Database : MEDLINE
Search on : Factor and XIII and Deficiency [Words]
References found : 1042 [refine]
Displaying: 1 .. 10   in format [Detailed]

page 1 of 105 go to page                         

  1 / 1042 MEDLINE  
              next record last record
select
to print
Photocopy
Full text

[PMID]: 29448295
[Au] Autor:Carcao M; Altisent C; Castaman G; Fukutake K; Kerlin BA; Kessler C; Lassila R; Nugent D; Oldenburg J; Garly ML; Rosholm A; Inbal A
[Ad] Address:Division of Haematology/Oncology and Child Health Evaluative Sciences, Research Institute, Hospital for Sick Children, University of Toronto, Toronto, Canada.
[Ti] Title:Recombinant FXIII (rFXIII-A2) Prophylaxis Prevents Bleeding and Allows for Surgery in Patients with Congenital FXIII A-Subunit Deficiency.
[So] Source:Thromb Haemost;, 2018 Feb 15.
[Is] ISSN:2567-689X
[Cp] Country of publication:Germany
[La] Language:eng
[Ab] Abstract:Recombinant factor XIII-A (rFXIII-A ) was developed for prophylaxis and treatment of bleeds in patients with congenital FXIII A-subunit deficiency. mentor™2 (NCT00978380), a multinational, open-label, single-arm, multiple-dosing extension to the pivotal mentor™1 trial, assessed long-term safety and efficacy of rFXIII-A prophylaxis in eligible patients (patients with severe [<0.05 IU/mL] congenital FXIII subunit A deficiency) aged ≥6 years. Patients received 35 IU/kg rFXIII-A (exact dosing) every 28 ± 2 days for ≥52 weeks. Primary endpoint was safety (adverse events including immunogenicity); secondary endpoints were rate of bleeds requiring FXIII treatment, haemostatic response after one 35 IU/kg rFXIII-A dose for breakthrough bleeds and withdrawals due to lack of rFXIII-A efficacy. Steady-state pharmacokinetic variables were also summarized. Elective surgery was permitted during the treatment period. Sixty patients were exposed to rFXIII-A ; their median age was 26.0 years (range: 7.0-77.0). rFXIII-A was well tolerated without any safety concerns. No non-neutralizing or neutralizing antibodies (inhibitors) against FXIII were detected. Mean annualized bleeding rate (ABR) was 0.043/patient-year. Mean spontaneous ABR was 0.011/patient-year. No patients withdrew due to lack of efficacy. Geometric mean FXIII trough level was 0.17 IU/mL. Geometric terminal half-life was 13.7 days. rFXIII-A prophylaxis provided sufficient haemostatic coverage for 12 minor surgeries without the need for additional FXIII therapy; eight procedures were performed within 7 days of the patient's last scheduled rFXIII-A dose, and four were performed 10 to 21 days after the last dose.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180301
[Lr] Last revision date:180301
[St] Status:Publisher
[do] DOI:10.1055/s-0038-1624581

  2 / 1042 MEDLINE  
              first record previous record next record last record
select
to print
Photocopy
Full text

[PMID]: 29476647
[Au] Autor:Tabibian S; Shams M; Naderi M; Dorgalaleh A
[Ad] Address:Department of Hematology and Blood Transfusion, School of Allied Medicine, Iran University of Medical Sciences, Tehran, Iran.
[Ti] Title:Prenatal diagnosis in rare bleeding disorders-An unresolved issue?
[So] Source:Int J Lab Hematol;, 2018 Feb 24.
[Is] ISSN:1751-553X
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Intracranial haemorrhage (ICH) is the most dreadful complication, and the main cause of death among patients with rare bleeding disorders (RBD) and prenatal diagnosis (PND) is a preventative lifesaving program. A total of 39 PNDs were reported in the literature through a search on PubMed, EMBASE, SCOPUS and Web of Science databases, most often for congenital factor (F) XIII and FVII deficiencies and rarely in FX, FV deficiencies and afibrinogenemia. The main cause to request a PND is ICH and related morbidity and mortality. Different molecular methods including direct sequencing and linkage analysis as well as polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) for a specific mutation are the most common used methods for PND, while factor assay and combination of molecular and factor assay also were used. In this research, 7 severely affected foetuses were identified during PND including 3 foetuses with FXIII deficiency, 3 with FVII deficiency and 1 with FX deficiency. Out of these 7 cases, intrauterine ICH occurred in 1 case with FXIII deficiency, 1 was electively aborted and 1 case with severe FVII deficiency received intrauterine factor transfusion. Postdelivery ICH was reported for 1 patient with severe FVII deficiency within the first month of life. All other pregnancies were uneventful.
[Pt] Publication type:JOURNAL ARTICLE; REVIEW
[Em] Entry month:1802
[Cu] Class update date: 180224
[Lr] Last revision date:180224
[St] Status:Publisher
[do] DOI:10.1111/ijlh.12789

