211378 Intracranial hemorrhage in the first two years of life in babies with bleeding disorders

Monday, November 9, 2009: 3:00 PM

J. Michael Soucie, PhD , Centers for Disease Control and Prevention, Atlanta, GA
Roshni Kulkarni, MD , Department of Pediatrics and Human Development, Michigan State University, East Lansing, MI
Thomas Abshire, MD , Emory University School of Medicine, Atlanta, GA
Donna DiMichele, MD , New York Weill Cornell Center, New York, NY
Bruce Evatt, MD , Centers for Disease Control and Prevention (ret), Atlanta, GA
Sue Geraghty, RN, MBA , University of Colorado at Denver and Health Sciences Center, Aurora, CO
Joan C. Gill, MD , The Blood Center of Wisconsin, Milwaukee, WI
W. Keith Hoots, MD , National Institutes of Health, Bethesda, MD
Robert L. Janco, MD , Wyeth Pharmaceuticals, Collegeville, PA
Marion Koerper, MD , University of California San Francisco, San Francisco, CA
Jeanne Lusher, MD , Children's Hospital of Michigan, Detroit, MI
Marilyn Manco-Johnson, MD , University of Colorado at Denver and Health Sciences Center, Aurora, CO
Prasad Mathew, MD , Department of Pediatrics, University of New Mexico, Albuquerque, NM
Diane Nugent, MD , Children's Hospital of Orange County, Orange, CA
Rodney Presley, PhD , Centers for Disease Control and Prevention, Atlanta, GA
Amy D. Shapiro, MD , Indiana Hemophilia & Thrombosis Center, Indianapolis, IN
  
Intracranial hemorrhage (ICH) is a common and serious manifestation in babies with bleeding disorders. We analyzed data collected through the Universal Data Collection System (UDC) by care providers from 135 federally funded hemophilia comprehensive care clinics in the U.S. Over a 6-year period, data were collected on 318 babies who had completed a 2-year enrollment since birth: 94% had hemophilia, 4% had von Willebrand disease, 1.3% had factor VII (FVII) deficiency, and 0.7% had factor XIII (FXIII) deficiency. There were 25 ICHs in 23 babies: 1 in FXIII deficiency, the remainder in hemophilia. The ICH in the FXIII patient was spontaneous, located intra/periventricularly, and resulted in development of a long-term seizure disorder. Among hemophilia B (HemB) patients, 8 of 62 (12.9%) had 9 ICH: 1 had mild, 1 had moderate, 6 had severe HemB; 5 of the 9 ICHs were subdural; 1 each were intracerebral, subarachnoid, epidural and intra/periventricular. Two were associated with delivery; the rest were spontaneous; 1 resulted in long-term focal neurologic deficit. Among hemophilia A (HemA) patients, 15 of 235 (6.4%) had 16 ICH: 1 had mild, 1 had moderate, 13 had severe HemA; 7 of the 16 ICHs were subdural, 3 epidural, 2 intracerebral, 2 intracerebral and subdural, 1 subarachnoid, and 1 cerebellar. Six were associated with delivery, 6 with trauma, 3 spontaneous, and 1 was iatrogenic; 5 babies had long-term effects including seizure disorders, focal neurologic deficits and paralysis. In conclusion, ICH is common in babies with severe bleeding disorders and results in substantial morbidity.

Learning Objectives:
Describe the rate of occurrence of intracranial hemorrhages in babies with bleeding disorders.

Presenting author's disclosure statement:

Qualified on the content I am responsible for because: I have over 15 years of research experience and have developed the surveillance system upon which the study is based.
Any relevant financial relationships? No

I agree to comply with the American Public Health Association Conflict of Interest and Commercial Support Guidelines, and to disclose to the participants any off-label or experimental uses of a commercial product or service discussed in my presentation.

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