Modeling geographic risk of complex congenital heart defects in Eastern Wisconsin.

BACKGROUND: Geographic variation may be an indicator of risk factors for birth defects. This study models the geographic distribution of three complex congenital heart defects (CHDs) in eastern Wisconsin, and evaluates effects of demographic census variables linked to geographic location. METHODS: Cases of Hypoplastic Left Heart Syndrome (HLHS), Tetralogy of Fallot (TOF) and d-Transposition of the Great Arteries (d-TGAs) born between1995 and 2004 were identified from three medical centers serving eastern Wisconsin. Case diagnoses were assigned by a pediatric cardiologist using echocardiographic records. Births by ZIP code were obtained from the State of Wisconsin. ZIP Code demographic variables were derived from 2000 census data. Numbers of cardiac defects by ZIP code were modeled using cluster analysis and Poisson generalized additive models (GAMs) for spatial coordinates including all and white only cases (excluding trisomies). GAM analyses were repeated adjusting for census variables. RESULTS: Four hundred forty-eight cases were ascertained. A significant south-to-north spatial gradient for HLHS, TOF, and combined CHDs, but not d-TGAs was identified. This gradient remained significant when census variables were included in the model for the full sample. In the analysis excluding non-white cases, findings were the same for TOF, combined CHDs, and d-TGAs. However, the geographic gradient for HLHS was not significant in the adjusted model. CONCLUSIONS: A south-to-north gradient was apparent for two of three complex CHDs in eastern Wisconsin. For white cases, demographic variation seems to explain some of this spatial gradient in HLHS. Further studies are needed to confirm demographic and other risk factors underlying this geographic gradient.

Effects of a culturally tailored intervention on changes in body mass index and health-related quality of life of Latino children and their parents.

PURPOSE: To evaluate the effects of a multicomponent, family-based, culturally tailored intervention for overweight Latino children and their parents. DESIGN: One group pretest/posttest with clinic comparison group. SETTING: Community health center in Milwaukee, Wisconsin. SUBJECTS: 54 Spanish-speaking, Latino families with children 8 to 11 years of age at enrollment and a body mass index (BMI) > 85th percentile. Thirty-one completed the 12-month follow-up. MAIN OUTCOME: Measures. BMI, fitness measures, self-reported eating patterns, sedentary behaviors, and quality of life scales. INTERVENTION: Index child and one (index) parent participated in interactive sessions and physical activity reinforced by family goal-setting, staff support, and supplemental activities. RESULTS: Small but statistically significant changes in child BMI z score (Δ = .13 SD, p < .001) and parent fitness (Δ = .74, p < .04) were documented by paired t-test. Quality of life increased significantly for children (combined Pediatric Quality of Life Inventory score Δ = 10.7, p < .001) and parents (Short Form Health Survey12 mental composite score Δ = 8, p < .022; Wilcoxon rank sum test). BMI z score for clinic comparison children (n = 31) increased significantly during the same time period (Δ = .23 SD, paired t = 4.32, p < .0002). CONCLUSIONS: A culturally tailored program for Latino families reduced BMI for enrolled children and significantly enhanced quality of life for children and parents. Intervention approaches that integrate cultural and social circumstances and emphasize goal setting and life style changes may be fruitful for this population of at-risk children.

The National Children's Study and the children of Wisconsin.

Prospective, multi-year epidemiologic studies such as the Framingham Heart Study and the Nurses' Health Study have proven highly effective in identifying risk factors for chronic illness and in guiding disease prevention. Now, in order to identify environmental risk factors for chronic disease in children, the US Congress authorized a National Children's Study as part of the Children's Health Act of 2000. Enrollment of a nationally representative cohort of 100,000 children will begin in 2008, with follow-up to continue through age 21. Environmental assessment and examination of biomarkers collected at specified intervals during pregnancy and childhood will be a major component of the Study. Recruitment at 105 sites across the United States is planned, and will begin at 7 Vanguard Centers in 2008, including Waukesha County, Wis. The National Children's Study will provide information on preventable risk factors for such chronic diseases as asthma, certain birth defects, neurobehavioral syndromes, and obesity. In addition, the National Children's Study will provide training in pediatric environmental health for the next generation of researchers and practitioners.

The National Children's Study Waukesha County, Wisconsin Vanguard Center.

The National Children's Study (NCS) is a large, longterm study designed to detect environmental influences on the health and development of children. Waukesha County, Wisconsin, was selected as 1 of 7 "Vanguard Centers" currently funded to finalize and lead the implementation of the study protocol. The authors provide an overview of key design and planning processes that will be used at all NCS Vanguard locations, the specific approaches to be used in the NCS Waukesha County Vanguard Center, and information about how Wisconsin physicians and other health care professionals can become involved in working with the NCS.

