32 research outputs found
Intermittent injections vs. continuous infusion of Factor VIII in haemophilia patients undergoing major surgery
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Updates in the management of severe coagulopathy in trauma patients
Coagulopathy is the major cause of bleeding-related mortality in patients who survive the operating room. Its association with hypothermia and metabolic acidosis is common and constitutes a vicious cycle. Usually, post-traumatic coagulopathy is an early event and may be present during surgery. The pathogenesis of severe post-traumatic coagulopathy is complex and multifactorial. Virtually every aspect of the normal coagulation cascade is affected in the cold, acidotic, exsanguinating trauma patient. In the last decade many surgeons have emphasized the role of prevention or early treatment of this vicious cycle. Damage control surgery with planned re-operations has demonstrated superiority over the traditional approach in cases where the patients' condition is deteriorating. Early control of surgical bleeding and significant contamination, together with vigorous correction of hypothermia and continuous resuscitation, has improved the survival of these patients. Recently, a new adjunct to the treatment of coagulopathy in trauma patients has been reported and is undergoing controlled animal trials. Recombinant activated factor VII (rFVIIa) was originally developed as a pro-hemostatic agent for the treatment of bleeding episodes in hemophilia patients. rFVIIa has been successfully used in moribund trauma patients in whom standard procedures had failed to correct bleeding. Preliminary preclinical and clinical studies are under way
Survival of hepatitis C-infected haemophilia patients is predicted by presence of cirrhosis but not by anti-viral treatment
Background/Purpose. Hepatitis C (HCV) is a major cause of morbidity and mortality in haemophilia patients who received clotting factor concentrates before the availability of virus-inactivated factors in the mid-1980s. Recently, it has been suggested that anti-HCV treated patients, particularly those achieving a sustained virological response (SVR) have an improved outcome. We sought to examine the survival of treated and untreated HCV-infected haemophilia patients.Material and methods. We studied overall and liver-related survival of patients with haemophilia and other congenital bleeding disorders between 2000 and 2010. The outcome was compared in 3 sub-groups: HCV mono-infected (N = 127), HCV/HIV co-infected (N = 28), and patients with either HCV-antibodies negative or persistent HCV RNA-negative (referred to as non-infected) (N = 45). Sixty-two (40%) (HCV and HCV/HIV) patients underwent anti-HCV treatment with an SVR rate of 40.3%.Results. Overall and liver-related 10-year survival were: 82.1 and 89.3%, 95.3 and 99.2 and 100% for HCV/HIV co-infected, HCV mono-infected and non-infected haemophilia patients, respectively (p = 0.015 and 0.023 for comparisons of HCV/HIV vs. HCV; p = 0.003 for comparison of HCV/HIV and non-infected). One HCV mono-infected and 3 co-infected patients died of end-stage liver disease (2 underwent liver transplantation). There was no survival benefit from anti-HCV treatment or from attaining of an SVR. Only clinically suspected cirrhosis remained as an independent predictor of survival.Conclusion. The prognosis of haemophilia patients who acquired HCV/HIV co-infection is worse than that of HCV mono-infected or non-infected or haemophiliacs. This is mainly due to liver-related mortality. Anti-HCV treatment or SVR had no observable impact on survival rate
An Assay of the Antithrombotic Action of Warfarin: Its Correlation with the Inhibition of Stasis Thrombosis in Rabbits
The molecular genetics of haemophilia A: screening for point mutations in the factor VIII gene using the restriction enzyme TaqI
A combination of Southern blotting and the analysis of polymerase chain reaction (PCR) amplified DNA fragments was used to screen the factor VIII genes of 527 haemophilia A patients for point mutations within TaqI restriction sites. Since this “directed search” strategy yielded only four gene lesions, it was concluded that its efficacy is less than that originally predicted. One novel point mutation was however found in a moderately severe haemophiliac; a CGA (Arg) to CTA (Leu) transversion at codon 2209, an evolutionarily conserved residue in the C2 domain of the factor VIII protein. The remaining three detected lesions, CGA (Arg)→TGA (Term) transitions at codons 2116, 2147 and 2307, respectively, have been reported before and are consistent with recurrent mutation at these hypermutable sites. A number of TaqI restriction site polymorphisms/rare variants were also noted. These variants appear to be population-specific but are nevertheless potentially useful in individual cases as intragenic markers for carrier detection and antenatal diagnosis
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