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https://www.readbyqxmd.com/read/29222293/current-treatment-algorithm-for-the-management-of-lower-risk-mds
#1
REVIEW
Aristoteles Giagounidis
Lower risk myelodysplastic syndromes (MDS), defined as MDS with a Revised International Prognostic Scoring System score ≤3.5 points, will remain a challenging entity in 2018. Supportive care continues to be the linchpin of treatment, although the options to reduce transfusion needs are broadening. To achieve red blood cell transfusion independence in non-del(5q) patients, erythropoiesis-stimulating agents remain a mainstay of therapy as long as endogenous erythropoietin levels are <500 U/L (and preferably <200 U/L)...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29222267/new-therapeutic-targets-in-transfusion-dependent-and-independent-thalassemia
#2
REVIEW
M Domenica Cappellini, Irene Motta
β-Thalassemias are characterized by reduced production of β-globin chain, resulting in α/β-chain unbalance and precipitation of α-globin-heme complexes and determining ineffective erythropoiesis. Ineffective erythropoiesis, chronic hemolytic anemia, and compensatory hematopoietic expansion are the disease hallmarks, and they are related to the severity of the chain unbalance. Several clinical forms of β-thalassemia, including the coinheritance of β-thalassemia with hemoglobin E resulting in hemoglobin E/β-thalassemia, have been described...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29222265/iron-overload-in-thalassemia-different-organs-at-different-rates
#3
REVIEW
Ali T Taher, Antoine N Saliba
Thalassemic disorders lie on a phenotypic spectrum of clinical severity that depends on the severity of the globin gene mutation and coinheritance of other genetic determinants. Iron overload is associated with increased morbidity in both patients with transfusion-dependent thalassemia (TDT) and non-transfusion-dependent thalassemia (NTDT). The predominant mechanisms driving the process of iron loading include increased iron burden secondary to transfusion therapy in TDT and enhanced intestinal absorption secondary to ineffective erythropoiesis and hepcidin suppression in NTDT...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29217234/outcomes-following-three-factor-inactive-prothrombin-complex-concentrate-versus-recombinant-activated-factor-vii-administration-during-cardiac-surgery
#4
Patrick C Harper, Mark M Smith, Nathan J Brinkman, Melissa A Passe, Darrell R Schroeder, Sameh M Said, Gregory A Nuttall, William C Oliver, David W Barbara
OBJECTIVE: To compare outcomes following inactive prothrombin complex concentrate (PCC) or recombinant activated factor VII (rFVIIa) administration during cardiac surgery. DESIGN: Retrospective propensity-matched analysis. SETTING: Academic tertiary-care center. PARTICIPANTS: Patients undergoing cardiac surgery requiring cardiopulmonary bypass who received either rFVIIa or the inactive 3-factor PCC. INTERVENTIONS: Outcomes following intraoperative administration of rFVIIa (263) or factor IX complex (72) as rescue therapy to treat bleeding...
July 12, 2017: Journal of Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/29215399/hemorrhage-of-liver-and-bone-metastases-as-a-result-of-rapid-response-to-dual-braf-mek-inhibition-in-metastatic-melanoma-a-case-report
#5
Tine Loyson, Emilie Werbrouck, Kevin Punie, Lawrence Bonne, Vincent Vandecaveye, Oliver Bechter
Combination therapy using a BRAF and MEK inhibitor significantly improves both progression-free and overall survival in patients with BRAF V600-mutated stage IV melanoma. Dual MAPK inhibition achieves an objective response in the majority of patients. We present a case of a woman with BRAF V600E-mutated malignant melanoma and rapidly progressing liver, bone, and lymph node metastases. The patient commenced dabrafenib and trametinib with clinical and biochemical signs of response after 2 days. On day 3 she developed grade 3 liver hemorrhage, which was successfully embolized...
December 5, 2017: Melanoma Research
https://www.readbyqxmd.com/read/29214694/myelodysplastic-syndromes-2018-update-on-diagnosis-risk-stratification-and-management
#6
Guillermo Montalban-Bravo, Guillermo Garcia-Manero
DISEASE OVERVIEW: The myelodysplastic syndromes (MDS) are a very heterogeneous group of myeloid disorders characterized by peripheral blood cytopenias and increased risk of transformation to acute myelogenous leukemia (AML). MDS occurs more frequently in older males and in individuals with prior exposure to cytotoxic therapy. DIAGNOSIS: Diagnosis of MDS is based on morphological evidence of dysplasia upon visual examination of a bone marrow aspirate and biopsy. Information obtained from additional studies such as karyotype, flow cytometry or molecular genetics is usually complementary and may help refine diagnosis...
