ABSTRACT
Intracranial hemorrhage (ICH) is a significant complication for children with hemophilia. Identifying risk factors may allow us to establish clinically relevant guidelines for the diagnosis and management of ICH. The purpose of this review is to nucleate evidence from the available literature on the incidence, risk factors, presentation, treatment, and outcomes of ICH that can be utilized to develop a clinically useful framework for the diagnosis and management of hemophiliac patients with the condition. An electronic MEDLINE and EMBASE literature search was undertaken using the key words 'intracranial hemorrhage and hemophilia' and setting limits as: Last 10 years and Review or Randomized Controlled Trial (RCT) or Clinical Trial, or Practice Guidelines. Following review of all articles using predetermined search words and criteria, 31 were retrieved with sufficient data to address our objectives. An algorithm is presented for the management of children (≥3 years-18 years) with hemophilia and suspected ICH. A standardized approach to ICH may reduce unnecessary exposure to radiation via computed tomography scan in a select group of children. Currently there is limited scientific evidence to recommend a diagnostic and therapeutic algorithm for neonates with hemophilia.
Subject(s)
Hemophilia A/complications , Intracranial Hemorrhages/etiology , Adolescent , Algorithms , Birth Injuries/complications , Blood Coagulation Factors/therapeutic use , Case-Control Studies , Child , Child, Preschool , Comorbidity , Craniocerebral Trauma/complications , Disease Management , Factor IX/antagonists & inhibitors , Factor IX/immunology , Factor IX/therapeutic use , Factor VIII/antagonists & inhibitors , Factor VIII/immunology , Factor VIII/therapeutic use , Female , Humans , Incidence , Infant , Infant, Newborn , Intracranial Hemorrhages/diagnostic imaging , Intracranial Hemorrhages/economics , Intracranial Hemorrhages/epidemiology , Intracranial Hemorrhages/therapy , Isoantibodies/blood , Length of Stay , Male , Meta-Analysis as Topic , Neonatal Screening , Randomized Controlled Trials as Topic , Recurrence , Risk Factors , Tomography, X-Ray Computed , Treatment OutcomeABSTRACT
BACKGROUND: In response to the transfusion- transmitted AIDS epidemic, Canadian authorities recommended the development of tracking systems and improved reporting of adverse events. This study describes the development of a verifiable and comprehensive regional tracking system for coagulation factor concentrates. STUDY DESIGN AND METHODS: The Hamilton- Niagara Regional Hemophilia Program received distribution and utilization data from Canadian Blood Services, 26 regional hospitals, and 70 individuals with bleeding disorders on home-based therapy. Purpose-specific software, the Canadian Hemophilia Assessment and Resource Management System (CHARMS), was used to store, monitor, analyze, and validate data. RESULTS: During a 1-year period (2001), all factor concentrates distributed in this region were accounted for. A higher proportion of FVIII and FIX concentrates (88 vs. 12%) was infused at home than in hospitals, and a higher proportion (63 vs. 28%) was used to prevent than to treat bleeds. During a period of shortage, a 5-percent reduction in utilization of both FVIII and FIX concentrates was documented. One recall was managed swiftly and efficiently. Two patients reported allergic skin reactions. CONCLUSION: A verifiable tracking system has been developed that can provide ongoing data for both clinical and administrative purposes. Data collection from patients needs to be made more efficient and real-time recording is an important future objective. Such a system can be instituted locally for less than 1.5 percent of the cost of the factor concentrate used.
