Search

Abstract 2231 Background Child maltreatment is a frequent cause of injury in the United States, occurring in approximately 695,000 children per year. Bleeding disorders can exacerbate and be confused with non-accidental injury (NAI); both of these diagnostic errors are life altering for the child and family. Despite the high incidence of child abuse and the independent relative high incidence of bleeding disorders, the optimal hemostatic evaluation is unclear for children who may be victims of NAI. Expert hematologic opinion recommends a multi-tiered approach, first investigating for common bleeding disorders, and subsequently investigating for rare defects in the coagulation and fibrinolytic pathways, if necessary. Further research is needed to develop evidence-based guidelines for the evaluation of bleeding disorders in children who may be victims of NAI. Objectives To review and analyze a five-year history of the hematologic investigation of children who presented with bleeding and/or bruising that was suspicious for NAI at Vanderbilt Children's Hospital (VCH). Our hypothesis is that there is a lack of a systematic approach for the hemostatic evaluation of children who present with bleeding symptoms and concern for NAI. Methods A retrospective cohort study design was employed. ICD-9 codes for NAI (995.5, 995.50, 995.54, 995.55, 995.59) were used. 354 medical records from 2007 – 2011 were reviewed and screened for inclusion and exclusion criteria, resulting in 198 fully evaluable patients. Medical records were then queried for details of clinical and laboratory evaluation that occurred at the initial presentation concerning for NAI. We defined a basic hematologic evaluation as a CBC, PT and PTT; and a comprehensive hematologic evaluation as a CBC, PT, PTT, factor VIII, IX and XI activity and von Willebrand evaluation. Data was analyzed using SPSS; statistical analysis was performed using frequencies and Chi-Square analysis. Outcomes The mean age for the studied population was 445 days (max 4687 days, minimum 6 days); 37% were male. Bleeding symptoms included intracranial hemorrhage (ICH) (40%) and bruising (58% without associated ICH, 73% with and without associated ICH), with approximately 60% demonstrating additional non-hematologic symptoms (i.e. fractures, burns). Hematologic laboratory tests performed included CBC in 66%, PT in 58%, and PTT in 55%; factor activity levels in 12% (primarily consisted of factors VIII and IX); and von Willebrand disease evaluation in 12% of subjects. Table 1 shows the percentage of laboratory tests obtained in the patients based on symptoms at presentation. Abnormal coagulation labs were seen in 33% of performed PT and 55% of PTT tests; 55% of abnormal PTs and 44% of abnormal PTTs repeated. Our defined basic evaluation was completed in 79% of patients with ICH and 36% of patients without ICH (p Conclusion Complete hematologic evaluation of children who present with bleeding symptoms and concern for NAI is inconsistent. While some children with other findings diagnostic for NAI may not require a hematologic work-up from a medico-legal perspective, it is still prudent to consider common bleeding disorders as a potential contributing factor in the severity of symptoms. At VCH, laboratory evaluation was obtained with greater frequency in patients with hematologic symptoms only. Given the variability of tests obtained and the disparity between expert opinion and our historical evaluation, further investigation into the optimal evaluation of these patients is warranted at this time. A prospective cohort study would allow comprehensive evaluation of all children suspected of NAI resulting in a clear understanding of laboratory abnormalities and incidence of bleeding disorders. Disclosures: No relevant conflicts of interest to declare.

Abstract 3385 Introduction: Hemophilia A, the result of reduced factor VIII (FVIII) activity, is an X-linked recessive bleeding disorder affecting one in 5,000 males born in the United States (U.S.). Females are designated heterozygous or carriers. While bleeding symptoms have been fully characterized in male patients with Hemophilia A, less is known about the female Hemophilia A carrier bleeding phenotype. Previous reports in Hemophilia A carriers suggest an increased bleeding tendency regardless of residual FVIII activity. Further, small studies suggest that Hemophilia A carriers report excessive bleeding symptoms including, but not limited to menorrhagia, epistaxis, surgical bleeding and hemarthrosis. Objective: To determine the attitudes and understanding of Hemophilia A carrier testing from the perspective of both providers and affected patients. Our global hypothesis is that genetically verified Hemophilia A carriers with normal FVIII activity will have increased clinically relevant bleeding. Methods: We employed a cross-sectional study design, and developed an electronic survey that was emailed to physicians and nurses employed at U.S. Hemophilia Treatment Centers (HTC). The email list was generated from listed current HTC providers on www.cdc.gov. A similar electronic survey was developed and distributed to female Hemophilia A carrier members of Hemophilia Federation of America. Questions were derived from the Female Universal Data Collection project questionnaire, and focused on the clinical understanding and management of Hemophilia A carriers. In addition, questions were developed to assess attitudes regarding the timing and intensity of testing. Data was analyzed using SPSS with descriptive statistics, Wilcoxon rank sum test and Pearson test. Results: In the provider survey there was a 49%(42/85) response rate from the physicians and 37% response rate (30/80) from nurses. Combined, 64% (47/72) had >10 years of clinical experience, 94% (68/72) work in primarily within an HTC and 97% (69/71) offer carrier testing. In the survey aimed at Hemophilia A carriers, a total of 38 responses were analyzed. The mean age was 35 ± 11 years, 76% (29/38) had a biologic son with Hemophilia A with 65% (24/37), 22% (8/37) and 14% (5/37) being severe, moderate or mild in severity. Carrier testing was performed after the age of 14 in 82% of those queried. When asked about the optimal timing of carrier testing, 78% (31/41) of physicians and 68% (19/28) of nurses recommend testing after 14 years of age while 69% (24/35) of carriers prefer testing to be done prior to this age (p While 78% (28/36) of the carrier's affected hemophiliac relatives receive care at an HTC only 26% (9/35) receive regular Hemophiliac care, and 67% (6/9) of those receiving regular care are followed at the same location as their affected hemophiliac relative. When queried, 51% (36/51) of providers compared to 78% (28/36) of carriers believe that Hemophilia A carriers with normal FVIII activity have an increased bleeding tendency (p Conclusion: Hemophilia A carriers report a higher frequency of bleeding than previously acknowledged. In contrast to preferences of Hemophilia specialists, Hemophilia A carriers prefer to know their carrier status prior to 14 years of age and be cared for in the same location as their affected relatives. Under recognition of Hemophilia A carrier bleeding is common amongst HTC providers, and data collected in a prospective cohort with appropriate controls is warranted. Disclosures: No relevant conflicts of interest to declare.