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Bleeding disorders

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https://www.readbyqxmd.com/read/29333065/hepatitis-c-infection-in-patients-with-hereditary-bleeding-disorders-epidemiology-natural-history-and-management
#1
REVIEW
Nikolaos Papadopoulos, Vasiliki Argiana, Melanie Deutsch
Hereditary bleeding disorders include a group of diseases with abnormalities of coagulation. Prior to 1990, infection with hepatitis C virus (HCV) was mainly transmitted via pooled plasma products as a treatment for hereditary bleeding disorders. Anti-HCV positivity in these patients may be as high as >70% in some areas, while some of them have also been coinfected with human immunodeficiency virus. Since about 20% of HCV-infected patients clear the infection naturally, chronic HCV infection represents a significant health problem in this group of patients...
January 2018: Annals of Gastroenterology: Quarterly Publication of the Hellenic Society of Gastroenterology
https://www.readbyqxmd.com/read/29332421/comparison-of-two-weight-based-desmopressin-dosing-strategies-for-spontaneous-bleeding
#2
Miguel J Franquiz, Michelle C Hines, Siu Yan Amy Yeung
BACKGROUND: The pharmacokinetics and pharmacodynamics of desmopressin are appropriate for adjusted body weight-based dosing, particularly in obese patients. OBJECTIVE: The objective of this study was to describe desmopressin dosing strategies, with emphasis on hemostatic outcomes among patients without preexisting bleeding disorders. METHODS: This was a single-center, retrospective cohort study of patients who received intravenous weight-based desmopressin for a hemostatic indication...
January 1, 2018: Annals of Pharmacotherapy
https://www.readbyqxmd.com/read/29330124/manipulating-the-epigenome-for-the-treatment-of-disorders-with-thrombotic-complications
#3
REVIEW
Faith A A Kwa, Denise E Jackson
The haemostatic system is tightly regulated to maintain homeostasis to avoid unwanted bleeding or thrombotic complications. Recent research has highlighted the importance of epigenetic changes, such as DNA methylation, histone modifications, and miRNA-based mechanisms, that alter gene expression. This can give rise to dysregulated haemostatic or vascular expressed molecules contributing to the development of thrombotic complications. Targeting these epigenetic changes could provide a new avenue for the treatment of pathological blood clots...
January 9, 2018: Drug Discovery Today
https://www.readbyqxmd.com/read/29325753/induced-hypothermia-in-patients-with-septic-shock-and-respiratory-failure-cass-a-randomised-controlled-open-label-trial
#4
Theis Skovsgaard Itenov, Maria Egede Johansen, Morten Bestle, Katrin Thormar, Lars Hein, Louise Gyldensted, Anne Lindhardt, Henrik Christensen, Stine Estrup, Henrik Planck Pedersen, Matthew Harmon, Uday Kant Soni, Silvia Perez-Protto, Nicolai Wesche, Ulrik Skram, John Asger Petersen, Thomas Mohr, Tina Waldau, Lone Musaeus Poulsen, Ditte Strange, Nicole P Juffermans, Daniel I Sessler, Else Tønnesen, Kirsten Møller, Dennis Karsten Kristensen, Alessandro Cozzi-Lepri, Jens D Lundgren, Jens-Ulrik Jensen
BACKGROUND: Animal models of serious infection suggest that 24 h of induced hypothermia improves circulatory and respiratory function and reduces mortality. We tested the hypothesis that a reduction of core temperature to 32-34°C attenuates organ dysfunction and reduces mortality in ventilator-dependent patients with septic shock. METHODS: In this randomised, controlled, open-label trial, we recruited patients from ten intensive care units (ICUs) in three countries in Europe and North America...