January 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/29214382/bleeding-in-primary-shoulder-arthroplasty
#7
Dominik Malcherczyk, Asma Abdelmoula, Thomas J Heyse, Christian D Peterlein, Brandon Greene, Bilal F El-Zayat
INTRODUCTION: The aim of this investigation was to analyse "total blood loss" (TBL), "blood transfusion rate" (BT) and the "amount of transfused blood units" (BU) between the different primary shoulder arthroplasty (SA) types: reverse, anatomical and stemless. Only primary SA was included. Further goal was to identify risk factors for TBL, amount of BU and BT rate. METHODS: A retrospective charts analysis of patients who received primary SA for degenerative shoulder pathology in our institution between 2004 and 2016 was performed...
December 6, 2017: Archives of Orthopaedic and Trauma Surgery
https://www.readbyqxmd.com/read/29212421/antineoplastic-chemotherapy-in-jehovah-s-witness-patients-with-acute-myelogenous-leukemia-refusing-blood-products-a-matched-pair-analysis
#8
Stefan Wilop, Rainhardt Osieka
BACKGROUND: Acute myelogenous leukemia (AML) may be cured in a substantial number of patients using intensive chemotherapeutic regimens leading to temporary severe myelosuppression. Patients belonging to the denomination of Jehovah's Witnesses (JW), however, are bound by their religious convictions not to accept blood products and are therefore at higher risk for life-threatening events. Reports how to handle this challenge are mainly anecdotal. MATERIAL AND METHODS: We here report in much more detail about our experience with nine patients belonging to the denomination of JW who were treated for AML in our department from 1998 to 2007 and who explicitly wished to receive chemotherapy without blood transfusions...
December 7, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/29211225/off-pump-versus-on-pump-coronary-artery-bypass-grafting-in-frail-patients-study-protocol-for-the-fragile-multicenter-randomized-controlled-trial
#9
Omar Asdrúbal Vilca Mejía, Michel Pompeu Barros Oliveira Sá, Maurilio Onofre Deininger, Luís Roberto Palma Dallan, Rodrigo Coelho Segalote, Marco Antonio Praça de Oliveira, Fernando Antibas Atik, Magaly Arrais Dos Santos, Pedro Gabriel Melo de Barros E Silva, Rodrigo Mussi Milani, Alexandre Ciappina Hueb, Rosangela Monteiro, Ricardo Carvalho Lima, Luiz Augusto Ferreira Lisboa, Luís Alberto Oliveira Dallan, John Puskas, Fabio Biscegli Jatene
INTRODUCTION: Advances in modern medicine have led to people living longer and healthier lives. Frailty is an emerging concept in medicine yet to be explored as a risk factor in cardiac surgery. When it comes to CABG surgery, randomized controlled clinical trials have primarily focused on low-risk (ROOBY, CORONARY), elevated-risk (GOPCABE) or high-risk patients (BBS), but not on frail patients. Therefore, we believe that off-pump CABG could be an important technique in patients with limited functional capacity to respond to surgical stress...
September 2017: Brazilian Journal of Cardiovascular Surgery
https://www.readbyqxmd.com/read/29210068/optimal-storage-conditions-for-apheresis-research-oscar-a-biomedical-excellence-for-safer-transfusion-best-collaborative-study
#10
Thomas J Gniadek, Henk S P Garritsen, David Stroncek, Zbigniew M Szczepiorkowski, David H McKenna
BACKGROUND: Cell therapy products are often stored and transported between sites. The aim of this study was to determine the effect of storage temperature, solution, and cell concentration on nonmobilized, peripheral blood-derived mononuclear cells (MNCs). STUDY DESIGN AND METHODS: This was a multicenter prospective study involving healthy volunteers who underwent nonmobilized MNC collection by apheresis. Products were processed at local laboratories and concentrated to either 100 × 106 or 300 × 106 nucleated cells/mL in 5% human serum albumin (HSA) or HypoThermosol FRS (HT; BioLife Solutions)...
