Georgia epidemiology report, Vol. 12, no. 3 (Mar. 1996)

Georgia Epidemiology Report

The Georgia Epidemiology Report is a publication of the Epidemiology Section of the Epidemiology and Prevention Branch, Division of Public Health, Department of Human Resources

March 1996

Volume 12 Number 3

Division Of Public Health
Patrick J. Meehan, M.D. - Director

Congenital Anomalies In Georgia

Epidemiology and Prevention Branch State Epidemiologist Kathleen E. Toomey, M.D., M.P.H.- Director

An estimated 1 in 33 newborns in Georgia is diagnosed with a major congenital anomaly. Each year this amounts to about 3,300 infants whose future well-being and even survival may be challenged. Infants with congenital anomalies often need spe-

Epidemiology Section Paul A. Blake, M.D., M.P.H.-Director

cial medical care, and account for 25-30% of all pediatric admissions nationwide, and for an estimated yearly expenditure of 1.4 billion dollars1 . Congenital anomalies are

Surveillance

the leading cause of infant mortality in Georgia (Figure 1), accounting for 18% of all

Jeffrey D. Berschling, M.P.H.; Karen R. Horvat, infant deaths in recent years.
M.P.H.; Jane E. Koehler, D.V.M, M.P.H.; Patrick L.

Osewe, M.D., M.P.H.; Preeti Pathela, M.P.H.; Russell C. Sexton Jr., M.H.S.; Sabrina Walton,

Figure 1. Causes of Infant Mortality, Georgia 1990-1994

M.S.P.H.

Chronic Disease

Nancy E. Stroup, Ph.D.-Director

Patricia M. Fox, M.P.H.; David M. Homa, Ph.D.,

M.P.H.; Thomas W. McKinley, M.P.H.; Edward E.

Pledger, M.P.A.; D. Lee Warner, M.P.H.

Tuberculosis

Bharat K. Pattni, M.B.B.S., M.P.H.

HIV\AIDS

Awal D. Khan, Ph.D., M.A.

Sexually Transmitted Diseases

Quimby E. McCaskill, M.P.H.; Dhelia Williamson,

M.S.

Office of Perinatal Epidemiology

Roger W. Rochat, M.D. - Director

Mary D. Brantley, M.P.H.; Raymond E. Gangarosa,

M.D., M.P.H.; Rebekah Hudgins, M.P.H.; Mary P.

Mathis, Ph.D., M.P.H.; Florina Serbanescu, M.D.;

Edward F. Tierney, M.P.H.

Preventive Medicine Residents Lorenzo D. Botto, M.D.; Isabella A. Danel, M.D., M.S.; Hector S. Izurieta, M.D., M.P.H.;Michael M. McNeal, M.D., M.P.H.; Peter Strebel, M.D.;Sherrilyn Wainwright, M.D.;
EIS Officers Luis G. Castellanos, M.D., Ph.D; Patricia M. Dietz, Dr.P.H.

LBW-low birthweight;RDS-Respiratory Distress Syndrome;SIDS-Sudden Infant Death Syndrome Source: Birth/Infant Death Linked files, Division of Public Health

Georgia Epidemiology Report Editorial Board
Editorial Executive Committee Patrick L. Osewe, M.D. - Editor Kathleen E. Toomey, M.D., M.P.H. Mary D. Brantley, M.P.H. Jeffrey D. Berschling, M.P.H.
Mailing List Edward E. Pledger, M.P.A.

The public is often concerned about environmental problems that may cause birth defects. Public concern about potential environmental causes of congenital anomalies, such as toxic-waste sites, the Gulf War, atomic plants, nuclear-waste storage sites, and places of employment often leads to public health investigations. Recently, Georgia health professionals have asked public health to investigate clusters of Downs syndrome, heart abnormalities and metabolic disorders. Some congenital anomalies can be prevented. Examples of these include neural tube defects, congenital syphilis, fetal alcohol syndrome, and diabetic embryopathy. Health care

Epidemiology Section, Epidemiology & Prevention Branch, Two Peachtree St., N.W., Atlanta, GA 30303-3186

