Replacement therapy for coronary artery bypass surgery in patients with hemophilia A and B

Replacement therapy for coronary artery bypass surgery in patients with hemophilia A and B INTRODUCTIONHemophilia A and B are X‐linked congenital bleeding disorders caused by mutations in clotting factor genes that result in deficiency of coagulation factor VIII (FVIII) and factor IX (FIX), respectively. The severity of bleeding is generally correlated with the clotting factor levels. The phenotype is severe when the clotting factor levels are lower than 1%, moderate when the levels are 1‐5%, and mild when the levels are 5‐40% of normal. Replacement of the deficient factor using specific factor concentrates, either plasma‐derived or recombinant, is a mainstay of therapy. Treatment consists of on‐demand regimens during bleeding episodes, either prophylactic treatment before invasive procedures or long‐term in patients with severe phenotype. Currently, the most serious and costly complication of factor replacement treatment is the development of antibodies, entitled inhibitors, to the infused factor. The presence of inhibitors typically makes factor replacement ineffective, and special treatment with bypassing agents such as activated prothrombin complex and activated factor VII concentrate is essential in managing bleeding events.Due to the improvement in the management of patients with hemophilia (PWH) A and B after the induction of factor concentrates the life expectancy of PWH has increased and is comparable to that among the non‐hemophilia population. Therefore, http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Journal of Cardiac Surgery Wiley

Replacement therapy for coronary artery bypass surgery in patients with hemophilia A and B

Loading next page...
 
/lp/wiley/replacement-therapy-for-coronary-artery-bypass-surgery-in-patients-0nospyatQx
Publisher
Wiley
Copyright
© 2018 Wiley Periodicals, Inc.
ISSN
0886-0440
eISSN
1540-8191
D.O.I.
10.1111/jocs.13530
Publisher site
See Article on Publisher Site

Abstract

INTRODUCTIONHemophilia A and B are X‐linked congenital bleeding disorders caused by mutations in clotting factor genes that result in deficiency of coagulation factor VIII (FVIII) and factor IX (FIX), respectively. The severity of bleeding is generally correlated with the clotting factor levels. The phenotype is severe when the clotting factor levels are lower than 1%, moderate when the levels are 1‐5%, and mild when the levels are 5‐40% of normal. Replacement of the deficient factor using specific factor concentrates, either plasma‐derived or recombinant, is a mainstay of therapy. Treatment consists of on‐demand regimens during bleeding episodes, either prophylactic treatment before invasive procedures or long‐term in patients with severe phenotype. Currently, the most serious and costly complication of factor replacement treatment is the development of antibodies, entitled inhibitors, to the infused factor. The presence of inhibitors typically makes factor replacement ineffective, and special treatment with bypassing agents such as activated prothrombin complex and activated factor VII concentrate is essential in managing bleeding events.Due to the improvement in the management of patients with hemophilia (PWH) A and B after the induction of factor concentrates the life expectancy of PWH has increased and is comparable to that among the non‐hemophilia population. Therefore,

Journal

Journal of Cardiac SurgeryWiley

Published: Jan 1, 2018

Keywords: ; ; ; ; ;

References

You’re reading a free preview. Subscribe to read the entire article.


DeepDyve is your
personal research library

It’s your single place to instantly
discover and read the research
that matters to you.

Enjoy affordable access to
over 18 million articles from more than
15,000 peer-reviewed journals.

All for just $49/month

Explore the DeepDyve Library

Search

Query the DeepDyve database, plus search all of PubMed and Google Scholar seamlessly

Organize

Save any article or search result from DeepDyve, PubMed, and Google Scholar... all in one place.

Access

Get unlimited, online access to over 18 million full-text articles from more than 15,000 scientific journals.

Your journals are on DeepDyve

Read from thousands of the leading scholarly journals from SpringerNature, Elsevier, Wiley-Blackwell, Oxford University Press and more.

All the latest content is available, no embargo periods.

See the journals in your area

DeepDyve

Freelancer

DeepDyve

Pro

Price

FREE

$49/month
$360/year

Save searches from
Google Scholar,
PubMed

Create lists to
organize your research

Export lists, citations

Read DeepDyve articles

Abstract access only

Unlimited access to over
18 million full-text articles

Print

20 pages / month

PDF Discount

20% off