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https://www.readbyqxmd.com/read/28432284/treatment-with-recombinant-factor-xiii-tretten-in-a-pregnant-woman-with-factor-xiii-deficiency
#1
Nizar Abdel-Samad
BACKGROUND Factor XIII deficiency is associated with recurrent miscarriages in women. CASE REPORT In this report, we present a patient with factor XIII deficiency and some comorbidities who had had previous miscarriages. She began treatment with factor XIII subunit A (XIII-A) replacement treatment Recombinant factor XIII (Tretten) at a dose of 2500 units monthly and was able, for the first time, to carry a pregnancy almost to term. Although she experienced some obstetrical complications, she delivered a healthy baby...
April 22, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28428079/mineralization-inhibiting-effects-of-transglutaminase-crosslinked-polymeric-osteopontin
#2
Betty Hoac, Valentin Nelea, Wenge Jiang, Mari T Kaartinen, Marc D McKee
Osteopontin (OPN) belongs to the SIBLING family (Small, Integrin-Binding LIgand N-linked Glycoproteins) of mineral-binding matrix proteins found in bones and teeth. OPN is a well-known inhibitor of matrix mineralization, and enzymatic modification of OPN can affect this inhibitory function. In bone, OPN exists both as a monomer and as a high-molecular-weight polymer - the latter is formed by transglutaminase-mediated crosslinking of glutamine and lysine residues in OPN to create homotypic protein assemblies...
April 17, 2017: Bone
https://www.readbyqxmd.com/read/28418908/plasma-protein-profiling-in-patients-undergoing-coronary-artery-bypass-grafting-surgery-and-clinical-significance
#3
Zhi-Peng Guo, Hai-Tao Hou, Rui Jing, Zhen-Guo Song, Xiao-Cheng Liu, Guo-Wei He
This study was designed to identify the protein profiling in patients with triple vessel coronary artery disease (CAD) undergoing CABG, in order to detect CAD-related differential proteins in these patients. CABG patients with triple vessel disease with/without left main stenosis (n =160) were compared to normal coronary angiographic subjects (n =160). Plasma samples of 20 males and 20 females in each group were analyzed with iTRAQ technique. ELISA test was used to test the chosen proteins from iTRAQ results in plasma samples from a new cohort of the CABG group (n=120, male/femal=61/59) and control (n =120, male/female=60/60)...
March 18, 2017: Oncotarget
https://www.readbyqxmd.com/read/28415608/collagen-type-iv-alpha-1-col4a1-and-collagen-type-xiii-alpha-1-col13a1-produced-in-cancer-cells-promote-tumor-budding-at-the-invasion-front-in-human-urothelial-carcinoma-of-the-bladder
#4
Makito Miyake, Shunta Hori, Yosuke Morizawa, Yoshihiro Tatsumi, Michihiro Toritsuka, Sayuri Ohnishi, Keiji Shimada, Hideki Furuya, Vedbar S Khadka, Youping Deng, Kenta Ohnishi, Kota Iida, Daisuke Gotoh, Yasushi Nakai, Takeshi Inoue, Satoshi Anai, Kazumasa Torimoto, Katsuya Aoki, Nobumichi Tanaka, Noboru Konishi, Kiyohide Fujimoto
Current knowledge of the molecular mechanism driving tumor budding is limited. Here, we focused on elucidating the detailed mechanism underlying tumor budding in urothelial cancer of the bladder. Invasive urothelial cancer was pathologically classified into three groups as follows: nodular, trabecular, and infiltrative (tumor budding). Pathohistological analysis of the orthotopic tumor model revealed that human urothelial cancer cell lines MGH-U3, UM-UC-14, and UM-UC-3 displayed typical nodular, trabecular, and infiltrative patterns, respectively...
March 21, 2017: Oncotarget
https://www.readbyqxmd.com/read/28412719/combined-measurement-of-factor-xiii-and-d-dimer-is-helpful-for-differential-diagnosis-in-patients-with-suspected-pulmonary-embolism
#5
Ning Tang, Ziyong Sun, Dengju Li, Jun Yang, Shiyu Yin, Qing Guan
BACKGROUND: D-dimer has been used to rule out pulmonary embolism (PE). Based on previous reports of decreased concentrations of coagulation factor XIII (FXIII) in venous thromboembolism, and no change in FXIII concentration in patients with acute cardiovascular disease, we evaluated the benefit of simultaneously measuring D-dimer and FXIII concentrations for diagnosing PE. METHODS: In this prospective single-center study, we enrolled 209 patients initially suspected of having PE, and measured their D-dimer and FXIII concentrations...
