Database : MEDLINE
Search on : Factor and Xi and Deficiency [Words]
References found : 1470 [refine]
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[PMID]: 29399875
[Au] Autor:Fitzsimons MG; Leaf RK; Mack J; Bendapudi PK; Shen T; Cameron DE
[Ad] Address:Division of Cardiac Anesthesia, Department of Anesthesia, Critical Care, and Pain Medicine, Massachusetts General Hospital, Boston, Massachusetts.
[Ti] Title:Perioperative management of a redo aortic root replacement in a patient with severe factor XI deficiency.
[So] Source:J Card Surg;33(2):86-89, 2018 Feb.
[Is] ISSN:1540-8191
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Factor XI deficiency is associated with significant bleeding in the setting of trauma and surgery. We present a patient with FXI deficiency and multiple red blood cell allo-antibodies requiring repeat aortic root replacement and discuss the perioperative management of patients with FXI deficiency undergoing cardiac surgery.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180222
[Lr] Last revision date:180222
[St] Status:In-Process
[do] DOI:10.1111/jocs.13526

  2 / 1470 MEDLINE  
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[PMID]: 29467123
[Au] Autor:McCarthy ML; Ordway SM; Jones RM; Perkins JG
[Ad] Address:Georgetown University School of Medicine, Washington DC, Columbia, USA.
[Ti] Title:Successful perioperative management in a patient with factor XI deficiency.
[So] Source:BMJ Case Rep;2018, 2018 Feb 21.
[Is] ISSN:1757-790X
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Factor XI (FXI) deficiency is an autosomal disorder which manifests as bleeding of varying severity. While homozygotes typically experience more dramatic bleeding symptoms, heterozygotes may experience clinically significant bleeding following surgical procedures or trauma, and therefore the condition is not purely recessive. The clinical significance of FXI deficiency is complicated in that FXI levels do not correlate well with bleeding severity, and in fact the bleeding risk is variable even for an individual in response to different haemostatic challenges. We present the case of a 74-year-old man of Ashkenazi Jewish heritage with a family and personal history of bleeding during surgical procedures, who presented with excessive bleeding following total thyroidectomy. He was found to have a FXI level of 52% (low normal). Genetic testing revealed that he was heterozygous for the c.403G>T mutation. This case demonstrates successful work-up and perioperative management of a patient with FXI deficiency.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180222
[Lr] Last revision date:180222
[St] Status:In-Process

  3 / 1470 MEDLINE  
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[PMID]: 29230938
[Au] Autor:Puetz J; Hugge C; Moser K
[Ad] Address:Division of Pediatric Hematology/Oncology, Department of Pediatrics, Saint Louis University School of Medicine, St. Louis, Missouri.
[Ti] Title:Normal aPTT in children with mild factor XI deficiency.
[So] Source:Pediatr Blood Cancer;65(4), 2018 Apr.
[Is] ISSN:1545-5017
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:It has been suggested that persons with factor XI deficiency can have a normal activated partial thromboplastin time (aPTT). This notion is based on limited data, especially in children. Because of the central role the aPTT plays in diagnostic algorithms for bleeding disorders, it is important to know if a normal aPTT eliminates the need for factor XI activity testing. Our institutional database contains seven children with factor XI deficiency, of whom four have a normal aPTT. This supports the hypothesis that children with factor XI deficiency can have a normal aPTT. Clinicians may wish to consider this evidence when evaluating children with abnormal bleeding.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1712
[Cu] Class update date: 180221
[Lr] Last revision date:180221
[St] Status:In-Data-Review
[do] DOI:10.1002/pbc.26910

