telfer, Banu Kaya
The pathophysiology, clinical presentation, and natural history of acute pain in sickle cell disease are unique and require a disease-centered approach that also applies general principles of acute and chronic pain management. The majority of acute pain episodes are managed at home without the need to access health care. The long-term consequences of poorly treated acute pain include chronic pain, adverse effects of chronic opioid usage, psychological maladjustment, poor quality of life, and excessive health care utilization...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29222286/improving-emergency-department-based-care-of-sickle-cell-pain
#17
REVIEW
Jeffrey A Glassberg
Pain is the leading cause of emergency department (ED) visits for individuals living with sickle cell disease (SCD). The care that is delivered in the ED is often cited by patients with SCD as the area of health care in greatest need of improvement. In 2014, the National Heart, Lung, and Blood Institute released guidelines for the care of SCD, including recommendations for the management of acute sickle cell pain in the ED. These guidelines provide a framework to understand the elements of ideal emergency sickle cell pain care; however, they do not provide guidance on barriers and facilitators to achieving these ideals in the complex system of the ED...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29222285/five-lessons-learned-about-long-term-pain-management-in-adults-with-sickle-cell-disease
#18
REVIEW
Joshua J Field
Chronic pain affects one-half of adults with sickle cell disease (SCD). Despite the prevalence of chronic pain, few studies have been performed to determine the best practices for this patient population. Although the pathophysiology of chronic pain in SCD may be different from other chronic pain syndromes, many of the guidelines outlined in the pain literature and elsewhere are applicable; some were consensus-adopted in the 2014 National Heart, Lung, and Blood Institute SCD Guidelines. Recommended practices, such as controlled substance agreements and monitoring of urine, may seem unnecessary or counterproductive to hematologists...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29207881/sickle-cell-disease-a-malady-beyond-a-hemoglobin-defect-in-cerebrovascular-disease
#19
Junaid Ansari, Youmna E Moufarrej, Rafal Pawlinski, Felicity N E Gavins
Sickle cell disease (SCD) is a devastating monogenic disorder that presents as a multisystem illness and affects approximately 100,000 individuals in the United States alone. SCD management largely focuses on primary prevention, symptomatic treatment and targeting of hemoglobin polymerization and red blood cell sickling. Areas covered: This review will discuss the progress of SCD over the last few decades, highlighting some of the clinical (mainly cerebrovascular) and psychosocial challenges of SCD in the United States...
December 5, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/29196100/mathematical-calculation-of-lifespan-of-transfused-rbcs-in-sickle-cell-disease-patients
#20
Jaehyup Kim, Amena Usmani, Nicole De Simone, Ravi Sarode
BACKGROUND: Transfusion of donor red blood cells (RBCs) remains an important part of management of sickle cell disease (SCD). However, the survival characteristics of transfused donor RBCs in SCD patients have not been well studied. We sought to calculate survival kinetics of transfused RBCs in SCD patients since it is unclear whether transfused RBCs get destroyed at faster rate as innocent bystander or persist longer due to decreased destruction capacity such as functional splenectomy...
November 24, 2017: Transfusion and Apheresis Science
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