  3 / 1042 MEDLINE  
              first record previous record next record last record
select
to print
Photocopy
Full text

[PMID]: 29460500
[Au] Autor:Muszbek L; Pénzes K; Katona É
[Ad] Address:Division of Clinical Laboratory Science Department of Laboratory Medicine, Faculty of Medicine, University of Debrecen, Debrecen, Hungary.
[Ti] Title:Auto-, and alloantibodies against factor XIII: laboratory diagnosis and clinical consequences.
[So] Source:J Thromb Haemost;, 2018 Feb 20.
[Is] ISSN:1538-7836
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Acquired FXIII deficiencies caused by autoantibodies against FXIII subunits represent rare but very severe bleeding diatheses. Alloantibodies in FXIII deficient patients also cause life-threatening bleeding complication, but they develop extremely rarely. In this review we provide an overview on the diagnosis and classification of anti-FXIII antibodies and analyze 48 patients with autoimmune FXIII deficiency and 4 additional FXIII deficient patients who developed anti-FXIII alloantibody. The patients were collected from peer-reviewed publications from which relevant data could be extracted. With the exception of two cases the antibodies were directed against FXIII-A. The difficulties in the diagnosis of FXIII deficiency in the presence of anti-FXIII antibodies are discussed and a scheme for the functional classification of the anti-FXIII antibodies is recommended. The three main categories are neutralizing and non-neutralizing antibodies and antibodies with combined effect. The methods being used for detecting and quantifying the inhibitory effect on FXIII activation and on the transglutaminase activity of activated FXIII are summarized and techniques for the classification of neutralizing anti-FXIII antibodies are outlined. The importance of clearance studies in these cases is emphasized. Binding assays, useful for the identification of non-neutralizing and combined type antibodies, were collected from literature and their informative power is demonstrated by examples. The most frequently occurring bleeding symptoms in patients with anti-FXIII antibodies were soft tissue bleeding, intracranial bleedings also occurred, but less frequently than in inherited FXIII deficiency. Treatment of such patients is extremely challenging, the main aim should be eradication of the antibody. This article is protected by copyright. All rights reserved.
[Pt] Publication type:JOURNAL ARTICLE; REVIEW
[Em] Entry month:1802
[Cu] Class update date: 180220
[Lr] Last revision date:180220
[St] Status:Publisher
[do] DOI:10.1111/jth.13982

  4 / 1042 MEDLINE  
              first record previous record next record last record
select
to print
Photocopy
Full text