January 8, 2018: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/29323665/arterial-tortuosity-syndrome-40-new-families-and-literature-review
#5
Aude Beyens, Juliette Albuisson, Annekatrien Boel, Mazen Al-Essa, Waheed Al-Manea, Damien Bonnet, Ozlem Bostan, Odile Boute, Tiffany Busa, Nathalie Canham, Ergun Cil, Paul J Coucke, Margot A Cousin, Majed Dasouki, Julie De Backer, Anne De Paepe, Sofie De Schepper, Deepthi De Silva, Koenraad Devriendt, Inge De Wandele, David R Deyle, Harry Dietz, Sophie Dupuis-Girod, Eudice Fontenot, Björn Fischer-Zirnsak, Alper Gezdirici, Jamal Ghoumid, Fabienne Giuliano, Neus Baena Diéz, Mohammed Z Haider, Joshua S Hardin, Xavier Jeunemaitre, Eric W Klee, Uwe Kornak, Manuel F Landecho, Anne Legrand, Bart Loeys, Stanislas Lyonnet, Helen Michael, Pamela Moceri, Shehla Mohammed, Laura Muiño-Mosquera, Sheela Nampoothiri, Karin Pichler, Katrina Prescott, Anna Rajeb, Maria Ramos-Arroyo, Massimiliano Rossi, Mustafa Salih, Mohammed Z Seidahmed, Elise Schaefer, Elisabeth Steichen-Gersdorf, Sehime Temel, Fahrettin Uysal, Marine Vanhomwegen, Lut Van Laer, Lionel Van Maldergem, David Warner, Andy Willaert, Tom R Collins, Andrea Taylor, Elaine C Davis, Yuri Zarate, Bert Callewaert
PurposeWe delineate the clinical spectrum and describe the histology in arterial tortuosity syndrome (ATS), a rare connective tissue disorder characterized by tortuosity of the large and medium-sized arteries, caused by mutations in SLC2A10.MethodsWe retrospectively characterized 40 novel ATS families (50 patients) and reviewed the 52 previously reported patients. We performed histology and electron microscopy (EM) on skin and vascular biopsies and evaluated TGF-β signaling with immunohistochemistry for pSMAD2 and CTGF...
January 11, 2018: Genetics in Medicine: Official Journal of the American College of Medical Genetics
https://www.readbyqxmd.com/read/29321442/acquired-von-willebrand-syndrome-due-to-aortic-valve-stenosis-in-a-case-with-antiphospholipid-antibody
#6
Hiroaki Tanaka, Yurie Nagai, Chihiro Kuwabara, Ryo Shimizu, Akihide Umeki, Tetsufumi Yamamoto
Acquired von Willebrand syndrome (AVWS) is a bleeding disorder caused by an acquired deficiency of von Willebrand factor (vWF). Some patients with AVWS show a low bleeding tendency and are diagnosed by the presence of a mild prolongation of activated partial thromboplastin time (APTT) preoperatively. Another cause of APTT prolongation is the presence of antiphospholipid antibody (aPL). We experienced a case of AVWS due to aortic valve stenosis in a patient with aPL in whom aortic valve replacement surgery was successful with vWF replacement...
January 11, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29319151/blood-and-conflict-managing-bleeding-disorders-in-gaza
#7
Hala Borno
No abstract text is available yet for this article.
January 2, 2018: Eastern Mediterranean Health Journal, la Revue de Santé de la Méditerranée Orientale
https://www.readbyqxmd.com/read/29317961/successful-treatment-of-rapidly-progressive-life-threatening-esophageal-submucosal-hematoma-in-a-patient-with-van-der-hoeve-syndrome
#8
Yasuhiro Watanabe, Naomi Shimizu, Masahiro Iwakawa, Takashi Yamaguchi, Noriko Ban, Hidetoshi Kawana, Atsuhito Saiki, Emiko Sakaida, Chiaki Nakaseko, Yasuhiro Matsuura, Nobuyuki Aotsuka, Hideaki Bujo, Ichiro Tatsuno
Osteogenesis imperfecta (OI) is a rare inherited disorder of the connective tissue with many reports on its association with bleeding diatheses. OI patients with blue sclera, hearing loss, and bone vulnerability are classified as having van der Hoeve syndrome. Here, we report the first case of rapidly progressing, massive esophageal submucosal hematoma in this syndrome. Bleeding in OI is reportedly due to defective capillary integrity and platelet dysfunction; however, our patient did not show such findings...
February 2018: Journal of Clinical Medicine Research
https://www.readbyqxmd.com/read/29317453/targeting-anticoagulant-protein-s-to-improve-hemostasis-in-hemophilia
#9
Raja Prince, Luca Bologna, Mirko Manetti, Daniela Melchiorre, Irene Rosa, Natacha Dewarrat, Silvia Suardi, Poorya Amini, José A Fernández, Laurent Burnier, Claudia Quarroz, Maria Desiré Reina Caro, Yasuhiro Matsumura, Johanna A Kremer Hovinga, John H Griffin, Hans-Uwe Simon, Lidia Ibba-Manneschi, François Saller, Sara Calzavarini, Anne Angelillo-Scherrer
Improved treatments are needed for hemophilia A and B, bleeding disorders affecting 400,000 people worldwide. We investigated whether targeting protein S could promote hemostasis in hemophilia by re-balancing coagulation. Protein S is an anticoagulant acting as cofactor for activated protein C and tissue factor pathway inhibitor (TFPI). This dual role makes PS a key regulator of thrombin generation. Here, we report that targeting protein S rebalances coagulation in hemophilia. Protein S gene targeting in hemophilic mice protected them against bleeding, especially when intra-articular...