April 17, 2017: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/28406553/detection-of-factor-xiii-deficiency-data-from-multicentre-exercises-amongst-uk-neqas-and-pro-rbdd-project-laboratories
#6
I Jennings, S Kitchen, M Menegatti, R Palla, I Walker, M Makris, F Peyvandi
INTRODUCTION: FXIII deficiency is a rare bleeding disorders, and specific FXIII assays are recommended to detect this deficiency. We investigated the performance and accuracy of FXIII investigations in two exercises, comparing centres enrolled in the PRO-RBDD project (prospective data collection on patients with fibrinogen and Factor XIII deficiencies), and UK NEQAS BC centres. METHODS: Samples from a FXIII deficient subject and a normal donor were sent to participating centres, to investigate for FXIII deficiency, and interpret their results...
April 13, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28388959/autosomal-recessive-inherited-bleeding-disorders-in-pakistan-a-cross-sectional-study-from-selected-regions
#7
Arshi Naz, Muhammad Younus Jamal, Samina Amanat, Ikram Din Ujjan, Akber Najmuddin, Humayun Patel, Fazle Raziq, Nisar Ahmed, Ayisha Imran, Tahir Sultan Shamsi
BACKGROUND: Autosomal recessive bleeding disorders (ARBDs) include deficiencies of clotting factors I, II, V, VII, X, XI, XIII, vitamin K dependent clotting factors, combined factor V & VIII, Von Willebrand Disease (vWD) type 3, Glanzmann's thrombasthenia (GT) and Bernard-Soulier syndrome. Patients with primary bleeding disorders from all the major provincial capitals of Pakistan were screened for ARBDs. Prothrombin (PT), activated partial thromboplastin time (APTT), bleeding time (BT) and fibrinogen levels were measured...
April 7, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28387755/transglutaminases-factor-xiii-a-and-tg2-regulate-resorption-adipogenesis-and-plasma-fibronectin-homeostasis-in-bone-and-bone-marrow
#8
Aisha Mousa, Cui Cui, Aimei Song, Vamsee D Myneni, Huifang Sun, Jin Jin Li, Monzur Murshed, Gerry Melino, Mari T Kaartinen
Appropriate bone mass is maintained by bone-forming osteoblast and bone-resorbing osteoclasts. Mesenchymal stem cell (MSC) lineage cells control osteoclastogenesis via expression of RANKL and OPG (receptor activator of nuclear factor κB ligand and osteoprotegerin), which promote and inhibit bone resorption, respectively. Protein crosslinking enzymes transglutaminase 2 (TG2) and Factor XIII-A (FXIII-A) have been linked to activity of myeloid and MSC lineage cells; however, in vivo evidence has been lacking to support their function...
April 7, 2017: Cell Death and Differentiation
https://www.readbyqxmd.com/read/28380473/successful-management-of-a-patient-with-autoimmune-hemorrhaphilia-due-to-anti-factor-xiii-13-antibodies-complicated-by-pulmonary-thromboembolism
#9
Yoshiyuki Ogawa, Kunio Yanagisawa, Masayoshi Souri, Masahiro Mihara, Chiaki Naito, Makiko Takizawa, Takuma Ishizaki, Takeki Mitsui, Hiroshi Handa, Tsukasa Osaki, Yoshihisa Nojima, Akitada Ichinose
Autoimmune hemophilia-like disease (hemorrhaphilia) due to anti-factor XIII (FXIII) antibodies (AH13) is a very rare, life-threatening bleeding disorder. A 77-year-old woman developed macrohematuria and a right renal pelvic hematoma. The coagulation times were not prolonged, but FXIII activity and antigen levels were severely and moderately reduced to 9 and 29% of normal values, respectively. Accordingly, the FXIII-specific activity turned out to be low. FXIII inhibitor and anti-FXIII-A subunit autoantibodies were detected by a 1:1 crossmixing test and immunoblot and immunochromatographic assays...
April 6, 2017: Acta Haematologica
https://www.readbyqxmd.com/read/28345289/severe-bleeding-diatheses-in-an-elderly-patient-with-combined-type-autoantibody-against-factor-xiii-a-subunit-novel-approach-to-the-diagnosis-and-classification-of-anti-factor-xiii-antibodies
#10
M Kun, N Szuber, É Katona, K Pénzes, A Bonnefoy, B Bécsi, F Erdődi, G E Rivard, L Muszbek
INTRODUCTION: Acquired factor XIII (FXIII) deficiency due to autoantibody is a rare, severe bleeding diathesis. Its laboratory diagnosis and classification represents a difficult task. AIM: Introduction of novel approaches into the diagnosis and characterization of anti-FXIII autoantibody and demonstration of their use in the diagnosis of a patient with autoimmune FXIII deficiency. METHODS: Factor XIII activity, FXIII antigen levels and the titre of anti-FXIII-A antibody were monitored throughout the course of the disease...