Phone: (404) 657-2588

FAX: (404) 657-2586

providers and communities can assist in primary prevention of congenital anomalies and reduce the health impact and public concern by supporting an integrated approach that includes monitoring, evaluation of services, and prevention. This report presents strategies to improve information systems and to prevent congenital anomalies in Georgia.
How we know what we know
Data on congenital anomalies in Georgia is derived from statewide and local sources. Vital records include birth, death and fetal death certificates and are a state-wide, populationbased system at the Division of Public Health.
The Metropolitan Atlanta Congenital Defects Program (MACDP) monitors congenital anomalies in five adjacent counties of metropolitan Atlanta since 1968. Cases include all still and live-born infants with a major birth defect diagnosed within the first year of life, and are actively ascertained by abstractors from multiple sources, including hospitals and labs. MACDP is managed at the Centers for Disease Control and Prevention (CDC) in Atlanta, and benefits from close collaboration with hospitals and local communities.
Improving the reporting system
The ideal monitoring system would be inexpensive, cover all pregnancies, be accurate, be timely, and be useful for improving the health care of affected infants. No system fully meets these criteria. MACDP has active case-ascertainment yielding data that is more complete, accurate, and timely than systems that rely only on reports from hospitals, but it requires substantial resources and is thus confined to five Atlanta counties, the only systematic monitoring system in Georgia.
How can we improve the coverage? One option is to improve the accuracy of data on congenital anomalies reported on vital records. The prevalence of congenital anomalies in the Metro Atlanta area is about 1% according to birth certificates compared with 3% reported by MACDP (Table 1). The birth certificate detected only 28% of MACDP cases, that were recognizable at birth; moreover, 23% of infants identified by the birth certificate as cases turned out not to have congenital anomalies recorded in their medical records.2,3
Monitoring anomalies must include both birth and fetal death certificates. In Atlanta fetal death certificates detected 2/3 of all cases of congenital anomalies registered by MACDP among stillbirths from 1989 through 1991 (M. Watkins, personal communication). However, MACDP medical record review could only confirm 1/4 of congenital anomalies reported on the fetal death certificate.
Impact of prenatal diagnosis
Since current monitoring systems rely on ascertainment from hospitals or vital records, they would miss most prenatally terminated cases. The rates in Table 1 will decrease as termination of affected pregnancies increases. If monitoring systems do not include prenatal terminations, clusters may be missed, the impact of congenital anomalies will be underestimated, and the efficacy of prevention will not be measurable.
Prevalence of congenital anomalies
Data from the Metropolitan Atlanta Congenital Defects Program indicate that 3% of all live births in Metropolitan Atlanta have a major congenital anomaly (Table 1, right column). If this figure is applied statewide, an estimated 3,300 newborns in Georgia are born each year with a major congenital anomaly.

Congenital heart defects are the most common, and account for 1/4 of all defects in MACDP (Table 1). Using rates from MACDP, we estimate that, statewide, anencephaly and spina bifida affect 60-70 infants each year .

Table 1. Number and rate of births with congenital anomalies among singleton live births, from birth certificate and MACDP1 data, Georgia, 1989-1994.

Defect Group

Birth Certificate

MACDP1

Georgia Metro-Atlanta Metro-Atlanta

No. Rate No. Rate No. Rate2

All defects

9,945

Selected defects3

Neural Tube Defects

Anencephaly

75

Spina Bifida

161

Hydrocephaly

151

Microcephaly

24

Cleft lip/palate

457

Heart anomalies

432

Esophageal atresia/TEF4 62

Anal atresia

34

Omphalocele

/gastroschisis

142

Diaphragmatic hernia

64

Renal agenesis

48

Clubfoot

330

Trisomy 21

342

15.31 2,401 10.62

0.12 24 0.11

0.25 38 0.17

0.23 43 0.19

0.04

7 0.03

0.70 126 0.56

0.66 116 0.51

0.10

9 0.04

0.05 10 0.04

0.22 51 0.23 0.10 26 0.11 0.07 16 0.07 0.51 103 0.46 0.53 102 0.45

6,839 30.24
39 0.17 91 0.40 105 0.46 102 0.45 293 1.30 1,755 7.76 39 0.17 75 0.34
106 0.47 53 0.23 75 0.33
364 1.61 241 1.07

1 Metropolitan Atlanta Congenital Defects Program 2 Rate per 1,000 singleton live births 3 Except for heart anomalies, the selected anomalies are usually diagnosable at birth 4 Tracheoesophageal fistula