[PMID]: 29446989
[Au] Autor:Haas T; Cushing MM; Asmis LM
[Ad] Address:a Department of Anaesthesia , Zurich University Children's Hospital , Zurich , Switzerland.
[Ti] Title:Comparison of the efficacy of two human fibrinogen concentrates to treat dilutional coagulopathy in vitro.
[So] Source:Scand J Clin Lab Invest;:1-6, 2018 Feb 15.
[Is] ISSN:1502-7686
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Both congenital and acquired fibrinogen deficiency can be safely treated with administration of fibrinogen concentrate. The aim of this study was to test the efficacy of a new fibrinogen product (Fibryga) compared to a licensed product (Haemocomplettan) in an in vitro model of dilutional coagulopathy. Ten blood specimens from healthy volunteers were diluted 1:1 with balanced crystalloid solution and subsequently supplemented with each fibrinogen concentrate at a dose replicating in vivo supplementation (50 mg kg ). Changes in clot firmness (FIBTEM and EXTEM assay), as well as changes in the fibrinogen antigen level, fibrinogen activity, factor XIII level and fibronectin levels were assessed at baseline, after dilution and after adding fibrinogen concentrate. There was no significant difference between the drugs in their in vitro ability to improve clot firmness in the FIBTEM assay (Fibryga: mean MCF 14.4 mm (SD 3.4 mm) vs. Haemocomplettan: MCF 14.1 mm (2.4); p = .584). Fibryga led to significantly higher clot firmness in EXTEM MCF: 56.7 mm (3.8) vs. 53.7 mm (3.7); p < .001). Distinct differences between FXIII levels (significantly higher in Fibryga; mean 40.9% (6.2%) vs. 31.0% (6.2%); p < .001) and fibronectin levels (significantly higher in Haemocomplettan; mean 0.008 g L (SD 0.002 g L ) vs. 0.002 g L (SD 0.002 g L ; p < .001) were observed between products. This is the first study to demonstrate that Fibryga and Haemocomplettan have similar efficacy in improving clot firmness in a dilutional hypofibrinogenemia model in vitro.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180215
[Lr] Last revision date:180215
[St] Status:Publisher
[do] DOI:10.1080/00365513.2018.1437645

  5 / 1042 MEDLINE  
              first record previous record next record last record
select
to print
Photocopy
Full text

[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

  6 / 1042 MEDLINE  
              first record previous record next record last record
select
to print
Photocopy
Full text

[PMID]: 29307277
[Au] Autor:Sun L; Yan Q; Wang Y; Luo H; Du P; Hassan R; Liu L; Jiang W
[Ad] Address:a Department of Medical Genetics, ZhongShan School of Medicine , Sun Yat-sen University , Guangzhou , People's Republic of China.
[Ti] Title:Pathogenicity analysis of variations and prenatal diagnosis in a hereditary coagulation factor XIII deficiency family.
[So] Source:Hematology;:1-9, 2018 Jan 07.
[Is] ISSN:1607-8454
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:OBJECTIVES: Prenatal diagnosis (PND) procedure is urgent to be established for timely management and fatal consequence prevention of factor XIII deficiency (FXIIID), and variations data among Chinese are very scanty. We aimed to find a novel mutation among Chinese and establish a rapid and precise PND procedure with pathogenicity analysis to contribute to the prevention of postpartum hemorrhage in pregnant women and central nervous system bleeding in newborns. METHODS: FXIIID was diagnosed by qualitative and quantitative tests of clot solubility test and enzyme-linked immunosorbent assay, respectively. Variations were detected by direct sequencing of F13A and F13B genes in the pedigree and the unborn fetus. Pathogenicity assessment of variations was based on American College of Medical Genetics and Genomics Guidelines. RESULTS: Ten variants in the F13A gene including a novel missense mutation in exon 10, a nonsense mutation in exon 4, a missense mutation in exon 12, 2 missense mutations in exon 14, 3 polymorphisms in intron 10, 2 polymorphisms in intron 14 were detected. Two variants in the F13B gene including a polymorphism in 3'UTR and a synonymous mutation were detected. The compound heterozygous mutations of the nonsense mutation and a novel missense mutation of the F13A gene caused the deficiency in proband, and the fetus which was evaluated to be unaffected by PND was born successfully and the results were verified by follow-up visits. DISCUSSION: We first established the PND procedure with pathogenicity assessment in FXIIID patients. The F13A gene mutations' spectrum of the Chinese Han population was enriched.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1801
[Cu] Class update date: 180108
[Lr] Last revision date:180108
[St] Status:Publisher
[do] DOI:10.1080/10245332.2017.1422315

  7 / 1042 MEDLINE  
              first record previous record next record last record
select
to print
Photocopy
Full text

