1,332 results match your criteria Factor XI Deficiency


Low endoscopy bleeding risk in patients with congenital bleeding disorders.

Haemophilia 2019 Feb 12. Epub 2019 Feb 12.

Division of Gastroenterology, McGill University Health Centre, Montreal, Quebec, Canada.

Introduction: Haemophilia A and haemophilia B, von Willebrand disease (VWD), factor VII deficiency and factor XI deficiency are congenital bleeding disorders predisposing to bleeding during invasive procedures. The ageing population of people with congenital bleeding disorders will likely increasingly require gastrointestinal endoscopy. The bleeding risk postgastrointestinal endoscopy and optimal prophylactic treatment regimens are not well described. Read More

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http://doi.wiley.com/10.1111/hae.13691
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http://dx.doi.org/10.1111/hae.13691DOI Listing
February 2019
11 Reads

A case of a severe factor XI deficiency in a Chinese woman with heavy menorrhagia.

Blood Coagul Fibrinolysis 2019 Mar;30(2):75-79

Department of Clinical Laboratory, The Second Affiliated Hospital of Wenzhou Medical University, Wenzhou, China.

: The current study was to elucidate the molecular defect in a 32-year-old Chinese woman with heavy menorrhagia and delayed wound healing. The F11 gene was amplified by PCR and screened for mutations. Then identified mutations were analyzed by in-silico programs and molecular modeling analysis. Read More

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http://Insights.ovid.com/crossref?an=00001721-201903000-0000
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http://dx.doi.org/10.1097/MBC.0000000000000795DOI Listing
March 2019
6 Reads

How to Optimize Activated Partial Thromboplastin Time (APTT) Testing: Solutions to Establishing and Verifying Normal Reference Intervals and Assessing APTT Reagents for Sensitivity to Heparin, Lupus Anticoagulant, and Clotting Factors.

Semin Thromb Hemost 2019 Feb 10;45(1):22-35. Epub 2019 Jan 10.

Department of Haematology, NSW Health Pathology, Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia.

The activated partial thromboplastin time (APTT) assay is a very common coagulation test, used for several reasons. The test is conventionally used for assessing the contact factor (intrinsic) pathway of blood coagulation, and thus for screening deficiencies in this pathway, most typically factors VIII, IX, and XI. The APTT is also sensitive to contact factor deficiencies, including factor XII, prekallikrein, and high-molecular-weight kininogen. Read More

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http://dx.doi.org/10.1055/s-0038-1677018DOI Listing
February 2019
6 Reads

Oral surgery for combined haemophilia A and B. The dos and don'ts presented in a clinical scenario.

Ann Ital Chir 2018 ;89:374-378

Background: Haemophilia A (factor VIII deficiency), B (factor IX deficiency) and C (factor XI deficiency) are common genetic bleeding disorders. Most often they are caused by the absence or defective function of coagulation factors, causing inefficient blood clots.

Case Report: The present manuscript describes a rare case of a combined haemophilia A and B patient, who underwent several extractions. Read More

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January 2018
5 Reads

FranceCoag: a 22-year prospective follow-up of the national French cohort of patients with inherited bleeding disorders.

Eur J Epidemiol 2018 Dec 5. Epub 2018 Dec 5.

Haemophilia Treatment Centre, APHM, Children Hospital La Timone, Aix Marseille University, INSERM, INRA, C2VN, Marseille, France.

FranceCoag is an ongoing open prospective multicentre cohort project aimed at improving epidemiological knowledge about inherited bleeding disorders in France. The main objective of this article was to evaluate the project's progress as of the 30th December 2016. Between 1994 and this date, of the 10,047 patients included in the study, 384 (3. Read More

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http://link.springer.com/10.1007/s10654-018-0468-7
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http://dx.doi.org/10.1007/s10654-018-0468-7DOI Listing
December 2018
8 Reads

[Phenotypic and genetic analysis of two pedigrees affected with hereditary coagulation FXII deficiency].

Zhonghua Yi Xue Yi Chuan Xue Za Zhi 2018 Dec;35(6):800-803

Department of Laboratory Medicine, the Second Affiliated Hospital, Wenzhou Medical University, Wenzhou, Zhejiang 325027, China.

Objective: To carry out phenotypic and genotypic analysis for two Chinese pedigrees affected with coagulation factor XII (F XII) deficiency.