March 26, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28335488/italian-registry-of-congenital-bleeding-disorders
#11
Adele Giampaolo, Francesca Abbonizio, Romano Arcieri, Hamisa Jane Hassan
In Italy, the surveillance of people with bleeding disorders is based on the National Registry of Congenital Coagulopathies (NRCC) managed by the Italian National Institute of Health (Istituto Superiore di Sanità). The NRCC collects epidemiological and therapeutic data from the 54 Hemophilia Treatment Centers, members of the Italian Association of Hemophilia Centres (AICE). The number of people identified with bleeding disorders has increased over the years, with the number rising from approx. 7000 in 2000 to over 11,000 in 2015...
March 19, 2017: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/28318109/agreement-between-factor-xiii-activity-and-antigen-assays-in-measurement-of-factor-xiii-a-french-multicenter-study-of-147-human-plasma-samples
#12
C Caron, R Meley, V Le Cam Duchez, M F Aillaud, C Lavenu-Bombled, F Dutrillaux, C Flaujac, A Ryman, C Ternisien, D Lasne, H Galinat, C Pouplard
INTRODUCTION: Factor XIII (FXIII) deficiency is a rare hemorrhagic disorder whose early diagnosis is crucial for appropriate treatment and prophylactic supplementation in cases of severe deficiency. International guidelines recommend a quantitative FXIII activity assay as first-line screening test. FXIII antigen measurement may be performed to establish the subtype of FXIII deficiency (FXIIID) when activity is decreased. METHODS: The aim of this multicenter study was to evaluate the analytical and diagnostic levels of performance of a new latex immunoassay, K-Assay(®) FXIII reagent from Stago, for first-line measurement of FXIII antigen...
March 20, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28187047/is-extracorporeal-co2-removal-really-safe-and-less-invasive-observation-of-blood-injury-and-coagulation-impairment-during-ecco2r
#13
Johannes Kalbhenn, Nadine Neuffer, Barbara Zieger, Axel Schmutz
Extracorporeal CO2-Removal (ECCO2R) is promoted with attributes like "safe" and "less invasive" compared to (high-flow) veno-venous ECMO-Systems. With our experience in coagulation disorders during ECMO-therapy with this observational study we for the first time prospectively evaluate hemolysis and coagulation disorders during ECCO2R. Eight consecutive patients with predominant hypercapnic respiratory failure were treated with the Hemolung® Respiratory Assist System (RAS) (Alung-Technologies, Pittsburg, USA)...
February 7, 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/28166593/impact-of-rare-bleeding-disorders-during-pregnancy-on-maternal-and-fetal-outcomes-review-of-29-pregnancies-at-a-single-center
#14
Gokcen Orgul, Fatih Aktoz, M Sinan Beksac
Objective This study aims to give information about the relationship between different types of factor deficiencies and maternal/obstetric outcomes. Methods We retrospectively reviewed the medical records of eight women with factor deficiency disorders. The demographic and clinical features of the patients after their last pregnancies were registered retrospectively. Results There were 29 pregnancies among the 8 patients. The spontaneous abortion rate was relatively high in two patients with factor XIII deficiency (80% and 57...
January 2017: Revista Brasileira de Ginecologia e Obstetrícia
https://www.readbyqxmd.com/read/28159767/ticagrelor-reversal-in-vitro-assessment-of-four-haemostatic-agents
#15
Leyla Calmette, Anne-Céline Martin, Bernard Le Bonniec, Diane Zlotnik, Isabelle Gouin-Thibault, Christilla Bachelot-Loza, Pascale Gaussem, Anne Godier
AIM: Management of ticagrelor-induced bleeding is challenging as platelet transfusion is ineffective. An effective strategy is needed. This study aimed to investigate in vitro the efficacy of four haemostatic drugs (HDs), namely recombinant activated factor VII (rFVIIa), fibrinogen concentrate (Fib), tranexamic acid (TXA) and factor XIII concentrate (FXIII) to improve the haemostatic capacity in the presence of ticagrelor. METHODS: Blood was spiked with ticagrelor then supplemented by either HD or control...
