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Sickle cell anemia

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https://www.readbyqxmd.com/read/29318647/realizing-effectiveness-across-continents-with-hydroxyurea-enrollment-and-baseline-characteristics-of-the-multicenter-reach-study-in-sub-saharan-africa
#1
Patrick T McGann, Thomas N Williams, Peter Olupot-Olupot, George A Tomlinson, Adam Lane, José Luís Reis da Fonseca, Robert Kitenge, George Mochamah, Ham Wabwire, Susan Stuber, Thad A Howard, Kathryn McElhinney, Banu Aygun, Teresa Latham, Brígida Santos, Léon Tshilolo, Russell E Ware
Despite its well-described safety and efficacy in the treatment of sickle cell anemia (SCA) in high-income settings, hydroxyurea remains largely unavailable in sub-Saharan Africa, where more than 75% of annual SCA births occur and many comorbidities exist. Realizing Effectiveness Across Continents with Hydroxyurea (REACH, ClinicalTrials.gov NCT01966731) is a prospective, Phase I/II open-label trial of hydroxyurea designed to evaluate the feasibility, safety, and benefits of hydroxyurea treatment for children with SCA in four sub-Saharan African countries...
January 10, 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/29313508/evaluation-of-transfusion-practices-in-sickle-cell-disease-in-senegal-cohort-study-of-1078-patients-with-sickle-cell-disease
#2
M Seck, A Tall, B F Faye, D Sy Bah, Y Guéye, A Sall, A O Touré, S Diop
Blood transfusion is an essential therapeutic tool in the treatment of sickle cell disease. Its indications and modalities vary from one country to another as a function of the clinical severity of the disease and the availability of blood products. This study seeks to evaluate the frequency and modalities of blood transfusions as well as their indications in patients with sickle cell syndrome at the Dakar hematology department from 1995 through 2015. This retrospective study reviewed the records of a cohort of 1078 patients with SS, SC, Sβ0 and Sβ + thalassemia sickle cell syndromes and collected data about the type of blood products, indications, modalities, and acute transfusion complications...
November 1, 2017: Médecine et Santé Tropicales
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
#3
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/29311011/-screening-for-nephropathy-in-major-sickle-cell-syndromes-in-patients-monitored-at-the-national-reference-center-for-sickle-cell-disease-in-niamey-niger
#4
Moumouni Garba, Zeinabou Maiga Moussa Tondi, Hassan Diongoule, Samaila Aboubacar, Abdou Ide, Nadège Ruddy Biyao-Nelson, Illiassou Soumaila, Soumana Alido
BACKGROUND: Sickle cell anemia is the most common hereditary hemopathy in the world. It is a disease that attacks all the systems of the organism. The kidneys are among the most sensitive organs of this disease. The main objective of this study is to detect sickle cell nephropathy in patients followed at the National Reference Center for Sickle Cell Disease in Niamey. METHODS: It is a prospective study carried out over a period of one year (January to December 2016)...
January 5, 2018: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/29309293/orthopaedic-manifestations-of-sickle-cell-disease
#5
Kelly L Vanderhave, Crystal A Perkins, Brian Scannell, Brian K Brighton
Sickle cell disease (SCD) is an autosomal recessive disorder that results in hemolytic anemia related to abnormal hemoglobin and erythrocyte levels. SCD is characterized by vascular occlusive episodes, visceral sequestration, and aplastic or hemolytic crises. These crises most commonly occur in bone. The orthopaedic manifestations of SCD comprise much of the morbidity associated with this disorder. Osteonecrosis and osteomyelitis are among the most disabling and serious musculoskeletal complications in patients with SCD...
January 5, 2018: Journal of the American Academy of Orthopaedic Surgeons
https://www.readbyqxmd.com/read/29303133/relationship-between-zinc-levels-and-anthropometric-indices-among-school-aged-female-children-with-sickle-cell-anemia-in-enugu-nigeria
#6
V O Onukwuli, A N Ikefuna, A R Nwokocha, I J Emodi, C B Eke
BACKGROUND: Sickle cell anaemia is one of the most common inherited disorders globally. Some affected children have retardation of physical growth which is also seen in those with zinc deficiency. OBJECTIVE: To assess the relationship between zinc levels and anthropometric indices of SCA children. METHODS: A cross- sectional, case-control study on young females aged 6-18 years at the UNTH, Enugu. Relevant clinical data as well as 24 hour dietary recall were collected...
November 2017: Nigerian Journal of Clinical Practice
https://www.readbyqxmd.com/read/29303127/assessment-of-biochemical-liver-function-tests-in-relation-to-age-among-steady-state-sickle-cell-anemia-patients
#7
S A Akuyam, A Abubakar, N Lawal, R Yusuf, S M Aminu, A Hassan, A Musa, A K Bello, I A Yahaya, P A Okafor
BACKGROUND AND OBJECTIVE: Multiorgan failure including liver dysfunction is a common finding in sickle cell anemia (SCA) patients, the cause of which is multifactorial with advancing age said to be a major determinant. There is a paucity of data on liver function among SCA patients in relation to age in northern Nigerian hospitals, including Ahmadu Bello University Teaching Hospital (ABUTH), Zaria. This study was to assess the biochemical liver function tests (LFTs) as they relate to age among SCA patients in steady state, with a view to improving the overall monitoring of these patients...