Methods: Plasma prothrombin time (PT), activated partial thromboplastin time (APTT), fibrinogen (FIB), thrombin time (TT), and blood coagulation factor VIII, IX, XI, XII activity (FVIII:C, FIX:C, FXI:C, FXII:C) were determined with one stage clotting assay on a STAGO coagulation analyzer. FXII antigen was determined with an enzyme linked immunosorbent assay (ELISA). Read More

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http://dx.doi.org/10.3760/cma.j.issn.1003-9406.2018.06.005DOI Listing
December 2018
2 Reads

The unusual pattern of hereditary bleeding disorders in the province of Newfoundland and Labrador-Canada's most Eastern Province.

Transfus Apher Sci 2018 Dec 30;57(6):713-716. Epub 2018 Oct 30.

Memorial University of Newfoundland Primary Healthcare Research Unit, Health Sciences Centre, 300 Prince Philip Dr., St. John's, NL A1B 3 V6, Canada. Electronic address:

Newfoundland and Labrador (NL), the most eastern province of Canada, is characterized by a unique topography and pattern of settlement. The current population is descended from a small founding population of indigenous Innu, Inuit and Mi'kmaq and an estimated 28,000 settlers. These settlers originated from Southwest England and Southeast Ireland and came to invest and work in one of the world's richest fisheries. Read More

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http://dx.doi.org/10.1016/j.transci.2018.10.010DOI Listing
December 2018
14 Reads

Emicizumab, the bispecific antibody to factors IX/IXa and X/Xa, potentiates coagulation function in factor XI-deficient plasma in vitro.

J Thromb Haemost 2019 Jan 10;17(1):126-137. Epub 2018 Dec 10.

Department of Pediatrics, Nara Medical University, Kashihara, Nara, Japan.

Essentials Emicizumab mimics factor (F)VIIIa cofactor function, augments the intrinsic tenase activity. We assessed the emicizumab-driven hemostatic function in FXI-deficient plasmas. Emicizumab improved the coagulation potentials in severe FXI-deficient plasma. Read More

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http://doi.wiley.com/10.1111/jth.14334
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http://dx.doi.org/10.1111/jth.14334DOI Listing
January 2019
15 Reads

Orthosilicic Acid Accelerates Bone Formation in Human Osteoblast-Like Cells Through the PI3K-Akt-mTOR Pathway.

Biol Trace Elem Res 2018 Nov 12. Epub 2018 Nov 12.

Department of Orthopedics, Qilu Hospital of Shandong University and Spine and Spinal Cord Disease Research Center, Shandong University, Jinan, Shandong, China.

Silicon is one of the essential trace elements in the human body; the deficiency of which may lead to bone diseases. Numerous animal experiments have shown that an appropriate increase in the intake of silicon is beneficial to enhancing bone density and toughness to prevent osteoporosis. However, the molecular mechanisms of the silicon-mediated osteogenesis process have not been sufficiently clarified. Read More

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http://link.springer.com/10.1007/s12011-018-1574-9
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http://dx.doi.org/10.1007/s12011-018-1574-9DOI Listing
November 2018
21 Reads

A Novel Rhein Derivative Modulates Bone Formation and Resorption and Ameliorates Estrogen-Dependent Bone Loss.

J Bone Miner Res 2019 Feb 5;34(2):361-374. Epub 2018 Nov 5.

Shanghai Key Laboratory for Bone and Joint Diseases, Shanghai Institute of Traumatology and Orthopaedics, Shanghai Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China.

Osteoporosis, an osteolytic disease that affects millions of people worldwide, features a bone remodeling imbalance between bone resorption by osteoclasts and bone formation by osteoblasts. Identifying dual target-directed agents that inhibit excessive bone resorption and increase bone formation is considered an efficient strategy for developing new osteoporosis treatments. Rhein, a natural anthraquinone, can be isolated from various Asian herbal medicines. Read More

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http://doi.wiley.com/10.1002/jbmr.3604
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http://dx.doi.org/10.1002/jbmr.3604DOI Listing
February 2019
14 Reads
6.832 Impact Factor

Factor XI Deficiency: The Key Is Individualization.