February 3, 2017: Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28137667/identification-of-novel-autoantigens-via-mass-spectroscopy-based-antibody-mediated-identification-of-autoantigens-ms-amida-using-immune-thrombocytopenic-purpura-itp-as-a-model-disease
#16
Julian Kamhieh-Milz, Viktor Sterzer, Hatice Celik, Omid Khorramshahi, Reham Fadl Hassan Moftah, Abdulgabar Salama
Immune thrombocytopenic purpura (ITP) is one of the best characterized autoimmune diseases. Autoantibodies (AABs) against platelet antigens are considered as the diagnostic hallmark of ITP, but are detectable in only 50% of patients. We designed and applied a novel proteomic approach termed Mass Spectroscopy-based Antibody-Mediated Identification of Autoantigens (MS-AMIDA) for platelet antigens. Patients were separated into patients with classical AABs [ITP(+)] and patients without AABs [ITP(-)]. Altogether, 181 potential AAGs were found in ITP(+) and 135 AAGs in ITP(-), with 34 and 23 AAGs reproducibly found in two runs of MS-AMIDA...
March 22, 2017: Journal of Proteomics
https://www.readbyqxmd.com/read/28129465/evaluation-of-coagulation-during-treatment-with-directly-acting-antivirals-in-patients-with-hepatitis-c-virus-related-cirrhosis
#17
Armando Tripodi, Roberta D'Ambrosio, Lidia Padovan, Giulia Tosetti, Alessio Aghemo, Massimo Primignani, Veena Chantarangkul, Flora Peyvandi, Massimo Colombo
BACKGROUND & AIMS: The effect of direct-acting-antivirals (DAA) on coagulation of hepatitis-C-virus (HCV)-related cirrhosis is unknown. METHODS: We investigated 28 patients on DAA treatment and performed prothrombin-time, thrombin generation with and without thrombomodulin, whole-blood thromboelastometry, as well as the individual procoagulants (II, VIII, XIII, von Willebrand) and anticoagulants, antithrombin and protein-C. RESULTS: Patients had undetectable HCV-RNA at the end-of- treatment and at 12-weeks after end-of-treatment (sustained virological response)...
January 27, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28091415/advances-of-coagulation-factor-xiii
#18
REVIEW
Da-Yu Shi, Shu-Jie Wang
OBJECTIVE: To provide a comprehensive literature review on roles of coagulation factor XIII (FXIII) in coagulation, wound healing, neoplasm, bone metabolism, and pregnancy. DATA SOURCES: All articles in PubMed with key words "Coagulation factor XIII", "wound", "leukemia", "tumor", "bone," and "pregnancy" with published date from 2001 to 2016 were included in the study. Frequently cited publications before 2000 were also included. STUDY SELECTION: We reviewed the role of FXIII in biologic processes as documented in clinical, animal, and in vitro studies...
January 20, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28035782/wonoep-appraisal-biomarkers-of-epilepsy-associated-comorbidities
#19
REVIEW
Teresa Ravizza, Filiz Y Onat, Amy R Brooks-Kayal, Antoine Depaulis, Aristea S Galanopoulou, Andrey Mazarati, Adam L Numis, Raman Sankar, Alon Friedman
Neurologic and psychiatric comorbidities are common in patients with epilepsy. Diagnostic, predictive, and pharmacodynamic biomarkers of such comorbidities do not exist. They may share pathogenetic mechanisms with epileptogenesis/ictogenesis, and as such are an unmet clinical need. The objectives of the subgroup on biomarkers of comorbidities at the XIII Workshop on the Neurobiology of Epilepsy (WONOEP) were to present the state-of-the-art recent research findings in the field that highlighting potential biomarkers for comorbidities in epilepsy...
December 30, 2016: Epilepsia
https://www.readbyqxmd.com/read/28028391/vascular-genetic-variants-and-ischemic-stroke-susceptibility-in-albanians-from-the-republic-of-macedonia
#20
Bajram Kamberi, Farije Kamberi, Mirko Spiroski
BACKGROUND: Acute first-ever ischemic stroke (FIS) is a heterogeneous, polygenic disorder. The contribution of vascular genetic variants as inherited causes of ischemic stroke has remained controversial. AIM: To examine the association of genetic variants in vascular factors with the occurrence of FIS. MATERIAL AND METHODS: The current research was performed in a group of 39 patients with FIS (study group) and 102 healthy volunteers (control group)...
December 15, 2016: Open Access Macedonian Journal of Medical Sciences
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