November 2017: Nigerian Journal of Clinical Practice
https://www.readbyqxmd.com/read/29296909/primum-non-nocere-the-case-against-transplant-for-children-with-sickle-cell-anemia-without-progressive-end-organ-disease
#8
Michael R DeBaun, Ellen Wright Clayton
This article has a companion Point by Fitzhugh and Walters.
December 12, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296908/the-case-for-hla-identical-sibling-hematopoietic-stem-cell-transplantation-in-children-with-symptomatic-sickle-cell-anemia
#9
Courtney D Fitzhugh, Mark C Walters
This article has a companion Counterpoint by DeBaun and Clayton.
December 12, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296825/disease-severity-and-slower-psychomotor-speed-in-adults-with-sickle-cell-disease
#10
Dana R Jorgensen, Andrea Metti, Meryl A Butters, Joseph M Mettenburg, Caterina Rosano, Enrico M Novelli
Psychomotor slowing is common in children with sickle cell disease (SCD), but little is known about its severity in adults. We conducted a cross-sectional study to quantify psychomotor speed, measured with the digit symbol substitution test (DSST), in relationship with disease severity in adults with SCD attending an outpatient clinic (n = 88, age 36.3 years). Genotype was used to group patients in "severe" (homozygous for hemoglobin S or compound heterozygous with β0 thalassemia) or "moderate" groups (compound heterozygous for HbS, with either HbC or β+ thalassemia)...
September 26, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296782/whole-exome-sequencing-for-rh-genotyping-and-alloimmunization-risk-in-children-with-sickle-cell-anemia
#11
Stella T Chou, Jonathan M Flanagan, Sunitha Vege, Naomi L C Luban, R Clark Brown, Russell E Ware, Connie M Westhoff
RH genes are highly polymorphic and encode the most complex of the 35 human blood group systems. This genetic diversity contributes to Rh alloimmunization in patients with sickle cell anemia (SCA) and is not avoided by serologic Rh-matched red cell transfusions. Standard serologic testing does not distinguish variant Rh antigens. Single nucleotide polymorphism (SNP)-based DNA arrays detect many RHD and RHCE variants, but the number of alleles tested is limited. We explored a next-generation sequencing (NGS) approach using whole-exome sequencing (WES) in 27 Rh alloimmunized and 27 matched non-alloimmunized patients with SCA who received chronic red cell transfusions and were enrolled in a multicenter study...
August 8, 2017: Blood Advances
https://www.readbyqxmd.com/read/29295792/sickle-cell-retinopathy-and-other-chronic-complications-of-sickle-cell-anemia-a-clinical-study-of-84-sub-saharan-african-cases-cameroon
#12
Y Bilong, M Dubert, G Koki, J J Noubiap, H N Pangetna, A Menet, D Chelo, L Offredo, S Jacob, S Belinga, A N A Yanda, S Kingue, X Jouven, B Ranque, L A Bella
OBJECTIVE: Sickle retinopathy is a severe complication of sickle cell disease than can lead to blindness. We aim to describe the epidemiology of sickle retinopathy in homozygous sickle cell (SS) African patients and to analyze its association with non-ophthalmologic disease complications of sickle cell anemia. METHODS: We conducted a nested study within the CADRE cohort in Cameroon. Eighty-four consecutive SS outpatients, aged 10 years and older, with no visual symptoms, underwent an ophthalmologic examination...
December 30, 2017: Journal Français D'ophtalmologie
https://www.readbyqxmd.com/read/29282441/platelet-transfusion-thresholds-among-children-admitted-to-a-pediatric-intensive-care-unit
#13
Batool Alsheikh, Madhuradhar Chegondi, Balagangadhar Totapally
Objective To evaluate the threshold for platelet transfusion in children admitted to a pediatric intensive care unit (PICU). This is a retrospective chart review study, conducted at our tertiary level PICU and is related to critically ill pediatric patients who required platelet transfusion. Methods We retrieved the target patient population data from our blood bank database. The patients were subdivided into four subgroups: hematologic (hematologic malignancies, bone marrow suppression, hemolytic anemia, and sickle cell disease), surgical (post-surgical, trauma and acute bleeding), the unstable fraction of inspired oxygen (FiO2 > 0...
October 24, 2017: Curēus
https://www.readbyqxmd.com/read/29280474/anemia-in-pregnancy-a-pragmatic-approach
#14
Dongmei Sun, Anne McLeod, Shital Gandhi, Ann Kinga Malinowski, Nadine Shehata
Importance: Anemia is common in pregnancy, ranging from 5.4% in developed countries to more than 80% in developing countries. Anemia in pregnancy has been associated with prematurity, low birth weight, and adverse pregnancy outcomes. Objective: This review uses clinical vignettes to illustrate the clinical presentations, approach to diagnosis, maternal and fetal implications, and treatment for the common etiologies of anemia in pregnancy. Evidence Acquisition: Literature review...