January 9, 2018: Blood
https://www.readbyqxmd.com/read/29315156/bleeding-risk-of-variceal-band-ligation-in-extrahepatic-portal-vein-obstruction-is-not-increased-by-oral-anticoagulation
#10
Maeva Guillaume, Camille Christol, Aurélie Plessier, Michèle Corbic, Jean-Marie Péron, Agnès Sommet, Pierre-Emmanuel Rautou, Yann Consigny, Jean-Pierre Vinel, Charles-Dominique Valla, Christophe Bureau
BACKGROUND AND OBJECTIVES: Noncirrhotic nontumoral extrahepatic portal vein obstruction (EHPVO) is the second leading cause of portal hypertension (PHT) and is mainly related to prothrombotic disorders. Patients with EHPVO often require prolonged oral anticoagulation therapy (OAT) together with variceal band ligation (VBL) to prevent thrombosis recurrence and PHT-related bleeding, respectively. The benefit-risk balance of VBL in this context remains unknown. We aimed to assess upper gastrointestinal bleeding (UGB) risk and variceal eradication efficacy in EHPVO patients undergoing a VBL program without stopping OAT...
January 8, 2018: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/29315065/-congenitally-missing-maxillary-lateral-incisors-long-term-periodontal-and-functional-evaluation-after-orthodontic-space-closure-with-first-premolar-intrusion-and-canine-extrusion
#11
Marco Rosa, Patrizia Lucchi, Simona Ferrari, Bjørn U Zachrisson, Alberto Caprioglio
INTRODUCTION: The aims of this investigation were to evaluate associations between orthodontic space closure (including first premolar intrusion and canine extrusion for esthetic reasons) and periodontal tissue deterioration over a 10-year period in subjects with one or both missing maxillary lateral incisors and to investigate the occurrence of signs or symptoms of temporomandibular disorder (TMD). METHODS: This was a retrospective cohort study comprising patients treated by the same orthodontist...
December 2017: L' Orthodontie Française
https://www.readbyqxmd.com/read/29314552/australian-multicentre-study-of-current-real-world-prophylaxis-practice-in-severe-and-moderate-haemophilia-a-and-b
#12
J A Mason, S Parikh, H Tran, J Rowell, S McRae
INTRODUCTION: With the emergence of novel treatment products for haemophilia and an increasing focus on the benefits of pharmacokinetic driven individualized prophylaxis, robust national data with regard to current patterns of factor consumption and adherence are required. AIM: To characterize current Australian practice with regard to use of prophylactic clotting factor infusions in patients with moderate or severe haemophilia A (HA) and haemophilia B (HB). METHODS: This was a retrospective, non-interventional study utilizing Australian Bleeding Disorder Registry (ABDR) data collected over a 12 month period...
January 3, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29314207/vitiligoid-variant-of-lichen-sclerosus-in-young-girls-with-darker-skin-types
#13
Margaret H Dennin, Sarah L Stein, Adena E Rosenblatt
BACKGROUND/OBJECTIVES: Vitiligo and lichen sclerosus are autoimmune disorders characterized by white discoloration, and both frequently affect the anogenital region. Vitiligoid lichen sclerosus refers to a superficial variant of lichen sclerosus in which the lesion appears clinically to be vitiligo based on the predominant presentation of depigmentation and minimal inflammation and sclerosis but histologically is consistent with lichen sclerosus. A limited number of reports have described vitiligoid lichen sclerosus, and from these reports, it appears to primarily affect darker-skinned people...
January 4, 2018: Pediatric Dermatology
https://www.readbyqxmd.com/read/29313587/hereditary-haemorrhagic-telangiectasia-with-severe-anemia-and-recurrent-cns-infections
#14
Nrushen Peesapati, Pbpr Naidu, S Sunitha, P V Sivaram
Hereditary Haemorrhagic Telangiectasia, also known as Osler-Rendu-Weber disease is a rare autosomal dominant disorder affecting small vessels of multiple systems whose main pathological change is the presence of abnormal arteriovenous communications. Usually presents as skin and mucosal telangiectasias, epistaxis, gastrointestinal bleeding and visceral arteriovenous malformations. Although the epistaxis and gastrointestinal blood loss can result in anaemia, patients with hereditary haemorrhagic telangiectasia rarely presents as severe anaemia1 or CNS infections...
September 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/29309758/anti-thrombotic-efficacy-of-s007-867-pre-clinical-evaluation-in-experimental-models-of-thrombosis-in-vivo-and-in-vitro
#15
Ankita Misra, Prem Prakash, Hobby Aggarwal, Priyanka Dhankani, Sachin Kumar, Chandra Prakash Pandey, Nicholas Pugh, Dominique Bihan, Manoj Kumar Barthwal, Richard W Farndale, Dinesh Kumar Dikshit, Madhu Dikshit
Pharmacological inhibition of platelet collagen interaction is a promising therapeutic strategy to treat intra-vascular thrombosis. S007-867 is a novel synthetic inhibitor of collagen-induced platelet aggregation. It has shown better antithrombotic protection than aspirin and clopidogrel with minimal bleeding tendency in mice. The present study is aimed to systematically investigate the antithrombotic efficacy of S007-867 in comparison to aspirin and clopidogrel in vivo and to delineate its mechanism of action in vitro...
