Database : MEDLINE
Search on : Afibrinogenemia [Words]
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  1 / 1619 MEDLINE  
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[PMID]: 29194665
[Au] Autor:Lissitchkov T; Madan B; Djambas Khayat C; Zozulya N; Ross C; Karimi M; Kavakli K; De Angulo GR; Almomen A; Schwartz BA; Solomon C; Knaub S; Peyvandi F
[Ad] Address:Department of Hemorrhagic Diathesis and Anemia, Specialized Hospital for Active Treatment (SHAT) "Joan Pavel,", Sofia, Bulgaria.
[Ti] Title:Efficacy and safety of a new human fibrinogen concentrate in patients with congenital fibrinogen deficiency: an interim analysis of a Phase III trial.
[So] Source:Transfusion;58(2):413-422, 2018 Feb.
[Is] ISSN:1537-2995
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:BACKGROUND: Fibrinogen concentrate is the preferred choice for fibrinogen replacement in congenital fibrinogen deficiency. This study investigated hemostatic efficacy of a new plasma-derived, double virus-inactivated (using two dedicated virus inactivation/elimination steps) human fibrinogen concentrate for on-demand treatment of bleeding episodes (BEs) and surgical prophylaxis. STUDY DESIGN AND METHODS: In this planned interim analysis of a prospective, multinational Phase III study (NCT02267226), 13 patients with afibrinogenemia (≥12 years) received fibrinogen concentrate (FIBRYGA, Octapharma AG). Hemostatic efficacy was assessed by investigators and an independent data monitoring and endpoint adjudication committee (IDMEAC) using objective four-point criteria and by thromboelastometry maximum clot firmness (MCF). RESULTS: Fibrinogen concentrate was used on-demand to treat 23 BEs in 11 patients, with 21 (91.3%) requiring a single infusion only. Treatment success was 95.7% (90% confidence interval [CI], 0.81-1.00; assessment missing for one BE) by investigators and 100% (90% CI, 0.88-1.00) by IDMEAC. Mean MCF increased significantly from 0.0 to 6.5 mm (95% CI, 5.65-7.40; p < 0.0001) at 1 hour postinfusion of a median (range) dose of 58.8 (33.9-101.7) mg/kg per BE. Four patients received fibrinogen concentrate as surgical prophylaxis, with intraoperative and postoperative treatment success rated 100% (90% CI, 0.50-1.00) by investigators and IDMEAC (median [range] dose per surgery 93.5 [34.1-225.4] mg/kg). No additional hemostatic interventions were required. No deaths, thromboses, or seroconversions were reported. CONCLUSION: These data showed that the new fibrinogen concentrate was efficacious for on-demand treatment of acute bleeding and surgical prophylaxis in congenital afibrinogenemia patients.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1712
[Cu] Class update date: 180228
[Lr] Last revision date:180228
[St] Status:In-Data-Review
[do] DOI:10.1111/trf.14421

  2 / 1619 MEDLINE  
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[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 / 1619 MEDLINE  
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[PMID]: 29316703
[Au] Autor:Neerman-Arbez M; Casini A
[Ad] Address:Department of Genetic Medicine and Development, Faculty of Medicine, University of Geneva, 1211 Geneva, Switzerland. marguerite.neerman-arbez@unige.ch.
[Ti] Title:Clinical Consequences and Molecular Bases of Low Fibrinogen Levels.
[So] Source:Int J Mol Sci;19(1), 2018 Jan 08.
[Is] ISSN:1422-0067
[Cp] Country of publication:Switzerland
[La] Language:eng
[Ab] Abstract:The study of inherited fibrinogen disorders, characterized by extensive allelic heterogeneity, allows the association of defined mutations with specific defects providing significant insight into the location of functionally important sites in fibrinogen and fibrin. Since the identification of the first causative mutation for congenital afibrinogenemia, studies have elucidated the underlying molecular pathophysiology of numerous causative mutations leading to fibrinogen deficiency, developed cell-based and animal models to study human fibrinogen disorders, and further explored the clinical consequences of absent, low, or dysfunctional fibrinogen. Since qualitative disorders are addressed by another review in this special issue, this review will focus on quantitative disorders and will discuss their diagnosis, clinical features, molecular bases, and introduce new models to study the phenotypic consequences of fibrinogen deficiency.
[Pt] Publication type:JOURNAL ARTICLE; REVIEW
[Em] Entry month:1801
[Cu] Class update date: 180213
[Lr] Last revision date:180213
[St] Status:In-Process