December 2017: Obstetrical & Gynecological Survey
https://www.readbyqxmd.com/read/29261629/false-positive-heat-damaged-rbc-scan-for-splenosis-case-of-retroperitoneal-extramedullary-hematopoiesis
#15
Joseph S Donald, Delphine L Chen, Jessica Peterson, Mark Perry
Extramedullary hematopoiesis (EMH) is a well-known compensatory response to severe anemia, most commonly due to a chronic hemolytic anemia such as thalassemia or sickle cell disease. We present a case of a 72-year-old man with autoimmune hemolytic anemia refractory to steroids and splenectomy. An incidental retroperitoneal mass on CT was suggestive of ectopic splenic tissue. However, an alternative diagnosis of EMH was considered, given an atypical appearance on heat-damaged Tc-RBC scan, and was confirmed with biopsy...
December 19, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/29260615/preliminary-evidence-for-cerebral-capillary-shunting-in-adults-with-sickle-cell-anemia
#16
Meher R Juttukonda, Manus J Donahue, Larry T Davis, Melissa C Gindville, Chelsea A Lee, Niral J Patel, Adetola A Kassim, Sumit Pruthi, Jeroen Hendrikse, Lori C Jordan
Elevated flow velocities in adults with sickle cell anemia (SCA) may cause rapid erythrocyte transit through capillaries. This phenomenon could present as dural venous sinus hyperintensity on arterial spin labeling (ASL)-MRI and could be indicative of capillary shunting. Here, the prevalence of ASL venous hyperintensities and association with relevant physiology in adults with SCA was investigated. SCA ( n = 46) and age-matched control ( n = 16) volunteers were recruited for 3.0 T MRI. Pseudo-continuous ASL-MRI was acquired for cerebral blood flow (CBF) calculation and venous hyperintensity determination; venous signal intensity and a categorical venous score (three raters; 0 = no hyperintensity, 1 = focal hyperintensity, and 2 = diffuse hyperintensity) were recorded...
January 1, 2017: Journal of Cerebral Blood Flow and Metabolism
https://www.readbyqxmd.com/read/29251012/enhancing-effect-of-hydroxyurea-on-hb-f-in-sickle-cell-disease-ten-year-egyptian-experience
#17
Ilham Youssry, Amina Abdel-Salam, Rania Ismail, Rayan Bou-Fakhredin, Rania Mohamed Samy, Fatma Ezz El-Deen, Ali T Taher
Patients with sickle cell disease experience hemolytic anemia and vaso-occlusions that result in pain, organ injury, and premature mortality. Several prospective studies have verified the efficacy and tolerability of hydroxyurea (HU), and demonstrated its efficacy in reducing painful vaso-occlusive crises (VOCs) in addition to its ability to increase Hb F levels. We aimed to evaluate the long-term effects of HU therapy on Hb F and assess its long term efficacy and safety in sickle cell disease patients. A retrospective study on 60 sickle cell disease patients was conducted...
December 18, 2017: Hemoglobin
https://www.readbyqxmd.com/read/29236859/mentha-pulegium-crude-extracts-induce-thiol-oxidation-and-potentiate-hemolysis-when-associated-to-t-butyl-hydroperoxide-in-human-s-erythrocytes
#18
Matheus C Bianchini, Dennyura O Galvão, Tatiana Tamborena, Claudia O Alves, Robson L Puntel
Mentha pulegium (Lamiaceae) tea has been used as a traditional medicine; however, the modulatory effect of M. pulegium extracts on damage to human erythrocytes associated to t-butyl hydroperoxide (t-BHP) exposure remains to be investigated. Accordingly, we perform this study in order to test the hypothesis that aqueous and ethanolic extracts of M. pulegium could modulate the hemolysis associated to t-BHP exposure, non-protein thiol (NPSH) oxidation and lipid peroxidation (measured as thiobarbituric acid reactive substances - TBARS) in human erythrocytes...
December 11, 2017: Anais da Academia Brasileira de Ciências
https://www.readbyqxmd.com/read/29232169/dietary-nonheme-iron-is-equally-bioavailable-from-ferritin-or-ferrous-sulfate-in-thalassemia-intermedia
#19
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/29229306/pseudo-continuous-arterial-spin-labeling-quantification-in-anemic-subjects-with-hyperemic-cerebral-blood-flow
#20
Adam Bush, Yaqiong Chai, So Young Choi, Lena Vaclavu, Scott Holland, Aart Nederveen, Thomas Coates, John Wood
PURPOSE: To investigate possible sources of quantification errors in global cerebral blood flow (CBF) measurements by comparing pseudo continuous arterial spin labeling (PCASL) and phase contrast (PC) MRI in anemic, hyperemic subjects. METHODS: All studies were performed on a Philips 3T Achieva MRI scanner. PC and PCASL CBF examinations were performed in 10 healthy, young adult subjects and 18 young adults with chronic anemia syndromes including sickle cell disease and thalassemia...
December 8, 2017: Magnetic Resonance Imaging
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