  4 / 1470 MEDLINE  
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[PMID]: 29388750
[Au] Autor:Gebhart J; Hofer S; Panzer S; Quehenberger P; Sunder-Plassmann R; Hoermann G; Eigenbauer E; Haslacher H; Kepa S; Kyrle PA; Eichinger S; Knöbl P; Eischer L; Mannhalter C; Ay C; Pabinger I
[Ad] Address:Department of Medicine I, Clinical Division of Hematology and Hemostaseology, Medical University of Vienna, Vienna, Austria.
[Ti] Title:High proportion of patients with bleeding of unknown cause in persons with a mild-to-moderate bleeding tendency: Results from the Vienna Bleeding Biobank (VIBB).
[So] Source:Haemophilia;, 2018 Feb 01.
[Is] ISSN:1365-2516
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:INTRODUCTION: Data on clinical characteristics and the prevalence of underlying coagulopathies in patients with mild-to-moderate bleeding disorders (MBDs) are scarce. AIM: We established the Vienna Bleeding Biobank (VIBB) to characterize and thoroughly investigate Austrian patients with MBDs. RESULTS: Four hundred eighteen patients (female = 345, 82.5%) were included. A platelet function defect (PFD) was diagnosed in 26 (6.2%) and a possible PFD in 30 (7.2%) patients. Eight patients (1.9%) were diagnosed with von Willebrand disease (VWD) (type 1 n = 6; type 2 n = 2), and 29 patients had low VWF (30-50 IU/dL). Deficiencies in factor VIII, IX, XI or XIII were found in 11 (2.6%), 3 (0.7%), 3 (0.7%) and 1 patient(s), 2 patients had dysfibrinogenaemia, and further 2 had possible PFD and FXI deficiency. Probable causal mutations were detected in 8 of 11 patients with FVIII deficiency, 2 of 3 patients with FIX deficiency and 2 of 8 patients with VWD. Three hundred three patients (72.5%) had normal results in the coagulation assays and were categorized as patients with bleeding of unknown cause (BUC). The bleeding score did not differ between patients with and without established diagnosis. A diagnosis of a bleeding disorder was more frequently made in men than in women (49.3% vs 22.9%). Male sex (OR 3.55, 95% CI: 2.02-6.22; P < .001) and blood group 0 (OR 1.86, 95% CI: 1.17-2.94; P = .008) were independently associated with diagnosis of a bleeding disorder. CONCLUSION: The high rate of patients with BUC despite in-depth haemostatic assessment underlines the incompleteness of available routine laboratory tests. Males with MBDs were more likely to be diagnosed with an established bleeding disorder than females.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180201
[Lr] Last revision date:180201
[St] Status:Publisher
[do] DOI:10.1111/hae.13422

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[PMID]: 29381511
[Au] Autor:Shander A; Friedman T; Palleschi G; Shore-Lesserson L
[Ti] Title:The Evolving Dilemma of Factor XI in Pregnancy: Suggestions for Management.
[So] Source:Anesth Analg;, 2018 Jan 29.
[Is] ISSN:1526-7598
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:A case of a patient with severe factor XI (FXI) deficiency who presented for her seventh labor and delivery is presented. The nature of FXI deficiency, its prevalence, and issues related to genetic screening are discussed. Published literature on the topic is reviewed, including criteria that were developed to assess bleeding, laboratory tools used to estimate bleeding risk, and available treatments. Within the context of this challenging clinical dilemma, specific recommendations are provided for the antepartum, intrapartum, and postpartum stages of pregnancy. These include recommendations that take into account both FXI levels and history of any abnormal bleeding. While there are effective treatments available, it is important to consider that institutional multidisciplinary protocols are needed to manage this complex disorder. More work is needed to define the best management protocols.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1801
[Cu] Class update date: 180130
[Lr] Last revision date:180130
[St] Status:Publisher
[do] DOI:10.1213/ANE.0000000000002836