[PMID]: 29269148
[Au] Autor:Carneiro JMGVM; Alves J; Conde P; Xambre F; Almeida E; Marques C; Luís M; Godinho AMMG; Fernandez-Llimos F
[Ad] Address:Centro Hospitalar de Lisboa Norte, Hospital de Santa Maria, Serviço de Anestesiologia, Lisboa, Portugal. Electronic address: joaoc17@hotmail.com.
[Ti] Title:Algoritmo de tratamento guiado pelo FXIII reduz a transfusão sanguínea na cirurgia de queimados. [FXIII-guided treatment algorithm reduces blood transfusion in burn surgery].
[So] Source:Rev Bras Anestesiol;, 2017 Dec 18.
[Is] ISSN:1806-907X
[Cp] Country of publication:Brazil
[La] Language:por
[Ab] Abstract:BACKGROUND AND OBJECTIVES: Major burn surgery causes large hemorrhage and coagulation dysfunction. Treatment algorithms guided by ROTEM and FVIIa reduce the need for blood products, but there is no evidence regarding factor XIII. Factor XIII deficiency changes clot stability and decreases wound healing. This study evaluates the efficacy and safety of factor XIII correction and its repercussion on transfusion requirements in burn surgery. METHODS: Randomized retrospective study with 40 patients undergoing surgery at the Burn Unit, divided into Group A, those with factor XIII assessment (n = 20), and Group B, those without assessment (n = 20). Erythrocyte transfusion was guided by a hemoglobin trigger of 10g.dL and the other blood products were by routine coagulation and ROTEM tests. Analysis of blood product consumption included units of erythrocytes, fresh frozen plasma, platelets, and fibrinogen. The coagulation biomarker analysis compared the pre- and post-operative values. RESULTS AND CONCLUSIONS: Group A (with factor XIII study) and Group B had identical total body surface area burned. All patients in Group A had a preoperative factor XIII deficiency, whose correction significantly reduced units of erythrocyte concentrate transfusion (1.95 vs. 4.05, p = 0.001). Pre- and post-operative coagulation biomarkers were similar between groups, revealing that routine coagulation tests did not identify factor XIII deficiency. There were no recorded thromboembolic events. Correction of factor XIII deficiency in burn surgery proved to be safe and effective for reducing perioperative transfusion of erythrocyte units.
[Pt] Publication type:ENGLISH ABSTRACT; JOURNAL ARTICLE
[Em] Entry month:1712
[Cu] Class update date: 171222
[Lr] Last revision date:171222
[St] Status:Publisher

  8 / 1042 MEDLINE  
              first record previous record next record last record
select
to print
Photocopy
Full text

[PMID]: 29188749
[Au] Autor:Hellmann C; Schmutz A; Kalbhenn J
[Ad] Address:Department of Anesthesiology and Critical Care, Medical Center - Faculty of Medicine, University of Freiburg, Freiburg, Germany.
[Ti] Title:Bleeding during veno-venous ECMO cannot reliably be predicted by rotational thrombelastometry (ROTEM™).
[So] Source:Perfusion;:267659117746231, 2017 Nov 01.
[Is] ISSN:1477-111X
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:INTRODUCTION: Veno-venous extracorporeal membrane oxygenation (vvECMO) used for respiratory support is associated with clinical bleeding in at least one third of patients. Mechanisms promoting bleeding, like acquired von Willebrand syndrome, cannot be identified by routine coagulation tests. This study was performed to evaluate rotational Thrombelastography (ROTEM™) for specific results predicting bleeding events during vvECMO. METHODS: Five hundred and thirty-four ROTEM™ analyses of 57 patients over 574 days have been evaluated. Patients were graded into three groups according to the severity of bleeding, following the Freiburg ECMO bleeding assessment. ROTEM™ results and basic as well as comprehensive laboratory coagulation tests have been compared among the three groups and overall between defined time points. RESULTS: Fourteen patients (25 %) presented without bleeding, 22 patients (39 %) showed mild bleeding and 21 patients (36 %) became evident with relevant clinical bleeding. No bleeding shock and no fatal bleeding event occurred. No case of hyperfibrinolysis was observed. Neither a statistical difference for ECMO blood flow nor duration of therapy among the groups could be shown. The only significant difference was clotting time (CT) in the InTEM analysis, with a median (IQR) of 175 (37.5) seconds in Group 1, 190 (54.5) seconds in Group 2 and 204 (90) seconds in Group 3. When comparing overall ROTEM™ analyses between defined time points, continuous worsening of CT can be found in ExTEM, FibTEM and ApTEM. Reduced A10, A20 and congruently maximum clot firmness, especially, developed in ExTEM and ApTEM and with a minor characteristic in InTEM, but not in FibTEM. ROTEM™ and coagulation-parameter results before 19 clinical relevant bleeding episodes compared to all other results only showed differences in FibTEM. CONCLUSION: ROTEM™ as a functional viscoelastic analysis does not provide additional information to basic and comprehensive laboratory tests during vvECMO. Bleeding events cannot be predicted by the means of specific ROTEM™ results.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1712
[Cu] Class update date: 171130
[Lr] Last revision date:171130
[St] Status:Publisher
[do] DOI:10.1177/0267659117746231

