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Condition: Haemophilia

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Total 41 results found since Jan 2013.

The Utility of Factor VIII Infusion in a Rare Case of SHAM Syndrome (P4.343)
Conclusions: Genetic testing confirmed the presence of SHAM syndrome with an 83 kb deletion involving both F8 and BRCC3 genes responsible for severe hemophilia and Moyamoya disease respectively. This case illustrates the first phenotypically and genetically confirmed adult case of SHAM syndrome with intracerebral hemorrhage and highlights the utility/safety of factor VIII infusion in this case.Disclosure: Dr. Roh has nothing to disclose. Dr. Roth has nothing to disclose. Dr. Al-Mufti has nothing to disclose. Dr. Chung has nothing to disclose. Dr. Connolly has nothing to disclose. Dr. Eisenberger has nothing to disclose. Dr...
Source: Neurology - April 3, 2016 Category: Neurology Authors: Roh, D., Roth, W., Al-Mufti, F., Chung, W., E. Sander, C., Eisenberger, A., Park, S., Claassen, J., Agarwal, S. Tags: Cerebrovascular Case Reports Source Type: research

The Man Who Grew Eyes
The train line from mainland Kobe is a marvel of urban transportation. Opened in 1981, Japan’s first driverless, fully automated train pulls out of Sannomiya station, guided smoothly along elevated tracks that stand precariously over the bustling city streets below, across the bay to the Port Island. The island, and much of the city, was razed to the ground in the Great Hanshin Earthquake of 1995 – which killed more than 5,000 people and destroyed more than 100,000 of Kobe’s buildings – and built anew in subsequent years. As the train proceeds, the landscape fills with skyscrapers. The Rokkō mounta...
Source: Science - The Huffington Post - October 11, 2015 Category: Science Source Type: news

Incidence of ischemic or hemorrhagic non-fatal strokes in hemophiliac adults and teenage children (P7.127)
Conclusions: The rate of stroke appears to be low in adults and teenage children in a cohort of hemophiliacs with predominantly moderate and severe disease.Disclosure: Dr. Bezzina has nothing to disclose. Dr. Malik has nothing to disclose. Dr. QURESHI has nothing to disclose.
Source: Neurology - April 8, 2015 Category: Neurology Authors: Bezzina, C., Malik, A., Qureshi, A. Tags: Cerebrovascular Disease and Interventional Neurology: Stroke in Young Source Type: research

Acquired Hemophilia A Associated with Internal Border-zone Infarction Triggered by Major Hemorrhage of Thigh and Leg: A Case Report
We describe a first case of internal border-zone infarction (I-BZI) complicated with acquired hemophilia A. A 79-year-old man was introduced from other hospital by cerebral infarction and severe anemia. His left thigh and leg were swollen with subcutaneous bleeding. Activated partial thromboplastin time was 99.4 seconds. Factor VIII activity was less than 1% and Factor VIII inhibitor concentration was 85 BU, respectively.
Source: Journal of Stroke and Cerebrovascular Diseases - October 10, 2014 Category: Neurology Authors: Masayuki Miyazaki, Shuuhei Nagami, Hisao Shimizu, Nobuyuki Takahashi Tags: Case Report Source Type: research

Atrial fibrillation in patients with haemophilia: a cross‐sectional evaluation in Europe
Summary Atrial fibrillation (AF) is a common health problem in the general population, but data on prevalence or management in patients with haemophilia (PWH) are lacking. The aims of this study were to analyse the prevalence of AF and risk factors for stroke using a cross‐sectional pan‐European design and to document current anticoagulation practice. The ADVANCE Working Group consists of members from 14 European haemophilia centres. Each centre retrieved data on their PWH with AF. From the total of 3952 adult PWH, 33 had AF with a mean age of 69 years (IQR 62–76). Haemophilia was severe in seven (21%), moderate in ...
Source: Haemophilia - March 1, 2014 Category: Hematology Authors: Roger E. G. Schutgens, R. Klamroth, I. Pabinger, M. Malerba, G. Dolan, Tags: Original Article Source Type: research