  4 / 1619 MEDLINE  
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[PMID]: 29286337
[Au] Autor:Simurda T; Zolkova J; Snahnicanova Z; Loderer D; Skornova I; Sokol J; Hudecek J; Stasko J; Lasabova Z; Kubisz P
[Ad] Address:National Centre of Haemostasis and Thrombosis, Department of Haematology and Transfusiology, Comenius University in Bratislava, Jessenius Faculty of Medicine and University Hospital in Martin, Kollarova Str. N. 2, Martin 036 59, Slovakia. tsimurda@orava.sk.
[Ti] Title:Identification of Two Novel Fibrinogen Bß Chain Mutations in Two Slovak Families with Quantitative Fibrinogen Disorders.
[So] Source:Int J Mol Sci;19(1), 2017 Dec 29.
[Is] ISSN:1422-0067
[Cp] Country of publication:Switzerland
[La] Language:eng
[Ab] Abstract:Congenital fibrinogen disorders are caused by mutations in one of the three fibrinogen genes that affect the synthesis, assembly, intracellular processing, stability or secretion of fibrinogen. Functional studies of mutant Bß-chains revealed the importance of individual residues as well as three-dimensional structures for fibrinogen assembly and secretion. This study describes two novel homozygous fibrinogen Bß chain mutations in two Slovak families with afibrinogenemia and hypofibrinogenemia. Peripheral blood samples were collected from all subjects with the aim of identifying the causative mutation. Coagulation-related tests and rotational thromboelastometry were performed. All exons and exon-intron boundaries of the fibrinogen genes ( , and ) were amplified by PCR followed by direct sequencing. Sequence analysis of the three fibrinogen genes allowed us to identify two novel homozygous mutations in the gene. A novel Bß chain truncation (BßGln180Stop) was detected in a 28-year-old afibrinogenemic man with bleeding episodes including repeated haemorrhaging into muscles, joints, and soft tissues, and mucocutaneous bleeding and a novel Bß missense mutation (BßTyr368His) was found in a 62-year-old hypofibrinogenemic man with recurrent deep and superficial venous thromboses of the lower extremities. The novel missense mutation was confirmed by molecular modelling. Both studying the molecular anomalies and the modelling of fibrinogenic mutants help us to understand the extremely complex machinery of fibrinogen biosynthesis and finally better assess its correlation with the patient's clinical course.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1712
[Cu] Class update date: 180213
[Lr] Last revision date:180213
[St] Status:In-Process

  5 / 1619 MEDLINE  
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[PMID]: 29220876
[Au] Autor:Ross C; Rangarajan S; Karimi M; Toogeh G; Apte S; Lissitchkov T; Acharya S; Manco-Johnson MJ; Srivastava A; Brand B; Schwartz BA; Knaub S; Peyvandi F
[Ad] Address:Department of Hematology, St John's Medical College and Hospital, Bangalore, India.
[Ti] Title:Pharmacokinetics, clot strength and safety of a new fibrinogen concentrate: randomized comparison with active control in congenital fibrinogen deficiency.
[So] Source:J Thromb Haemost;16(2):253-261, 2018 Feb.
[Is] ISSN:1538-7836
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Essentials Congenital afibrinogenemia causes a potentially life-threatening bleeding and clotting tendency. Two human fibrinogen concentrates (HFCs) were compared in a randomized pharmacokinetic study. Bioequivalence was not shown for AUC , which was significantly larger for the new HFC. Increases in clot strength were comparable, and no thromboses or deaths occurred in the study. SUMMARY: Background Human fibrinogen concentrate (HFC) corrects fibrinogen deficiency in congenital a-/hypofibrinogenemia. Objectives To assess pharmacokinetics (PK), effects on thromboelastometry maximum clot firmness (MCF), and safety of a new double virus-inactivated/eliminated, highly purified HFC vs. active control. Patients/Methods In this multinational, randomized, phase II, open-label, crossover study in 22 congenital afibrinogenemia patients aged ≥ 12 years, 70 mg kg of new HFC (FIBRYGA, Octapharma AG) or control (Haemocomplettan P/RiaSTAP™, CSL Behring GmbH) were administered, followed by crossover to the other concentrate. Fibrinogen activity, PK and MCF in plasma were assessed. Results The concentrates were not bioequivalent for the primary endpoint, AUC (mean ratio, 1.196; 90% confidence interval [CI], 1.117, 1.281). Remaining PK parameters (C , IVR, t , MRT) reflected bioequivalence between concentrates, except for clearance (mean ratio, 0.836; 90% CI, 0.781, 0.895) and V (mean ratio, 0.886; 90% CI, 0.791, 0.994). Mean AUC was significantly larger for the new HFC (1.62 ± 0.45 vs. 1.38 ± 0.47 h kg g L  mg , P = 0.0001) and mean clearance was significantly slower (0.665 ± 0.197 vs. 0.804 ± 0.255 mL h  kg , P = 0.0002). Mean MCF increased from 0 mm to 9.68 mm (new HFC) and 10.00 mm (control) 1-hour post-infusion (mean difference, -0.32 mm; 95% CI, -1.70, 1.07, n.s.). No deaths, thromboses, viral seroconversions or serious related adverse events occurred. Conclusions Bioequivalence was not demonstrated for AUC , clearance and V . Larger AUC and slower clearance were observed for the new HFC. Remaining pharmacokinetic parameters reflected bioequivalence to control. Safety profiles and increases in clot strength were comparable between concentrates.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1712
[Cu] Class update date: 180212
[Lr] Last revision date:180212
[St] Status:In-Data-Review
[do] DOI:10.1111/jth.13923