  9 / 1042 MEDLINE  
              first record previous record next record last record
select
to print
Photocopy
Full text

[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 / 1042 MEDLINE  
              first record previous record
select
to print
Photocopy
Full text

[PMID]: 29095761
[Au] Autor:Gheidishahran M; Dorgalaleh A; Tabibian S; Shams M; Sanei Moghaddam E; Khosravi S; Naderi M; Kahraze S; Lotfi F; Kazeme A; Safa M
[Ad] Address:aDepartment of Hematology and Blood Transfusion, School of Allied Medicine, Iran University of Medical Sciences, Tehran bBlood Transfusion Research Center, High Institute for Research and Education in Transfusion Medicine cGenetic Research Center in Non-Communicable Disease, Zahedan University of Medical Sciences, Zahedan, Iran.
[Ti] Title:Molecular diagnosis of factor XIII deficiency, data from comprehensive coagulation laboratory in Iran.
[So] Source:Blood Coagul Fibrinolysis;, 2017 Nov 01.
[Is] ISSN:1473-5733
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:: Diagnosis of factor XIII (FXIII) deficiency (FXIIID) as a rare bleeding disorder is a challenge worldwide. Thus, in the present study, we used different methods including two molecular methods for detection of FXIIID. This study was conducted on individuals suspected to FXIIID. All individuals were checked by two routinely used methods of clot solubility test in Iran and two other clot solubility tests as well as FXIII activity and antigen assays. Molecular analysis was performed by PCR-restriction fragment length polymorphism (PCR-RFLP) and tetra-primer amplification refractory mutation system (T-ARMS)-PCR for only FXIIID mutation in southeast Iran (p.Trp187Arg), previously associated with severe FXIIID. Out of 151 individuals, 26 had abnormal clot solubility test with all four methods. PCR-RFLP revealed that 27 patients were homozygotes for p.Trp187Arg, whereas 12 were heterozygotes. Molecular analysis revealed that in routinely used clot solubility combinations, two homozygotes (∼8%) were missed, whereas in two other combinations, one patient (∼4%) was missed. One false positive result was observed in routinely used methods, whereas further combinations don't have false positive. T-ARMS-PCR had three discrepancies with PCR-RFLP and sequencing confirmed that the results of T-ARMS-PCR were false. FXIII antigen assay diagnosed all homozygotes, whereas in FXIII activity assay, two homozygotes had higher than 5% FXIII activity that inconsistent with severe deficiency. It seems that clot solubility test is not enough sensitive and specific and molecular analysis is the most reliable method for detection of FXIIID in areas such Iran with one or few specific mutations.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1711
[Cu] Class update date: 171102
[Lr] Last revision date:171102
[St] Status:Publisher
[do] DOI:10.1097/MBC.0000000000000679


page 1 of 105 go to page                         
   


Refine the search
  Database : MEDLINE Advanced form   

    Search in field  
1  
2
3
 
           



Search engine: iAH v2.6 powered by WWWISIS

BIREME/PAHO/WHO - Latin American and Caribbean Center on Health Sciences Information