Anesth Analg 2019 Jan;128(1):e10-e9

Departments of Anesthesiology, Perioperative and Pain Medicine and Obstetrics, and Gynecology & Reproductive Sciences, Icahn School of Medicine at Mount Sinai, New York, New York,

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http://dx.doi.org/10.1213/ANE.0000000000003869DOI Listing
January 2019
14 Reads

Paired APTTs of low and high lupus anticoagulant sensitivity permit distinction from other abnormalities and achieve good lupus anticoagulant detection rates in conjunction with dRVVT.

Int J Lab Hematol 2019 Feb 24;41(1):60-68. Epub 2018 Sep 24.

Department of Haemostasis and Thrombosis, Viapath Analytics, Guy's & St Thomas' Hospitals, London, UK.

Introduction: A prolonged activated partial thromboplastin time (APTT) may be indicative of a specific or multiple factor deficiency, therapeutic anticoagulation, presence of a nonspecific factor inhibitor, or lupus anticoagulant (LA). Recently, pairing of the LA-sensitive APTT and standard APTT reagents, Cephen LS and Cephen, respectively, has been shown to be effective in LA detection. The present study aimed to evaluate the usefulness of this reagent pair for discriminating between causes of APTT elevation and the detection of LA in conjunction with dilute Russell's viper venom time (dRVVT). Read More

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http://dx.doi.org/10.1111/ijlh.12921DOI Listing
February 2019
10 Reads

Practical aspects of extended half-life products for the treatment of haemophilia.

Ther Adv Hematol 2018 Sep 6;9(9):295-308. Epub 2018 Sep 6.

Angelo Bianchi Bonomi Haemophilia and Thrombosis Centre, Maggiore Hospital Policlinic, Milan, Italy.

Haemophilia A and haemophilia B are congenital X-linked bleeding disorders caused by deficiency of coagulation factor VIII (FVIII) and IX (FIX), respectively. The preferred treatment option for patients with haemophilia is replacement therapy. For patients with severe disease, prophylactic replacement of coagulation factor is the treatment of choice; this has been shown to reduce arthropathy significantly, reduce the frequency of bleeds and improve patients' quality of life. Read More

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http://dx.doi.org/10.1177/2040620718796429DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6130100PMC
September 2018
38 Reads

[Identification of compound heterozygous mutations p.Gly400Val and p.Arg532Ter of the F11 gene in a Chinese patient with hereditary factor XI deficiency].

Zhonghua Yi Xue Yi Chuan Xue Za Zhi 2018 Aug;35(4):522-526

Department of Laboratory Medicine, the Second Affiliated Hospital and Yuying Children's Hospital of Wenzhou Medical University, Wenzhou, Zhejiang 325027, China.

Objective: To investigate the phenotype and genotype defect characteristics of a Chinese patient with hereditary factor XI deficiency.

Methods: The activated partial thromboplastin time (APTT), prothrombin time (PT), FXI activity (FXI:C) of the proband and his relatives were measured by a clotting method using automatic coagulation analyzer. FXI antigen (FXI:Ag) was assayed by enzyme-linked immunosorbent assay (ELISA). Read More

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http://dx.doi.org/10.3760/cma.j.issn.1003-9406.2018.04.014DOI Listing
August 2018
21 Reads

Inherited Bleeding Disorders in the Obstetric Patient.

Transfus Med Rev 2018 10 12;32(4):237-243. Epub 2018 Jul 12.

Bloodworks Northwest, Seattle, WA; Department of Medicine, University of Washington, Seattle, WA. Electronic address:

Inherited bleeding disorders increase the risk of bleeding in the obstetric patient. Randomized controlled trials to compare prophylactic or therapeutic interventions are rare, and guidance documents rely heavily on expert opinion. Here we report the results of a systematic review of the literature for the treatment and prevention of peripartum bleeding in women with an inherited bleeding disorder. Read More

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http://dx.doi.org/10.1016/j.tmrv.2018.06.003DOI Listing
October 2018
14 Reads

Factor XI promotes hemostasis in factor IX-deficient mice.

J Thromb Haemost 2018 Oct 16;16(10):2044-2049. Epub 2018 Aug 16.

Department of Pathology, Microbiology and Immunology, Vanderbilt University Medical Center, Nashville, TN, USA.