Impact on infant mortality in Georgia
From 1990 through 1994, 1,078 Georgia infants died before their first birthday from congenital anomalies, accounting for 18% of all infant deaths (Figure 1). Congenital anomalies were the first cause of death among white infants, and the second leading cause for African-American infants.
The high proportion of infant deaths from congenital anomalies reflects not only the high prevalence at birth of congenital anomalies (3%) but also the high risk of death associated with some anomalies. The mortality of infants with congenital anomalies was more than 50 times higher than the mortality experienced by infants with no congenital anomalies or low birth weight (16.4% vs. 0.3%), and was 10 times higher than among infants with low birth weight alone.
Preventable anomalies: time for action.
Neural tube defects, fetal alcohol syndrome, congenital syphilis, and diabetic and rubella embryopathies are examples of congenital anomalies that can be prevented by concerted efforts of health care providers, educators, communities, and families (Table 2).
Based on studies from MACDP and from the Division of Public Health, we estimate that from 1992 through 1994 about 200 infants in Georgia were born with anencephaly or spina bifida, half of whom would have not been affected had their mothers taken appropriate folic acid supplements from before conception; about 70 infants were born with fetal alcohol syndrome, and 275 infants with congenital syphilis.
A practical challenge for prevention is that most harmful and/or protective factors may have already influenced the fetus by the time the pregnancy is recognized. A logical approach to prevention would then include planning the pregnancy, so that preventive measures can be implemented from

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before conception (Table 2). An estimated 50% of pregnancies in the U.S. are intended; increasing this proportion would decrease other adverse outcomes related to unintended pregnancies. Moreover, using barrier methods of contraception would also decrease the risk of syphilis infection.
Table 2. Preventive strategies for some congenital anomalies.
Pre-conceptional care q folic acid supplements to prevent neural tube defects q control maternal diabetes to prevent embryopathy q reduce alcohol use to prevent Fetal Alcohol Syndrome q promote planned pregnancy q use barrier methods of contraception to prevent syphilis
Early prenatal care q diagnose and treat syphilis to prevent congenital syphilis q reduce alcohol use to prevent Fetal Alcohol Syndrome
Early Immunization q Vaccinate prepuberal girls against rubella to prevent ru-
bella embryopathy
Food Fortification q Implement fortification of enriched flour with folic acid, to
increase folic acid use by all women of reproductive age
Education on all of the above.
Preconceptional education is a cornerstone of all these preventive efforts. Current efforts in Georgia to prevent neural tube defects through the use of folic acid center on education and supplement usage, and include training, development of educational materials, identification of fortified cereals for use in the WIC (Women and Infant Children) program. Nutritional counseling training of public health nurses and nutritionists, workshops on nutrition competency-based skills, and training in pre-conceptional health are conducted by the Office of Nutrition and sponsored by Womens Health. The March of Dimes is assisting public health to develop nutrition education materials on the use of folic acid.
Summary
1. Congenital anomalies affect 3% of newborns and are the leading cause of infant mortality in Georgia.
2. A public health approach to congenital anomalies includes monitoring the prevalence, improving the accuracy of registration, evaluating services to those affected, and promoting and evaluating prevention programs.
3. Currently Georgia lacks a statewide network to monitor congenital anomalies.
4. Medical records staff could improve the usefulness of vital records for selected anomalies by ensuring that medical records are used for reporting medical data on birth and death certificates.
5. The most effective prevention starts before conception and continues through early prenatal care. Regular daily consumption of 0.4 milligrams of folic acid beginning before conception, rubella vaccination, early prenatal screening for syphilis, and reduced alcohol consumption are examples of feasible measures of prevention.