January 5, 2018: Biochemical Pharmacology
https://www.readbyqxmd.com/read/29305925/use-of-therapeutic-hypothermia-among-patients-with-coagulation-disorders-a-nationwide-analysis
#16
Mahek Shah, Kaushal Parikh, Brijesh Patel, Manyoo Agarwal, Lohit Garg, Sahil Agrawal, Shilpkumar Arora, Nilay Patel, Nainesh Patel, William H Frishman
OBJECTIVES: The study aimed to assess the impact of therapeutic hypothermia (TH) on bleeding and in-hospital mortality among patients with coagulation disorders (CD). BACKGROUND: TH affects coagulation factors and platelets putting patients at risk for bleeding and worse outcomes. Effect of TH among patients with CD remains understudied. METHODS: Between 2009 and 2014, a total of 6,469 cases of TH were identified using the National Inpatient Sample out of which 1,036 (16...
January 3, 2018: Resuscitation
https://www.readbyqxmd.com/read/29302279/prior-oral-conditions-in-patients-undergoing-heart-valve-surgery
#17
Francisco-Javier Silvestre, Irene Gil-Raga, Mayte Martinez-Herrera, Dorina Lauritano, Javier Silvestre-Rangil
Background: Patients scheduled for heart valve surgery should be free of any oral infectious disorders that might pose a risk in the postoperative period. Few studies have been made on the dental conditions of such patients prior to surgery. The present study describes the most frequent prior oral diseases in this population group. Material and Methods: A prospective, observational case-control study was designed involving 60 patients (30 with heart valve disease and 30 controls, with a mean age of 71 years in both groups)...
November 2017: Journal of Clinical and Experimental Dentistry
https://www.readbyqxmd.com/read/29301557/development-and-content-validation-of-a-patient-reported-endometriosis-pain-daily-diary
#18
Floortje E van Nooten, Jennifer Cline, Celeste A Elash, Jean Paty, Matthew Reaney
BACKGROUND: Endometriosis is a common gynecological disorder that causes inflammation and pelvic pain. Endometriosis-related pain is best captured with patient-reported outcome (PRO) measures, however, assessment of endometriosis-related pain in clinical trials has been difficult in the absence of a reliable and valid PRO instrument. We describe the development of the Endometriosis Pain Daily Diary (EPDD), an electronic PRO developed as a survey instrument to assess endometriosis-related pain and its impact on patients' lives...
January 4, 2018: Health and Quality of Life Outcomes
https://www.readbyqxmd.com/read/29296746/genetic-variants-in-adamts13-as-well-as-smoking-are-major-determinants-of-plasma-adamts13-levels
#19
Qianyi Ma, Paula M Jacobi, Brian T Emmer, Colin A Kretz, Ayse Bilge Ozel, Beth McGee, Chava Kimchi-Sarfaty, David Ginsburg, Jun Z Li, Karl C Desch
The metalloprotease ADAMTS13 cleaves von Willebrand factor (VWF) in circulating blood, limiting the size of VWF multimers and regulating VWF activity. Abnormal regulation of VWF contributes to bleeding and to thrombotic disorders. ADAMTS13 levels in plasma are highly variable among healthy individuals, although the heritability and the genetic determinants of this variation are unclear. We performed genome-wide association studies of plasma ADAMTS13 concentrations in 3244 individuals from 2 independent cohorts of healthy individuals...
June 27, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296726/novel-approach-to-genetic-analysis-and-results-in-3000-hemophilia-patients-enrolled-in-the-my-life-our-future-initiative
#20
Jill M Johnsen, Shelley N Fletcher, Haley Huston, Sarah Roberge, Beth K Martin, Martin Kircher, Neil C Josephson, Jay Shendure, Sarah Ruuska, Marion A Koerper, Jaime Morales, Glenn F Pierce, Diane J Aschman, Barbara A Konkle
Hemophilia A and B are rare, X-linked bleeding disorders. My Life, Our Future (MLOF) is a collaborative project established to genotype and study hemophilia. Patients were enrolled at US hemophilia treatment centers (HTCs). Genotyping was performed centrally using next-generation sequencing (NGS) with an approach that detected common F8 gene inversions simultaneously with F8 and F9 gene sequencing followed by confirmation using standard genotyping methods. Sixty-nine HTCs enrolled the first 3000 patients in under 3 years...
May 23, 2017: Blood Advances
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