  6 / 1619 MEDLINE  
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[PMID]: 29388741
[Au] Autor:Karimi M; Bordbar M; Aali M; Bazrafshan A; Tavoosi H; Gerdabi J
[Ad] Address:Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
[Ti] Title:Successful delivery in an patient with afibrinogenemia after three abortions: A case report and review of the literature.
[So] Source:Haemophilia;, 2018 Feb 01.
[Is] ISSN:1365-2516
[Cp] Country of publication:England
[La] Language:eng
[Pt] Publication type:LETTER
[Em] Entry month:1802
[Cu] Class update date: 180201
[Lr] Last revision date:180201
[St] Status:Publisher
[do] DOI:10.1111/hae.13415

  7 / 1619 MEDLINE  
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[PMID]: 29351094
[Au] Autor:Wang Y; Chen W; Ma P; Zhu L; Wang M
[Ad] Address:Department of Clinical Laboratory, The Affiliated Hospital of Xuzhou Medical University, Xuzhou.
[Ti] Title:Clinical and molecular characterization of nine Chinese patients affected by hypofibrinogenemia or dysfibrinogenemia.
[So] Source:Blood Coagul Fibrinolysis;, 2018 Jan 17.
[Is] ISSN:1473-5733
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:: Congential fibrinogen deficiency is a rare bleeding disorder caused by various mutations in three fibrinogen genes. It can be subdivided into four categories: afibrinogenemia, hypofibrinogenemia, hypodysfibrinogenemia and dysfbrinogenemia. This study was to elucidate the molecular defects in nine unrelated Chinese patients with hypofibrinogenemia or dysfibrinogenemia. Three fibrinogen genes were amplified by PCR and screened for variants. The identified variants were analyzed by bioinformatics prediction and molecular modeling analysis. Genetic screening disclosed seven different missense mutations, four of which were novel. All of the mutations were expected to impair the protein function/structure as assessed by bioinformatics prediction. This study has increased our knowledge of the mutational spectrum underlying fibrinogen deficiency.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1801
[Cu] Class update date: 180119
[Lr] Last revision date:180119
[St] Status:Publisher
[do] DOI:10.1097/MBC.0000000000000699

  8 / 1619 MEDLINE  
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[PMID]: 29240685
[Au] Autor:Paraboschi EM; Duga S; Asselta R
[Ad] Address:Department of Biomedical Sciences, Humanitas University, Via Rita Levi Montalcini 4, 20090 Pieve Emanuele, Milan, Italy. elvezia_maria.paraboschi@hunimed.eu.
[Ti] Title:Fibrinogen as a Pleiotropic Protein Causing Human Diseases: The Mutational Burden of Aα, Bß, and γ Chains.
[So] Source:Int J Mol Sci;18(12), 2017 Dec 14.
[Is] ISSN:1422-0067
[Cp] Country of publication:Switzerland
[La] Language:eng
[Ab] Abstract:Fibrinogen is a highly pleiotropic protein that is involved in the final step of the coagulation cascade, wound healing, inflammation, and angiogenesis. Heterozygous mutations in Aα, Bß, or γ fibrinogen-chain genes ( , , ) have been described as being responsible for fibrinogen deficiencies (hypofibrinogenemia, hypo-dysfibrinogenemia, dysfibrinogenemia) and for more rare conditions, such as fibrinogen storage disease and hereditary renal amyloidosis. Instead, biallelic mutations have been associated with afibrinogenemia/severe hypofibrinogenemia, i.e., the severest forms of fibrinogen deficiency, affecting approximately 1-2 cases per million people. However, the "true" prevalence for these conditions on a global scale is currently not available. Here, we defined the mutational burden of the , , and genes, and estimated the prevalence of inherited fibrinogen disorders through a systematic analysis of exome/genome data from ~140,000 individuals belonging to the genome Aggregation Database. Our analysis showed that the world-wide prevalence for recessively-inherited fibrinogen deficiencies could be 10-fold higher than that reported so far (prevalence rates vary from 1 in 106 in East Asians to 24.5 in 106 in non-Finnish Europeans). The global prevalence for autosomal-dominant fibrinogen disorders was estimated to be ~11 in 1000 individuals, with heterozygous carriers present at a frequency varying from 3 every 1000 individuals in Finns, to 1-2 every 100 individuals among non-Finnish Europeans and Africans/African Americans. Our analysis also allowed for the identification of recurrent (i.e., -p.Ala108Gly, -Thr47Ile) or ethnic-specific mutations (e.g., -p.Gly103Arg in Admixed Americans, -p.Ser245Phe in Africans/African Americans).
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1712
[Cu] Class update date: 180109
[Lr] Last revision date:180109
[St] Status:In-Process