Essentials Mice lacking factor IX (FIX) or factor XI (FXI) were tested in a saphenous vein bleeding model. FIX-deficient mice displayed a hemostatic defect and FXI-deficient mice were similar to wild type mice. Infusion of FXI or over-expression of FXI in FIX-deficient mice improved hemostasis. Read More

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http://dx.doi.org/10.1111/jth.14243DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6173617PMC
October 2018
9 Reads

Cerebral Dopamine Neurotrophic Factor (CDNF) Has Neuroprotective Effects against Cerebral Ischemia That May Occur through the Endoplasmic Reticulum Stress Pathway.

Int J Mol Sci 2018 Jun 29;19(7). Epub 2018 Jun 29.

Department of Cell Biology and Neurobiology, School of Basic Medicine, Shandong University, No. 44 Wenhua Xi Road, Jinan 250012, China.

Cerebral dopamine neurotrophic factor (CDNF), previously known as the conserved dopamine neurotrophic factor, belongs to the evolutionarily conserved CDNF/mesencephalic astrocyte-derived neurotrophic factor MANF family of neurotrophic factors that demonstrate neurotrophic activities in dopaminergic neurons. The function of CDNF during brain ischemia is still not known. MANF is identified as an endoplasmic reticulum (ER) stress protein; however, the role of CDNF in ER stress remains to be fully elucidated. Read More

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http://dx.doi.org/10.3390/ijms19071905DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6073452PMC
June 2018
7 Reads

Management of Severe Factor XI Deficiency with Factor XI Concentrate to Facilitate Prolonged Use of a Thoracic Epidural Catheter.

Pain Med 2019 Jan;20(1):198-199

Department of Haematology, Royal North Shore Hospital, St. Leonards, New South Wales, Australia.

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http://dx.doi.org/10.1093/pm/pny126DOI Listing
January 2019
3 Reads

Factor XI Deficiency and Pregnancy: Abnormal Lab Values or Significant Risk?

Anesth Analg 2018 Jul;127(1)

Vanderbilt University School of Medicine Northwestern University Feinberg School of Medicine

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http://dx.doi.org/10.1213/ANE.0000000000003501DOI Listing
July 2018
2 Reads

Severe Ankle Arthritis After Multiple Hemarthrosis Secondary to Factor XI Deficiency: A Case Report.

J Foot Ankle Surg 2018 Nov - Dec;57(6):1242-1245. Epub 2018 Jun 8.

Program Director, Podiatric Surgical Residency Program, Beaumont Hospital-Wayne, Wayne, MI.

Hemophilic arthropathy of the ankle is an extremely unique condition that affects a small subset of the population in the United States. We present the case of a 66-year-old male with factor XI deficiency, which, over years of repetitive microtrauma as a professional soccer player, led to multiple hemarthrosis and severe arthropathy of his right ankle. The patient successfully underwent total ankle replacement arthroplasty and was able to return to recreational sports and competitive road cycling. Read More

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http://dx.doi.org/10.1053/j.jfas.2018.03.019DOI Listing
February 2019
1 Read

Abnormal plasma clot formation and fibrinolysis reveal bleeding tendency in patients with partial factor XI deficiency.

Blood Adv 2018 05;2(10):1076-1088

Department of Pathology and Laboratory Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC.

Individuals with factor XI (FXI) deficiency have a variable bleeding risk that cannot be predicted from plasma FXI antigen or activity. This limitation can result in under- or overtreatment of patients and risk of bleeding or thrombosis. Previously, plasma clot fibrinolysis assays showed sensitivity to bleeding tendency in a small cohort of patients with severe FXI deficiency. Read More

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http://dx.doi.org/10.1182/bloodadvances.2017015123DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5965046PMC
May 2018
6 Reads

Successful hemostatic management of major surgery for cervical spondylotic myelopathy in a patient with severe factor XI deficiency.

Int J Hematol 2018 Oct 30;108(4):443-446. Epub 2018 Apr 30.

Department of Hematology, Gunma University Graduate School of Medicine, Maebashi, Japan.

Factor XI deficiency (FXID) is a rare bleeding disorder caused by mutations in the F11 gene. Spontaneous bleeding in patients with factor XI deficiency is rare, but major bleeding may occur after surgery or trauma. The basic method for hemostatic treatment is replacement of the missing factor using FXI concentrate or fresh frozen plasma (FFP). Read More

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http://dx.doi.org/10.1007/s12185-018-2462-yDOI Listing
October 2018
5 Reads

Activated partial thromboplastin time derivative curves: helpful diagnostic tool in mixing test interpretation.