December 6, 2017: Transfusion
https://www.readbyqxmd.com/read/29206727/assessment-of-myocardial-function-and-injury-by-echocardiography-and-cardiac-biomarkers-in-african-children-with-severe-plasmodium-falciparum-malaria
#11
Simon Kotlyar, Peter Olupot-Olupot, Julius Nteziyaremye, Samuel O Akech, Sophie Uyoga, Rita Muhindo, Christopher L Moore, Kathryn Maitland
OBJECTIVES: Perturbed hemodynamic function complicates severe malaria. The Fluid Expansion as Supportive Therapy trial demonstrated that fluid resuscitation, involving children with severe malaria, was associated with increased mortality, primarily due to cardiovascular collapse, suggesting that myocardial dysfunction may have a role. The aim of this study was to characterize cardiac function in children with severe malaria. DESIGN: A prospective observational study with clinical, laboratory, and echocardiographic data collected at presentation (T0) and 24 hours (T1) in children with severe malaria...
December 4, 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/29203157/-anticoagulation-impact-on-bleeding-risk-during-holep-monocentric-study-of-156-patients
#12
P Neuville, R Codas, E Ravier, D Maucort-Boulch, L Badet, H Fassi-Fehri
OBJECTIVE: Compare the length of hospital stay and the complications after HoLEP between three groups of patients: a control group, a group with antiplatelet therapy, a group with anticoagulation therapy. MATERIALS: Retrospective cohort study that included all consecutive patients who underwent HoLEP for prostatic hyperplasia in our center from may 2013 to may 2016. Anticoagulated patients and patients under clopidogrel had respectively a relay with heparine and aspirine...
December 1, 2017: Progrès en Urologie
https://www.readbyqxmd.com/read/29200160/glucose-homeostasis-and-effect-of-chelation-on-%C3%AE-cell-function-in-children-with-%C3%AE-thalassemia-major
#13
Sunil Gomber, Aashima Dabas, Shilpa Bagmar, Sri Venkata Madhu
OBJECTIVE: To assess the prevalence of impaired glucose tolerance in β-thalassemia major and correlate it with chelation therapy. MATERIALS AND METHODS: Sixty-seven subjects with β-thalassemia major, aged 1 to 20 years, were enrolled in our prospective cohort. Clinical details were recorded. Baseline oral glucose tolerance test, serum insulin, C peptide, and insulin resistance were measured. The biochemical profile was repeated after 6 months. RESULTS: The mean age of subjects was 7...
December 1, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29199112/-management-of-patients-with-major-beta-thalassemia-in-a-paediatric-department-in-the-south-of-tunisia-about-26-cases
#14
I Maaloul, O Laaroussi, I Jedidi, L Sfaihi, S Kmiha, T Kamoun, H Aloulou, M Hachicha
AIM: Our objectives were to assess the management of patients with major thalassemia and identify the various complications and monitoring means. PATIENTS AND METHODS: A retrospective study was conducted on 26 β-thalassemic patients in the department of paediatrics, Hédi Chaker hospital, Sfax, Tunisia during a period of 25 years (from 1 January 1990 to 31 December 2014). RESULTS: The mean age of the beginning of transfusion was 11.5 months...
November 30, 2017: Transfusion Clinique et Biologique: Journal de la Société Française de Transfusion Sanguine
https://www.readbyqxmd.com/read/29198634/intraoperative-administration-of-4-factor-prothrombin-complex-concentrate-reduces-blood-requirements-in-cardiac-transplantation
#15
Gina H Sun, Visal Patel, Ingrid Moreno-Duarte, Farhad Zahedi, Eric Ursprung, Greg Couper, Fred Y Chen, Ian J Welsby, Raymond Comenzo, Grace Kao, Frederick C Cobey
OBJECTIVE: Assessing the efficacy of intraoperative 4-factor prothrombin complex concentrate (4F-PCC) use in blood product utilization, time to chest closure, intensive care unit (ICU) and hospital length of stay (LOS), thromboembolic complications, renal injury and mortality in left ventricular assist device (LVAD) patients on home anticoagulation therapy with warfarin, undergoing orthotopic heart transplantation (OHT). DESIGN: Retrospective analysis of OHT patients at Tufts Medical Center from May 2013 to October 2016...