  9 / 1619 MEDLINE  
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[PMID]: 29245350
[Au] Autor:Wu X; Zhao P; Dong L; Zhang X
[Ad] Address:aDepartment of Pharmacy, Ruijin Hospital Suzhou Branch Affiliated to Shanghai Jiaotong University School of MedicinebDepartment of Pharmacy, Jiangsu Shengze Hospital, SuzhoucDepartment of Critical Care MedicinedDepartment of Pharmacy, Wuxi People's Hospital Affiliated to Nanjing Medical University, Wuxi, PR China.
[Ti] Title:A case report of patient with severe acute cholangitis with tigecycline treatment causing coagulopathy and hypofibrinogenemia.
[So] Source:Medicine (Baltimore);96(49):e9124, 2017 Dec.
[Is] ISSN:1536-5964
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:RATIONALE: Tigecycline is the first member of the glycylcycline family. There are rarely reports of tigecycline causing coagulopathy and hypofibrinogenemia until now. We report a case on tigecycline-associated coagulopathy and hypofibrinogenemia and discuss the characteristics of the adverse reaction. PATIENT CONCERNS: A 47-year-old male patient with severe acute cholangitis who developed sepsis was treated with a high dosage (100 mg twice daily) of tigecycline. He experienced coagulopathy and hypofibrinogenemia as substantiated by increased levels of prolonged prothrombin time (PT), the international normalized ratio (INR) and activated partial thromboplastin time (APTT), and in particular, the fibrinogen (FIB) levels obviously decreased. DIAGNOSES: Coagulopathy and hypofibrinogenemia. INTERVENTIONS: We discontinued tigecycline and gave the patient several blood products to prevent spontaneous bleeding. OUTCOMES: The adverse reaction disappeared after the withdrawal of tigecycline. After 30 days of hospitalization, the patient discharged with symptom free. LESSONS: We suggest that coagulation parameters should be closely monitored in patients treated with tigecycline, specifically in patients who may be renal insufficiency, female or use the high-dose.
[Mh] MeSH terms primary: Anti-Bacterial Agents/adverse effects
Blood Coagulation Disorders/chemically induced
Minocycline/analogs & derivatives
[Mh] MeSH terms secundary: Afibrinogenemia/chemically induced
Anti-Bacterial Agents/therapeutic use
Cholangitis/complications
Humans
Male
Middle Aged
Minocycline/adverse effects
Minocycline/therapeutic use
Sepsis/drug therapy
Sepsis/etiology
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Nm] Name of substance:0 (Anti-Bacterial Agents); 70JE2N95KR (tigecycline); FYY3R43WGO (Minocycline)
[Em] Entry month:1801
[Cu] Class update date: 180105
[Lr] Last revision date:180105
[Js] Journal subset:AIM; IM
[Da] Date of entry for processing:171217
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000009124

  10 / 1619 MEDLINE  
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[PMID]: 28952920
[Au] Autor:Milani S; Aliakbarian M; Amouian S
[Ad] Address:From the Surgical Oncology Research Center, Mashhad University of Medical Sciences, Mashhad, Iran.
[Ti] Title:Afibrinogenemia Acquired by Liver Transplant.
[So] Source:Exp Clin Transplant;, 2017 Sep 26.
[Is] ISSN:2146-8427
[Cp] Country of publication:Turkey
[La] Language:eng
[Ab] Abstract:Liver transplant is a life-saving procedure in patients with end-stage liver disease. However, this procedure may be associated with transmission of various deficiencies of proteins synthesized by the liver. Factor I (fibrinogen) deficiency is one of the rare inherited coagulation disorders with an extremely low risk of transmission by liver transplant. We report a case of a patient with no inherited coagulation disorders but who demonstrated disturbance of fibrinogen after liver transplant. This case highlights the ever-present risk of donor-to-recipient disease transmission during transplant and emphasizes the difficulty in procuring organs from donors in which standard blood tests are insufficient to determine the likelihood of this event.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1709
[Cu] Class update date: 170927
[Lr] Last revision date:170927
[St] Status:Publisher
[do] DOI:10.6002/ect.2016.0338


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