Blood Coagul Fibrinolysis 2018 Jun;29(4):410-414

Division of Hematology, Department of Pediatrics, Erciyes University Faculty of Medicine, Kayseri, Turkey.

: The mixing test is used to evaluate whether prolonged activated partial thromboplastin time (APTT) is due to an inhibitor or a factor deficiency. The coagulation reaction is demonstrated with APTT derivative curves on the ACL TOP series. We aimed to determine the utility of APTT derivative curves in the mixing test process. Read More

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http://dx.doi.org/10.1097/MBC.0000000000000728DOI Listing
June 2018
6 Reads

Deep venous thrombosis treated by rivaroxaban in a young patient with type Ia carbohydrate-deficient glycoprotein (CDG) syndrome.

Ann Biol Clin (Paris) 2018 Apr;76(2):217-223

Service d'hématologie biologique, Hôpital Lariboisière, AP-HP, Paris, France.

Congenital disorders of glycosylation (CDG) are rare inborn diseases of glycan component of N-glycosylated proteins. We report here the case of a 28-year-old patient with CDG syndrome type Ia, who presented with a deep venous thrombosis in the left suro-popliteal vein with no known triggers or antecedents. The patient was treated with rivaroxaban for six months. Read More

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http://dx.doi.org/10.1684/abc.2018.1324DOI Listing
April 2018
10 Reads

Factor XI deficiency is not associated with an increased risk of pneumonia and pneumonia-related mortality.

Haemophilia 2018 Jul 2;24(4):634-640. Epub 2018 Apr 2.

Department of Community Medicine and Epidemiology, Lady Davis Carmel Medical Center, Haifa, Israel.

Introduction: Drugs targeting factor XI (FXI) shows promising results in reducing postoperative VTE. Recently, researchers have shown that FXI knockout mice had a worse outcome when infected with pathogens for pneumonia, raising concerns about the safety of these drugs.

Aim: To investigate the effect of FXI deficiency on the incidence of pneumonia and outcomes of pneumonia in humans. Read More

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http://dx.doi.org/10.1111/hae.13463DOI Listing
July 2018
6 Reads

miR-21 depletion in macrophages promotes tumoricidal polarization and enhances PD-1 immunotherapy.

Oncogene 2018 06 15;37(23):3151-3165. Epub 2018 Mar 15.

Department of Cancer Biology, Lerner Research Institute, Cleveland Clinic, Cleveland, OH, 44195, USA.

MicroRNA-21 (miR-21) is one of the most abundant microRNAs in mammalian cells. It has been intensively studied for its role in regulating apoptosis and oncogenic transformation. However, the impact of miR-21 on host anti-tumor immunity remains unknown. Read More

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http://www.nature.com/articles/s41388-018-0178-3
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http://dx.doi.org/10.1038/s41388-018-0178-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5993583PMC
June 2018
11 Reads

A novel mutation (Tyr503Cys) in a severe factor XI deficiency.

Blood Coagul Fibrinolysis 2018 Apr;29(3):338-343

Department of Clinical Laboratory, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, China.

: The objective is to study a gene mutation (Tyr503Cys) found in a Chinese consanguineous marriage family with inherited factor XI (FXI) deficiency (cross-reacting material positive, type II). The FXI activity and FXI antigen were tested with clotting assay and ELISA, respectively. The FXI gene was amplified by PCR with direct sequencing. Read More

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http://Insights.ovid.com/crossref?an=00001721-900000000-9865
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http://dx.doi.org/10.1097/MBC.0000000000000718DOI Listing
April 2018
9 Reads

Successful perioperative management in a patient with factor XI deficiency.

BMJ Case Rep 2018 Feb 21;2018. Epub 2018 Feb 21.

Hematology-Oncology, Walter Reed National Military Medical Center, Bethesda, Maryland, USA.

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. Read More

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http://dx.doi.org/10.1136/bcr-2017-222434DOI Listing
February 2018
11 Reads

Perioperative management of a redo aortic root replacement in a patient with severe factor XI deficiency.

J Card Surg 2018 Feb 4;33(2):86-89. Epub 2018 Feb 4.

Cardiac Surgery Division, Department of Surgery, Massachusetts General Hospital, Boston, Massachusetts.