A Case Study: Neural Tube Defects
Neural tube defects (NTDs), which include anencephaly and spina bifida, affect about 1 in 2,000 births in Georgia. NTDs exemplify both ends of the spectrum of health impact: anencephaly is uniformly lethal, while spina bifida causes long term paralysis, significant disability, and high lifetime costs. Neural tube defects occur with highest prevalence among hispanics, lowest among African-American, and intermediate among whites. According to birth certificate data from 1989 through 1993, NTDs were most common in northeastern and southwestern Georgia, compared to the rest of the state.
We do not know the true impact of neural tube defects statewide. Commonly used systems like birth certificates underestimate the impact (Table 1, second vs. third column), while even the best reporting systems would miss some cases prenatally diagnosed and terminated (in Atlanta, 1/3 of the total4).
At least 50% of neural tube defects could be prevented through daily consumption of 0.4 mg of folic acid by women of childbearing age5. All effective NTD prevention strategies have two points in common: a) they must begin before conception and b) they are most effective when implemented collectively by families, communities, health care providers, and public health workers (Table 2). Prevention involves the promotion of pre-conceptional care, and, more specifically, nutritional education and promotion of supplemental use of folic acid to all women of childbearing age. Recently, the Food and Drug Administration announced that it would add folic acid to the list of vitamins added to enriched flour, thus adding a further approach to the prevention of neural tube defects.
Finally, prevention strategies must be evaluated, which again underscores the importance of having in place an accurate system of monitoring the occurrence of neural tube defects, even among those pregnancies terminated after prenatal diagnosis.
References
1. Lynberg M., Edmonds, L. State use of birth defects surveillance. In:From data to action. L. Wilcox, J. Marks (Eds), U.S. Department of Health and Human Services, Public Health Service, Centers for Disease Control and Prevention, 1994.
2. Watkins M.L. et al. The surveillance of birth defects: the usefulness of the revised US standard birth certificate. Am J Publ Health, 1996, in press.
3. Mathis M.P. et al. Birth certificates as a source of fetal alcohol syndrome case ascertainment, Georgia, 1989-1992. In: Mortality and Morbidity Report vol.44, n.13:251-3, Apr 7, 1995.
4. Roberts H.E. et al. Impact of prenatal diagnosis on the birth prevalence of neural tube defects, Atlanta, 1990-1991. Pediatrics vol.96 n.5:880-3, 1995.
5. Centers for Disease Control and Prevention. Recommendations for the use of folic acid to reduce the number of cases of spina bifida and other neural tube defects. Mortality and Morbidity Report vol. 41, n.RR-14, Sept 11, 1992.
This report was contributed by R. Hudgins, L. Botto, R. Rochat; Office of Perinatal Epidemiology, DPH, GA DHR.

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The Georgia Epidemiology Report Epidemiology and Prevention Branch Two Peachtree St., NW Atlanta, GA 30303-3186

March 1996

Volume 12 Number 3

Reported Cases of Selected Notifiable Diseases in Georgia

Profile for December 1995

Selected Notifiable Diseases Campylobacteriosis Giardiasis H. influenzae B Meningococcal Disease Rubella Salmonellosis Shigellosis Viral Meningitis Tuberculosis Congenital Syphilis Early Syphilis Other Syphilis Cryptosporidiosis E. coli O157:H7 Legionnaires' Disease Lyme Disease Mumps Pertussis

Total Reported for December
1995 41 32 6 16 0 109 5 16 68 7 183 83 4 1 0 0 1 3

Previous 3 Months Total

Ending in December

1995 1994 1993

194

267

133

146

111

131

10

8

18

41

15

22

0

0

0

448

402

296

161

604

105

37

12

32

176

211

260

16

13

18

601

626

767

254

202

189

26

9

2

7

12

7

0

13

5

0

15

7

3

4

4

5

8

8

Previous 12 Months Total

Ending in December

1995 1994 1993

1031

1080

700

572

463

388

71

67

71

124

82

94

0

7

0

1639

1584

1303

1339

1887

471

100

80

172

747

740

809

55

53

112

2507

2654

3864

1057

835

976

110

20

14

28

26

15

14

118

35

14

127

44

11

18

20

30

37

56

The cumulative numbers in the above table reflect the date the disease was first diagnosed rather than the date the report was received at the

state office; and therefore are subject to change over time due to late reporting. The 3 month delay in the disease profile for a given month is

designed to minimize any changes that may occur. This method of summarizing data is expected to provide a better overall measure of disease

trends and patterns in Georgia.

* Data not available for this time period

AIDS Profile Update

Report Period

Total Cases Reported *

Percent Female

MSM

Risk Group Distribution (%)

Race Distribution (%)

IDU MSM&IDU HS Blood Unknown White Black Other

Last 12 Mos 03/95 to 02/96 5 Yrs Ago 03/90 to 02/91 Cumulative 01/80 to 02/96

2342 1292 14946

18.6 9.8 13.6

45.7 18.7

4.2

62.9 15.6

7.2

53.3 18.9

6.0

13.6

1.3

16.5

35.4 61.3 3.4

7.6

2.5

4.1

48.7 50.2 1.2

9.8

2.1

9.7

42.1 50.2 1.2

MSM - Men having sex with men

IDU - Injection drug users

* Case totals are accumulated by date of report to the Epidemiology Section

HS - Heterosexual

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