tm national center on birth defects and developmental disabilities kernicterus surveillance...
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National Center on Birth Defects and Developmental DisabilitiesNational Center on Birth Defects and Developmental DisabilitiesNational Center on Birth Defects and Developmental DisabilitiesNational Center on Birth Defects and Developmental Disabilities
Kernicterus Surveillance Kernicterus Surveillance
Presented at the FDA Pediatric Advisory Committee Meeting onCurrent Epidemiology and Therapeutic Interventions Relevant to
Hyperbilirubinemia in the Term & Near-term NewbornWashington DC: June 11, 2003
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Overview: Framework for DD surveillanceOverview: Framework for DD surveillance
• Health education & prevention strategies
• Public policy
• Monitor prevalence rates, trends, & prevention programs
• Provide registry of cases
Surveillance
Systems
Epidemiological Studies
Prevention Programs
• Identify risk & protective factors
• Address public concerns
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Definitions– Attributable to an impairment in physical,
cognitive, speech or language, psychological, or self-care areas
Measurement – Not always straight forward, especially for
conditions that rely on a behavioral patterns Population-based– Limited surveillance and not always well defined
Above issues can make generalizing results difficult or impossible to interpret
Overview: Complexities of DD surveillanceOverview: Complexities of DD surveillance
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Issues: Complexities of KI surveillanceIssues: Complexities of KI surveillance
Range of impairment and associated conditions– Brain damage that is associated with athetoid
cerebral palsy, hearing loss, vision impairment, dental dysplasia, and sometimes mental retardation
Changes in level of awareness and use of diagnosis over time
Variability in how cases are diagnosed– Physical markers and behavioral pattern utilized
for identification Early onset but often delayed diagnosis
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No systematic population-based surveillance in place to monitor kernicterus or hyperbilirubinemia
Case reports from convenience samples or select populations (e.g., medical insurance records)
No accepted standard for surveillance definition
Historical perspectives: KI surveillanceHistorical perspectives: KI surveillance
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True population estimates unknown to date Case reports from a convenience sample– Johnson, Bhutani, Brown (2002). 90 cases
reported from 1984-Jan 2001 Rates versus numbers
Prevalence of kernicterusPrevalence of kernicterus
– Rate = # of people with condition
# of people in the population Is kernicterus on the rise?
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Summary of issues for KI surveillanceSummary of issues for KI surveillance
Case definition– Debate over cut-offs for inclusion
Low prevalence condition– Requires a substantial population to detect cases
Lack of recognition– Acute event with specific features– Permanent damage, long-term clinical features
Litigation– Possible deterrent for case identification
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Evaluation of extant databases– National Hospital Discharge Data
Preponderance of hyperbilirubinemia codes KI codes not readily used
– MADDSP surveillance Examined athetoid CP cases
Kernicterus is not a reportable condition– CSTE approached regarding national reporting
Managed Care System– Proposed cooperative agreement– Limited interest
Preliminary actions: CDCPreliminary actions: CDC
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PERT announcement 2002 Objectives– Applicant should seek to review cases of extreme
jaundice in otherwise healthy full-term infants – Provide a body of evidence to inform why cases of
extreme jaundice may lead to kernicterus and why kernicterus may be re-emerging
– A forum of concerned scientists and health-care professionals will be convened to develop a strategic plan for national kernicterus prevention
Current activities: CDC surveillance effortsCurrent activities: CDC surveillance efforts
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UMDNJ Robert Wood Johnson Medical SchoolPrincipal Investigators: T. Hegyi, B. Ostfeld, A. Petrova
Infant mortality and morbidity of kernicterus Surveillance Identification of risk factors (clinical & systemic) Support network
Objectives of award recipientObjectives of award recipient
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UMDNJ Robert Wood Johnson Medical SchoolPrincipal Investigators: T. Hegyi, B. Ostfeld, A. Petrova
IRB submissions and approvals Initial discussion & process for population-based
surveillance with the NJ Dept of Health Analyses of extant data on infant morbidity and
mortality due to kernicterus
Progress to dateProgress to date
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Preliminary results: MorbidityPreliminary results: Morbidity
UMDNJ Robert Wood Johnson Medical SchoolPrincipal Investigators: T. Hegyi, B. Ostfeld, A. Petrova
1992 – 2001(NJ Hospital Discharge Data)– 82 cases of kernicterus– 7.5/100,000 live births (cumulative incidence)– Significant variation by race and ethnicity with the
lowest rate among Hispanics and the highest rate among Asians
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Pennsylvania Hospital, University of PennsylvaniaPrincipal Investigators: V. Bhutani & L. Johnson
Surveillance Identification of risk factors (clinical & systemic) Prevention Taskforce/Steering Committee National Prevention Campaign
Objectives of award recipientObjectives of award recipient
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Pennsylvania Hospital, University of Pennsylvania Principal Investigators: V. Bhutani & L. Johnson
Initial Advisory Board teleconference Peer Review Group meeting for consensus on
kernicterus definition (planned) Database development Collaboration with PICK on kernicterus
educational video
Progress to dateProgress to date
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Future directionsFuture directions
Partner for prevention– American Academy of Pediatrics, AWHONN,
HRSA, JCAHO, March of Dimes, NICHD, PICK, University researchers, others…
Forum for developing consensus on surveillance case definition
Identify a mechanism for population-based surveillance– State– National