August 3, 2017: Journal of Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/29195008/consensus-predictive-model-for-the-prediction-of-human-k562-cell-growth-inhibition-through-enalos-cloud-platform
#16
Antreas Afantitis, Georgios Leonis, Roberto Gambari, Georgia Melagraki
Beta thalassemia is an inherited hematologic disorder caused by various mutations of the β-globin gene, thus resulting in a significant decrease of adult hemoglobin (HbA) production. Since no other specific treatment exists to date, apart from blood transfusion and chelation therapy, the increase of fetal hemoglobin (HbF) levels by drug molecules is considered of great potential in β-thalassemia treatment, being expected to counterbalance the impaired production of HbA. Within this context, we have worked towards the development of a predictive model that will allow the identification of new possible HbF inducers...
December 1, 2017: ChemMedChem
https://www.readbyqxmd.com/read/29193160/multicenter-experience-with-mechanical-circulatory-support-using-a-new-diagonal-pump-in-233-children
#17
Brigitte Stiller, Robert Jan Houmes, André Rüffer, Matthias Kumpf, Andreas Müller, Florian Kipfmüller, Harald Köditz, Susanne Herber Jonat, Claudia Schmoor, Christoph Benk, Dick Tibboel, Thilo Fleck
Technological innovations in pediatric extracorporeal life support circuits can reduce system-related complications and may improve patients' outcome. The Deltastream DP3 (Medos Medizintechnik AG, Stolberg, Germany) is a novel rotational pump with a diagonally streamed impeller that can be used over a broad range of flows. We collected patient data from seven pediatric centers to conduct a retrospective cohort study. We examined 233 patients whose median age was 1.9 (0-201) months. The DP3 system was used for cardiopulmonary support as veno-arterial extracorporeal membrane oxygenation (ECMO) in 162 patients...
November 29, 2017: Artificial Organs
https://www.readbyqxmd.com/read/29192880/negative-pressure-dressing-management-of-recurrent-postoperative-epidural-hematoma-and-uncontrollable-intraoperative-hemorrhage-in-posterior-spine-surgery-report-of-4-cases
#18
Kenneth C Foxx, Addisu Mesfin, Robert Molinari
There is a paucity of literature describing the management of recurrent symptomatic postoperative epidural hematoma or uncontrollable intraoperative hemorrhage in posterior spine surgery. Traditional management with hematoma evacuation and wound closure over suction drains may not be effective in certain cases, and it can lead to recurrence and neurological injury. The authors report 3 cases of recurrent symptomatic postoperative epidural hematoma successfully managed with novel open-wound negative-pressure dressing therapy (NPDT), as well as 1 case of uncontrollable intraoperative hemorrhage that was primarily managed with the same technique...
December 1, 2017: Journal of Neurosurgery. Spine
https://www.readbyqxmd.com/read/29192575/-current-dilemmas-on-the-transmission-of-hepatitis-e-virus
#19
E de Weerd-de Jong, A M Peters, J C Rahamat-Langendoen, N M A Blijlevens
Immunocompromised patients are especially at risk for developing chronic hepatitis E virus (HEV) infection, which may result in progressive liver disease and cirrhosis. In addition, treatment of chronic HEV infection in these patients often includes dose reduction of immunosuppressive therapy and this may lead to severe flare-ups of the underlying condition or even rejection of transplant material. Therefore prevention of HEV transmission is being more and more recognised as an essential step to stop increasing HEV seroprevalence...
2017: Nederlands Tijdschrift Voor Geneeskunde
https://www.readbyqxmd.com/read/29191945/thrombopoietin-mutation-in-congenital-amegakaryocytic-thrombocytopenia-treatable-with%C3%A2-romiplostim
#20
Alessandro Pecci, Iman Ragab, Valeria Bozzi, Daniela De Rocco, Serena Barozzi, Tania Giangregorio, Heba Ali, Federica Melazzini, Mohamed Sallam, Caterina Alfano, Annalisa Pastore, Carlo L Balduini, Anna Savoia
Congenital amegakaryocytic thrombocytopenia (CAMT) is an inherited disorder characterized at birth by thrombocytopenia with reduced megakaryocytes, which evolves into generalized bone marrow aplasia during childhood. Although CAMT is genetically heterogeneous, mutations of MPL, the gene encoding for the receptor of thrombopoietin (THPO), are the only known disease-causing alterations. We identified a family with three children affected with CAMT caused by a homozygous mutation (p.R119C) of the THPO gene. Functional studies showed that p...
November 30, 2017: EMBO Molecular Medicine
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