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. Read More

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http://dx.doi.org/10.1111/jocs.13526DOI Listing
February 2018
7 Reads

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).

Haemophilia 2018 May 1;24(3):405-413. Epub 2018 Feb 1.

Department of Medicine I, Clinical Division of Hematology and Hemostaseology, Medical University of Vienna, Vienna, Austria.

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. Read More

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http://dx.doi.org/10.1111/hae.13422DOI Listing
May 2018
10 Reads

The Evolving Dilemma of Factor XI in Pregnancy: Suggestions for Management.

Anesth Analg 2018 Jun;126(6):2032-2037

Department of Anesthesiology, Northshore University Hospital at Northwell Health, Manhasset, New York.

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. Read More

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http://dx.doi.org/10.1213/ANE.0000000000002836DOI Listing
June 2018
5 Reads

Assessment of two contact activation reagents for the diagnosis of congenital factor XI deficiency.

Thromb Res 2018 03 3;163:64-70. Epub 2018 Jan 3.

Servicio de Hematología y Oncología Médica, Hospital Universitario Morales Meseguer, Centro Regional de Hemodonación, Universidad de Murcia, IMIB-Arrixaca, CIBERER, Murcia, Spain. Electronic address:

Introduction: Congenital FXI deficiency, a coagulopathy associated with low bleeding risk but thrombotic protection, is usually diagnosed by prolonged APTT and confirmed by coagulation assays. Recent evidences suggest that FXI deficiency might be underestimated. Sensitive and reliable methods to detect FXI deficiency are required. Read More

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http://dx.doi.org/10.1016/j.thromres.2017.12.023DOI Listing
March 2018
4 Reads

[Analysis on the novel compound heterozygous mutation FⅪ of a patient with hereditary factor Ⅺ deficiency].

Zhonghua Yi Xue Za Zhi 2017 Dec;97(48):3774-3778

Department of Laboratory Medicine, the People's Hospital of Wenzhou, Wenzhou 325000 , China.

To investigate the clinical phenotype and genotype characteristics of a Chinese hereditary factor Ⅺ deficiency pedigree. The activated partial thromboplastin time (APTT), prothrombin time (PT), FⅪ activity (FⅪ: C) were measured by clotting method using automatic coagulation analyzer. The FⅪ antigen (FⅪ: Ag) was assayed by enzyme-linked immunosorbent assay (ELISA). Read More

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http://dx.doi.org/10.3760/cma.j.issn.0376-2491.2017.48.004DOI Listing
December 2017
12 Reads

Villin-1 and Gelsolin Regulate Changes in Actin Dynamics That Affect Cell Survival Signaling Pathways and Intestinal Inflammation.

Gastroenterology 2018 04 21;154(5):1405-1420.e2. Epub 2017 Dec 21.

Department of Biology and Biochemistry, University of Houston, Houston, Texas; Department of Allied Health, Baylor College of Medicine, Houston, Texas. Electronic address:

Background & Aims: Cell stress signaling pathways result in phosphorylation of the eukaryotic translation initiation factor 2 subunit alpha (EIF2S1 or EIF2A), which affects regulation of protein translation. Translation reprogramming mitigates stress by activating pathways that result in autophagy and cell death, to eliminate damaged cells. Actin is modified during stress and EIF2A is dephosphorylated to restore homeostasis. Read More

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http://dx.doi.org/10.1053/j.gastro.2017.12.016DOI Listing
April 2018
10 Reads

2017 Scientific Sessions Sol Sherry Distinguished Lecture in Thrombosis: Factor XI as a Target for New Anticoagulants.

Arterioscler Thromb Vasc Biol 2018 02 21;38(2):304-310. Epub 2017 Dec 21.

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.

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. Read More

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http://dx.doi.org/10.1161/ATVBAHA.117.309664DOI Listing
February 2018
12 Reads

Normal aPTT in children with mild factor XI deficiency.

Pediatr Blood Cancer 2018 Apr 12;65(4). Epub 2017 Dec 12.

Department of Pathology, Saint Louis University School of Medicine, St. Louis, Missouri.

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. Read More

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http://dx.doi.org/10.1002/pbc.26910DOI Listing
April 2018
8 Reads

Elderly apolipoprotein E‑/‑ mice with advanced atherosclerotic lesions in the aorta do not develop Alzheimer's disease-like pathologies.