  6 / 1470 MEDLINE  
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[PMID]: 29269514
[Au] Autor:Weitz JI; Fredenburgh JC
[Ad] Address:From the Department of Medicine (J.I.W., J.C.F.) and Department of Biochemistry and Biomedical Sciences (J.I.W.), McMaster University, Hamilton, Ontario, Canada; and Thrombosis and Atherosclerosis Research Institute (J.I.W., J.C.F.), Hamilton, Ontario, Canada. weitzj@taari.ca.
[Ti] Title:2017 Scientific Sessions Sol Sherry Distinguished Lecture in Thrombosis: Factor XI as a Target for New Anticoagulants.
[So] Source:Arterioscler Thromb Vasc Biol;, 2017 Dec 21.
[Is] ISSN:1524-4636
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:The goal of anticoagulant therapy is to attenuate thrombosis without compromising hemostasis. Although the direct oral anticoagulants are associated with less intracranial hemorrhage than vitamin K antagonists, bleeding remains their major side effect. Factor XI has emerged as a promising target for anticoagulants that may be safer than those currently available. The focus on factor XI stems from epidemiological evidence of its role in thrombosis, the observation of attenuated thrombosis in factor XI-deficient mice, identification of novel activators, and the fact that factor XI deficiency is associated with only a mild bleeding diathesis. Proof-of-concept comes from the demonstration that compared with enoxaparin, factor XI knockdown reduces venous thromboembolism without increasing bleeding after elective knee arthroplasty. This article rationalizes the selection of factor XI as a target for new anticoagulants, reviews the agents under development, and outlines a potential path forward for their development.
[Pt] Publication type:JOURNAL ARTICLE; REVIEW
[Em] Entry month:1712
[Cu] Class update date: 171222
[Lr] Last revision date:171222
[St] Status:Publisher

  7 / 1470 MEDLINE  
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[PMID]: 29207114
[Au] Autor:Shnerb Ganor R; Harats D; Schiby G; Rosenblatt K; Lubitz I; Shaish A; Salomon O
[Ad] Address:The Bert W. Strassburger Lipid Center, Sheba Medical Center, Tel­Hashomer 5265601, Israel.
[Ti] Title:Elderly apolipoprotein E­/­ mice with advanced atherosclerotic lesions in the aorta do not develop Alzheimer's disease-like pathologies.
[So] Source:Mol Med Rep;17(2):2488-2492, 2018 Feb.
[Is] ISSN:1791-3004
[Cp] Country of publication:Greece
[La] Language:eng
[Ab] Abstract:Atherosclerosis and Alzheimer's disease (AD) are a major cause of morbidity and mortality in Western societies. These diseases share common risk factors, which are exhibited in old age, including hypertension, diabetes, hypercholesterolemia and apolipoprotein (Apo) ε4 allele. We previously demonstrated that factor XI (FXI) deficiency in mice reduced the atherosclerotic plaque area in coronary sinuses and the aortic arch. This led us to investigate whether FXI deficiency in elderly ApoE knockout (KO) mice would decrease pathological alterations compatible with atherosclerosis and AD. The present study used ApoE/factor XI double KO (ApoE/FXI DKO) mice aged 64 weeks and age­matched ApoE KO mice to serve as a control group. The ApoE KO mice developed an advanced atherosclerotic lesion area in the aortic arch, which was reduced by 33% in the DKO mice. However, neither atherosclerosis nor AD­associated pathological alterations in the elderly mice brains were observed in either the DKO mice or the ApoE KO mice. The results advocate a dichotomy between the brain and peripheral blood vessels. Therefore, the ApoE KO and DKO mice cannot serve as mouse models for studying AD or pathological brain changes compatible with atherosclerosis. The mechanism by which ApoE KO protects against brain pathology should be further studied as it may prove helpful for future treatment of senile dementia.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1712
[Cu] Class update date: 171219
[Lr] Last revision date:171219
[St] Status:In-Process
[do] DOI:10.3892/mmr.2017.8127

  8 / 1470 MEDLINE  
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[PMID]: 29178608
[Au] Autor:Rimoldi V; Paraboschi EM; Menegatti M; Peyvandi F; Salomon O; Duga S; Asselta R
[Ad] Address:Humanitas Clinical and Research Center, Rozzano, Milan, Italy.
[Ti] Title:Molecular investigation of 41 patients affected by coagulation factor XI deficiency.
[So] Source:Haemophilia;, 2017 Nov 27.
[Is] ISSN:1365-2516
[Cp] Country of publication:England
[La] Language:eng
[Pt] Publication type:LETTER
[Em] Entry month:1711
[Cu] Class update date: 171127
[Lr] Last revision date:171127
[St] Status:Publisher
[do] DOI:10.1111/hae.13378

