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The Congenital Malformations Registry: A Brief Overview

The Congenital Malformations Registry: A Brief Overview. Why Birth Defects?. Relatively common -- 3-5% Leading cause of infant mortality Major cause of childhood mortality Major cause of childhood morbidity Cause of a birth defect not known in 70% of cases

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The Congenital Malformations Registry: A Brief Overview

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  1. The Congenital Malformations Registry: A Brief Overview

  2. Why Birth Defects? • Relatively common -- 3-5% • Leading cause of infant mortality • Major cause of childhood mortality • Major cause of childhood morbidity • Cause of a birth defect not known in 70% of cases • Hope for Prevention - Folic Acid

  3. Why Birth Defects? • Relatively common -- 3-5% • Leading cause of infant mortality • Major cause of childhood mortality • Major cause of childhood morbidity • Cause of a birth defect not known in 70% of cases • Hope for Prevention - Folic Acid

  4. INFANT DEATHS AND MORTALITY RATES FOR THE FIVE LEADING CAUSES OF INFANT DEATH BY RACE AND HISPANIC ORIGIN OF MOTHER: UNITED STATES, 2001 LINKED FILE (Rates Per 100,000 Live Births in Specified Group)

  5. Why Birth Defects? • Relatively common -- 3-5% • Leading cause of infant mortality • Major cause of childhood mortality • Major cause of childhood morbidity • Cause of a birth defect not known in 70% of cases • Hope for Prevention - Folic Acid

  6. Why Birth Defects? • Relatively common -- 3-5% • Leading cause of infant mortality • Major cause of childhood mortality • Major cause of childhood morbidity • Cause of a birth defect not known in 70% of cases • Hope for Prevention - Folic Acid

  7. Why Birth Defects? • Relatively common -- 3-5% • Leading cause of infant mortality • Major cause of childhood mortality • Major cause of childhood morbidity • Cause of a birth defect not known in 70% of cases • Hope for Prevention - Folic Acid

  8. Why Birth Defects? • Relatively common -- 3-5% • Leading cause of infant mortality • Major cause of childhood mortality • Major cause of childhood morbidity • Cause of a birth defect not known in 70% of cases • Hope for Prevention - Folic Acid

  9. Congenital Malformations Registry INFORMATION THAT IS: Complete Accurate Timely Large, well-defined population

  10. Congenital Malformations Registry INFORMATION THAT IS: CHEAP!!

  11. Intensive Mandatory hospital reporting withoutfollow-up and quality control Mandatory hospital reporting withfollow-up and quality control BD Surveillance - United States

  12. BD Surveillance - United States Intensive MHR w/ follow-up & quality control MHR w/o follow-up & quality control Planning or newly implemented None Source: Centers for Disease Control and Prevention (CDC)

  13. Congenital Malformations Registry Background Population Coverage: Statewide approximately 260,000 to 300,000 births annually Designed for surveillance, research & to provide data to health programs to aid in the development of needs assessment Established in October, 1982 as a result of Love Canal One of the largest statewide, population-based registries

  14. Congenital Malformations Registry Case Definition Children diagnosed up to 2 years of age who were born or reside in New York State with a major congenital malformation, chromosomal anomaly or persistent metabolic defect. *List of reportable ICD-9-CM codes available on NYSDOH Public WebSite: www.health.state.ny.us

  15. Congenital Malformations Registry Surveillance System Registry receives approximately 20,000 reports per year on 10,000-11,000 children Cases can be diagnosed with 20+ major malformations Cases can be reported multiple times from one or more hospitals & by physicians up to the age of 2 Reports from all sources are maintained and linked with a unique case number For Sale Electronic Card File 120,000 (5x8) Cards

  16. Current Reporting Sources 180 hospitals account for over 98% of all CMR reports Confidential Case Report Form -67 SPARCS/UDS Electronic Reporting - 21 Health Provider Network (HPN) Internet Reporting - 88 (74% of reports) Confidential Case Report Form

  17. HPN Home Page

  18. CMR Home Page on the HPN

  19. Use HPN Reporting Arnot Ogden Lakeside Memorial Strong Memorial Do Use HPN Reporting All the Rest! HPN Reporting in Fingerlakes Regional Hospitals

  20. See Handouts

  21. NTD SURVEILLANCE, HSA 6 1990-1996 (Prevalence in CMR)

  22. 1. Development of baseline birth defect data and monitoring of trends 2. Identification of geographic areas of concern 3. Identify cases for etiologic studies 4. Planning, Advocacy, Education Uses of the CMR

  23. 5. Studies of economic impact 6. Follow up studies of survival, long term effects 7. Identification of children in need of special services 8. Evaluation of intervention strategies Uses of the CMR - continued

  24. CDC - Fetal Alcohol Syndrome Surveillance NIH/Emory University - Nondisjunction and Etiologies of Trisomy 21 NIH - Folate genes and cardiac defects CDC - Muscular Dystrophy Surveillance CDC - New York Center for Birth Defects Research and Prevention Funded Research

  25. Congressional mandate in 1996 to establish regional Centers for epidemiological research Effort to build etiologic research capacity on existing birth defects surveillance systems Centers for Birth Defects Research and Prevention

  26. Cooperative agreements currently awarded to nine states to: Bolster ongoing surveillance activities Conduct local research studies Collaborate in the National Birth Defects Prevention Study (NBDPS) Centers for Birth Defects Research and Prevention

  27. MA NY IA UT CA NC AR GA TX Centers for Birth Defects Research and Prevention

  28. Population-based case-control study 3 main components Ascertainment of cases Maternal Interview Collection of DNA National Birth Defects Prevention Study (NBDPS)

  29. Orofacial cleft Craniosynostosis Obstructive heart defects Septal heart defects Esophageal atresia National Birth Defects Prevention Study (NBDPS) • Focuses on birth defects with unknown etiology • Excludes chromosomal abnormalities and single-gene disorders • Includes about 30 major types of birth defects including: • Microtia • Spina bifida • Gastroschisis • Omphalocele • Limb defects

  30. CMR match with Early Intervention Evaluation of pulse oximetry screening for cardiovascular malformation in newborns Clinical Guidelines for work up of a child with malformations Follow-up of Children with congenital cardiac defects Past & Present Studies

  31. Risk of birth defects in foreign versus US born Hispanics Evaluation of birth defects surveillance in New York Mode of Delivery of infants with abdominal wall defects and survival Childhood Cancer and Birth Defects Studies Continued*

  32. Caffeine and selected birth defects* Maternal thyroid disease/medications and selected birth defects Maternal asthma/asthma meds and selected birth defects* Maternal chronic hypertension and selected birth defects* Pesticides and limb reduction defects Maternal occupation and oral clefts/limb reduction Maternal Alcohol use and cardiovascular defects* Water disinfection by-products and cardiac defects Planned Analyses

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