Mol Med Rep 2018 Feb 21;17(2):2488-2492. Epub 2017 Nov 21.

Thrombosis Unit, Sheba Medical Center, Tel‑Hashomer 5265601, Israel.

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. Read More

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http://dx.doi.org/10.3892/mmr.2017.8127DOI Listing
February 2018
14 Reads

Molecular investigation of 41 patients affected by coagulation factor XI deficiency.

Haemophilia 2018 03 27;24(2):e50-e55. Epub 2017 Nov 27.

Humanitas Clinical and Research Center, Rozzano, Milan, Italy.

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http://dx.doi.org/10.1111/hae.13378DOI Listing
March 2018
2 Reads

Inhibitors in Patients with Congenital Bleeding Disorders Other Than Hemophilia.

Semin Thromb Hemost 2018 Sep 17;44(6):595-603. Epub 2017 Nov 17.

Italian National Blood Centre, National Institute of Health, Rome, Italy.

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. Read More

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http://dx.doi.org/10.1055/s-0037-1607441DOI Listing
September 2018
9 Reads

Factor XI gene variants in factor XI-deficient patients of Southern Italy: identification of a novel mutation and genotype-phenotype relationship.

Hum Genome Var 2017 9;4:17043. Epub 2017 Nov 9.

IRCCS 'Casa Sollievo della Sofferenza', Unità di Aterosclerosi e Trombosi, San Giovanni Rotondo (Foggia), Italy.

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. Read More

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http://www.nature.com/articles/hgv201743
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http://dx.doi.org/10.1038/hgv.2017.43DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5678205PMC
November 2017
28 Reads

Limited role of kininogen in the host response during gram-negative pneumonia-derived sepsis.

Am J Physiol Lung Cell Mol Physiol 2018 03 9;314(3):L397-L405. Epub 2017 Nov 9.

Center of Experimental and Molecular Medicine, Academic Medical Center, University of Amsterdam , Amsterdam , The Netherlands.

High-molecular-weight kininogen (HK), together with factor XI, factor XII and prekallikrein, is part of the contact system that has proinflammatory, prothrombotic, and vasoactive properties. We hypothesized that HK plays a role in the host response during pneumonia-derived sepsis. To this end mice were depleted of kininogen (KNG) to plasma HK levels of 28% of normal by repeated treatment with a specific antisense oligonucleotide (KNG ASO) for 3 wk before infection with the common human sepsis pathogen Klebsiella pneumoniae via the airways. Read More

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http://dx.doi.org/10.1152/ajplung.00288.2017DOI Listing
March 2018
10 Reads

[Characteristics of Induced by Needling Zusanli (ST 36) in 527 Healthy Volunteers with Different Constitutions].

Zhen Ci Yan Jiu 2016 Dec;41(6):535-9

Hanzhong City Central Hospital, Hanzhong 723000.

Objective: To explore the relationship between the theory of constitution in terms of Chinese medicine and clinical efficacy of acupuncture treatment via analyzling the characteristics of during acupuncture stimulation of Zusanli (ST 36) in healthy volunteers with different constitutions.

Methods: In the present study, a total of 527 healthy undergraduate student volunteers (267 girls and 260 boys) were recruited. They received questionnaire first about their constitutions in accordance with Professor WANG Qi's in terms of Chinese medicine. Read More

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December 2016
20 Reads

α-Linolenic Acid Inhibits Receptor Activator of NF-κB Ligand Induced (RANKL-Induced) Osteoclastogenesis and Prevents Inflammatory Bone Loss via Downregulation of Nuclear Factor-KappaB-Inducible Nitric Oxide Synthases (NF-κB-iNOS) Signaling Pathways.

Med Sci Monit 2017 Oct 24;23:5056-5069. Epub 2017 Oct 24.

Department of Orthopedics, Shan Xi Provincial People's Hospital, Taiyuan, Shanxi, China (mainland).

BACKGROUND Inflammation is a major cellular strain causing increased risk of osteo-degenerative diseases. Omega-3 fatty acids have been great source in suppressing inflammation. We investigated the effect of α-linolenic acid (ALA) on RANKL-stimulated osteoclast differentiation, LPS-induced and ovariectomized bone loss in mice models. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5665607PMC
October 2017
8 Reads

Single-Dose, Preoperative Vitamin-D Supplementation Decreases Infection in a Mouse Model of Periprosthetic Joint Infection.