  9 / 1470 MEDLINE  
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[PMID]: 29165739
[Au] Autor:Franchini M; Marano G; Mengoli C; Piccinini V; Pupella S; Vaglio S; Liumbruno GM
[Ad] Address:Italian National Blood Centre, National Institute of Health, Rome, Italy.
[Ti] Title:Inhibitors in Patients with Congenital Bleeding Disorders Other Than Hemophilia.
[So] Source:Semin Thromb Hemost;, 2017 Nov 17.
[Is] ISSN:1098-9064
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:The most worrying complication of replacement therapy for severe hemophilia A and B is currently the occurrence of inhibitory alloantibodies against infused factor VIII and factor IX, respectively. Inhibitors compromise the management of hemorrhage in affected patients, with a considerable increase in complications, disability, and costs. While these alloantibodies have been extensively studied in the past years in hemophilia A and B, those occurring in patients with other inherited bleeding disorders are less well characterized and still poorly understood, mostly due to the rarity of these hemorrhagic conditions. This narrative review will deal with inhibitors arising in patients with inherited bleeding disorders other than "classical" hemophilia, focusing in particular on those developing in patients with congenital deficiency of coagulation factor V, factor VII, factor XI, and factor XIII.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1711
[Cu] Class update date: 171122
[Lr] Last revision date:171122
[St] Status:Publisher
[do] DOI:10.1055/s-0037-1607441

  10 / 1470 MEDLINE  
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[PMID]: 29138690
[Au] Autor:Tiscia GL; Favuzzi G; Lupone MR; Cappucci F; Schiavulli M; Mirabelli V; D'Andrea G; Chinni E; Giuliani N; Caliandro R; Grandone E
[Ad] Address:IRCCS 'Casa Sollievo della Sofferenza', Unità di Aterosclerosi e Trombosi, San Giovanni Rotondo (Foggia), Italy.
[Ti] Title:Factor XI gene variants in factor XI-deficient patients of Southern Italy: identification of a novel mutation and genotype-phenotype relationship.
[So] Source:Hum Genome Var;4:17043, 2017.
[Is] ISSN:2054-345X
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Congenital Factor XI (FXI) deficiency shows a high variability in clinical phenotype. To date, many allele variants have been shown to cause this bleeding disorder. However, the genotype-phenotype relationship is difficult to establish. This report provides insights into this bleeding disorder. Sixteen unrelated Italian index cases with congenital FXI deficiency and their relatives were investigated. After the identification of the deficiency, we obtained DNA from each subject and analyzed the FXI gene using direct sequencing. We identified 5 and 11 individuals with severe and moderate deficiency of FXI activity, respectively. Most patients (8/16) carried mutations in the Apple 2 domain and 4 patients showed c.403G>T (p.Glu135*; type II mutation). Four novel compound heterozygosities were identified. Bleeding symptoms were present in two severely deficient subjects carrying the combinations c.901T>C (p.Phe301Leu)/c.1556G>A (p.Trp519*) and c.943G>A (p.Glu315)/c.1556G>A (p.Trp519*), respectively. Bleeding episodes were also observed in the presence of a moderate deficiency in two individuals heterozygous for c.449C>T (p.Thr150Met) and c.1253G>T (p.Gly418Val), respectively. One novel mutation, c.1682C>A (p.Ala561Asp), was identified as potentially deleterious in an asymptomatic individual. We confirm an unclear prediction of phenotype from mutational data. The FXI levels should be coupled with FXI analysis for a more comprehensive prediction of the bleeding phenotype in FXI deficiency.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1711
[Cu] Class update date: 171117
[Lr] Last revision date:171117
[St] Status:PubMed-not-MEDLINE
[do] DOI:10.1038/hgv.2017.43


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