Details:

Alternative Title:
Haemophilia
Personal Author:
Puetz, John ; Soucie, J. Michael ; Kempton, Christine L. ; Monahan, Paul E.
Puetz, John ; Soucie, J. Michael ; Kempton, Christine L. ; Monahan, Paul E. Less -
Corporate Authors:
Hemophilia Treatment Center Network (HTCN) Investigators
Description:
Background

Several risk factors for inhibitors have recently been described for hemophilia A. It has been assumed that similar risk factors are also relevant for hemophilia B, but there is limited data to confirm this notion.

Objectives

To determine the prevalence of and risk factors associated with inhibitors in hemophilia B

Methods

The database of the Universal Data Collection (UDC) project of the Centers for Disease Control for the years 1998 – 2011 was queried to determine the prevalence of inhibitors in hemophilia B subjects. In addition, disease severity, race/ethnicity, age, factor exposure, and prophylaxis usage were evaluated to determine their impact on inhibitor prevalence.

Results

Of the 3800 male subjects with hemophilia B enrolled in the UDC database, 75 (2%) were determined to have an inhibitor at some point during the study period. Severe disease (OR 13.1, 95% CI 6.2-27.7), black race (OR 2.2, 95% CI 1.2-4.1), and age less than 11 (OR 2.5, 95% CI 1.5-4.0) were found to be significantly associated with having an inhibitor. There was insufficient data to determine if type of factor used and prophylaxis were associated with inhibitors.

Conclusions

Inhibitors in hemophilia B are much less prevalent than hemophilia A, especially in patients with mild disease. Similar factors associated with inhibitors in hemophilia A also seem to be present for hemophilia B. The information collected by this large surveillance project did not permit evaluation of potential risk factors related to treatment approaches and exposures, and additional studies will be required.


Subject:
[+]
Adolescent
Adult
Article
Child
Child, Preschool
Databases, Factual
Ethnicity
Factor IX
Hemophilia B
Humans
Infant
Inhibitors
Male
Middle Aged
Prevalence
Race
Risk Factors
UDC
Young Adult

Source:
Haemophilia. 20(1):25-31
Pubmed ID:
23855900
Pubmed Central ID:
PMC4520536
Document Type:
Journal Article
Funding:
CC999999/Intramural CDC HHS/United States
Collection(s):
CDC Public Access
Main Document Checksum:
[+]
urn:sha256:1c60f58d6519bb8e6d5b431989604432f984ba0716cdf885a381303ef0b94097
File Type:

Supporting Files

• 	nihms-709978-f0001.gif	gif
  	nihms-709978-f0001.jpg	jpeg
  	nihms-709978.nxml	xml

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