J Bone Joint Surg Am 2017 Oct;99(20):1737-1744

1Department of Orthopaedic Surgery, David Geffen School of Medicine at the University of California, Los Angeles, Los Angeles, California.

Background: Despite recent advances, infection remains the most common etiology of arthroplasty failure. Recent work suggests that 25-hydroxyvitamin D (25D) deficiency correlates with the frequency of periprosthetic joint infection (PJI). We endeavored to examine whether 25D3 deficiency leads to increased bacterial burden in vivo in an established mouse model of PJI and, if so, whether this effect can be reversed by preoperative 25D3 supplementation. Read More

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http://dx.doi.org/10.2106/JBJS.16.01598DOI Listing
October 2017
30 Reads

Acquired factor XI deficiency and therapeutic plasma exchange.

J Clin Apher 2018 Jun 13;33(3):427-430. Epub 2017 Oct 13.

Department of Pathology, University of Chicago, Chicago, Illinois.

Congenital factor XI (FXI) deficiency is associated with a variable bleeding phenotype. Recent reports have documented the use of therapeutic plasma exchange to rapidly and isovolumetrically increase FXI levels before invasive procedures in patients with congenital FXI deficiency. We report a case of acquired FXI deficiency in a pregnant woman with lupus. Read More

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http://dx.doi.org/10.1002/jca.21593DOI Listing
June 2018
11 Reads

High incidence of FXI deficiency in a Spanish town caused by 11 different mutations and the first duplication of F11: Results from the Yecla study.

Haemophilia 2017 Nov 27;23(6):e488-e496. Epub 2017 Sep 27.

Servicio de Hematología y Oncología Médica, Hospital Universitario Morales Meseguer, Centro Regional de Hemodonación, IMIB-Arrixaca, Universidad de Murcia, CIBERER, Murcia, Spain.

Introduction: Factor XI (FXI) deficiency is a rare disorder with molecular heterogeneity in Caucasians but relatively frequent and molecularly homogeneous in certain populations.

Aim: To characterize FXI deficiency in a Spanish town of 60 000 inhabitants.

Methods: A total of 324 764 APTT tests were screened during 20 years. Read More

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http://dx.doi.org/10.1111/hae.13356DOI Listing
November 2017
3 Reads

Three Factor 11 Mutations Associated with Factor XI Deficiency in a Turkish Family.

Turk J Haematol 2018 Mar 26;35(1):79-80. Epub 2017 Sep 26.

İstinye University Faculty of Medicine, Department of Medical Genetics, İstanbul, Turkey.

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http://dx.doi.org/10.4274/tjh.2017.0140DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5843782PMC
March 2018
6 Reads

Targeting the Type II Secretion System: Development, Optimization, and Validation of a High-Throughput Screen for the Identification of Small Molecule Inhibitors.

Front Cell Infect Microbiol 2017 28;7:380. Epub 2017 Aug 28.

Department of Microbiology and Immunology, University of Michigan Medical SchoolAnn Arbor, MI, United States.

Nosocomial pathogens that develop multidrug resistance present an increasing problem for healthcare facilities. Due to its rapid rise in antibiotic resistance, is one of the most concerning gram-negative species. typically infects immune compromised individuals resulting in a variety of outcomes, including pneumonia and bacteremia. Read More

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http://dx.doi.org/10.3389/fcimb.2017.00380DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5581314PMC
July 2018
18 Reads

Comments on 'Management of parturients with Factor XI deficiency - 10 year case series and review of the literature'.

Eur J Obstet Gynecol Reprod Biol 2017 Oct 4;217:176. Epub 2017 Sep 4.

Oxford University Hospital, United Kingdom.

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http://dx.doi.org/10.1016/j.ejogrb.2017.08.043DOI Listing
October 2017
6 Reads

Management of severe factor XI deficiency in cardiac surgery: A case report and review of the literature.

Haemophilia 2017 11 31;23(6):e512-e514. Epub 2017 Aug 31.

Division of Hematology & Oncology, Oregon Health & Science University, Portland, OR, USA.

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http://dx.doi.org/10.1111/hae.13311DOI Listing
November 2017
5 Reads