Cardiovascular comorbidities are increased in US patients with haemophilia A: a retrospective database analysis
This study evaluated the prevalence of cardiovascular comorbidities among USA males with haemophilia A, relative to an unaffected general male population with similar characteristics. Males with haemophilia A and continuous insurance coverage were identified by ICD‐9‐CM code 286.0 (1 January 2007–31 December 2009) using the MarketScan® Commercial and Medicare Research Databases. Individuals with haemophilia A were exact matched 1:3 with males without a diagnosis of haemophilia A. The prevalence of cardiovascular comorbidities identified by ICD‐9‐CM code was determined for matched cohorts. Of the study population...
Source: Haemophilia - November 29, 2013 Category: Hematology Authors: J. Pocoski, A. Ma, C. M. Kessler, S. Boklage, T. J. Humphries Tags: Original Article Source Type: research

Heparin, grad students, a clinical revolution and giving credit where it's due
The story of a grad student who overcame remarkable odds only to be denied his moment of glory, or a tale of dark deceit and devilish doings? The story of heparin is as complicated as the chemistry itselfBlood is remarkable.A liquid that carries nutrients, waste products and the ever-vigilant cells of the immune system around the body, blood rapidly turns into a solid when it leaves its veins and arteries and becomes exposed to bodily tissues or the air outside. This process of solidification – clotting, or coagulation – is executed and controlled by a complex set of reactions and interactions primarily involving the e...
Source: Guardian Unlimited Science - September 4, 2013 Category: Science Authors: Richard P Grant Tags: theguardian.com Blogposts Science Source Type: news

Associated Risk of Recombinant Activated Factor VIIa Application.
Conclusion: The proof of direct causality of the events in relation to the administration of FVIIa is difficult because the temporal and therapeutic relationships with concomitant vasoconstrictive and procoagulant therapies were not obvious. However, there remains a suspicion that a higher rate of mesenteric infarctions may be provoked by the administration of FVIIa. PMID: 23803235 [PubMed - in process]
Source: The Heart Surgery Forum - June 1, 2013 Category: Cardiovascular & Thoracic Surgery Authors: Kiessling AH, Nitsch J, Strouhal U, Kornberger A, Zierer A, Moritz A Tags: Heart Surg Forum Source Type: research

Gene breakthrough boosts hope of drug for blood diseases
Discovery could prevent lethal blood clots and help children with rare platelet function disorderNoah Edwards is four years old and suffers from a disorder that prevents his blood from clotting. He bleeds profusely when cut and his face and body are easily bruised.His condition, called platelet function disorder, is a constant worry for his mother, Ruby. However, thanks to Noah's involvement in a remarkable project, funded by the British Heart Foundation, his prospects of leading a normal life have received a major boost.Researchers at Birmingham University have uncovered the genetic roots of platelet function disorder, a ...
Source: Guardian Unlimited Science - January 13, 2013 Category: Science Authors: Robin McKie Tags: University of Birmingham Genetics Biology News Medical research Higher education UK news The Observer Science Source Type: news

Thrombocytopenia and In-hospital Mortality Risk among Ischemic Stroke Patients
Background: Thrombocytopenia has been associated with increased mortality in nonstroke conditions. Because its role in acute ischemic stroke is less well understood, we sought to determine whether thrombocytopenia at admission for acute ischemic stroke was associated with in-hospital mortality.Methods: We used data from a retrospective cohort of stroke patients (1998-2003) at 5 U.S. hospitals. Risk factors considered included conditions that can lead to thrombocytopenia (e.g., liver disease), increase bleeding risk (e.g., hemophilia), medications with antiplatelet effects (e.g., aspirin), and known predictors of mortality ...
Source: Journal of Stroke and Cerebrovascular Diseases - September 12, 2012 Category: Neurology Authors: Jason J. Sico, Michael S. Phipps, John Concato, Carolyn K. Wells, Albert C. Lo, Steven E. Nadeau, Linda S. Williams, Aldo J. Peixoto, Mark Gorman, John L. Boice, Dawn M. Bravata Tags: Original Articles Source Type: research