National Children's Study: environmental exposures in Waukesha County.

The National Children's Study (NCS), launched in September of 2005, will investigate the effects of environmental exposures and children's health and development. Waukesha County, Wis was selected as 1 of 7 sites to spearhead this ambitious undertaking. Residents of Waukesha County may experience different kinds of environmental exposures from water, land, and air based on where they live, work, and play. A selected number of Waukesha County's environmental exposures described briefly in this report will serve the NCS well with their heterogeneity of potential exposures: from private well water and community water supplies that obtain water from both surface and groundwater; from the variable exposures to ambient air pollution from mobile sources, local industrial sources, and distant sources (ozone); and the different levels of exposures from soil and dust depending on the prevalence of pesticide use and lead-based paints. By combining data gathered from Waukesha County's participants with other study sites, a holistic picture of environmental exposures in the United States can be evaluated as it influences the health of our nation's children.

Alcohol dehydrogenase 2*3 affects alterations in offspring facial morphology associated with maternal ethanol intake in pregnancy.

BACKGROUND: Ethanol intake during pregnancy alters offspring facial morphology. However, significant variation that may be due to genetic diversity in ethanol metabolizing enzymes occurs. The alcohol dehydrogenase 1B*3 (ADH1B*3) allele is protective for offspring developmental outcome after maternal alcohol drinking in pregnancy and may explain the spectrum of facial morphology. METHODS: Faces of infants with known ADH1B genotype, whose mothers' ADH1B genotypes and ethanol intake were determined during pregnancy, were photographed using uniform procedures. Photographs were scanned and the inner canthal distance, palpebral fissure length, and distance from the bridge of the nose to the bottom of the upper lip were measured by an investigator who was blinded to genotype and ethanol exposure. RESULTS: Among 247 photographed infants, each facial measurement varied >2-fold. Median absolute ethanol daily intake was 0.5 oz among mothers who reported drinking in the periconceptional period (N = 173) and 0.17 oz among mothers who reported drinking before the first prenatal visit (N = 62). Controlling for the amount of maternal ethanol intake just before the first prenatal visit and infant sex, the three-way interaction among the absence of a maternal and offspring ADH1B*3 allele and the presence of ethanol consumption just before the first prenatal visit was associated with smaller facial measurements (p = 0.002, MANCOVA). CONCLUSIONS: These are the first observations of a significant gene-environment interaction explaining variation in facial morphology associated with ethanol use in pregnancy. This positive effect of ADH1B*3 is consistent with its known positive effect on offspring birth weight and developmental outcome after in utero ethanol exposure.

The Wisconsin Fetal Alcohol Syndrome Screening Project.

PROBLEM: Fetal Alcohol Syndrome (FAS) is preventable, under-diagnosed, and under-reported. Wisconsin rates for alcohol use and binge drinking in childbearing-age women exceed the national average. FAS prevalence in Wisconsin has not previously been systematically evaluated. METHODS: The Wisconsin Fetal Alcohol Syndrome Screening Project (WFASSP) used a multi-stage, multisource prospective population-based screening methodology to identify children born in 1998-1999 in Southeast Wisconsin who met a surveillance case definition for FAS. The 4-stage methodology used screening of electronic birth files, abstraction of neonatal medical records, and direct assessment of facial features, growth, and development at age 2 to 3 years. RESULTS: The FAS prevalence rate was 0.23 per 1000 births. Children directly evaluated had fewer demographic, pregnancy, and maternal substance use risk factors than lost-to-follow-up children. Thirty-two percent of children with weight and head circumference below the 10th percentile at birth were developmentally delayed and 47% had at least one physical growth delay. CONCLUSIONS: The WFASSP methodology identified children who had not previously been diagnosed with FAS. Using the combination of weight and head circumference below the 10th percentile at birth is a useful methodology for identifying children at substantial risk for growth and developmental delays from FAS or other unspecified etiologies.

Excess birth prevalence of Hypoplastic Left Heart syndrome in eastern Wisconsin for birth cohorts 1997-1999.

BACKGROUND: Hypoplastic Left Heart syndrome (HLHS) is a group of cardiac malformations involving underdevelopment of the left heart with an inability to maintain systemic circulation. Because of a clinical impression of excess HLHS prevalence, we completed a medical record review of cases born from 1997 through 1999 who were Wisconsin residents and seen at the Children's Hospital of Wisconsin (CHW). METHODS: Cases were identified either in the CHW medical records database or the Division of Pediatric Cardiology database and confirmed by echocardiogram, catheterization, surgery, or autopsy. U.S. and international surveillance systems were used to estimate population risk. Rates per 10,000 births were computed for eastern Wisconsin and four regions within this portion of the state, and compared to the estimated population risk. The same methods were used to evaluate whether rates for tetralogy of Fallot and transposition of the great arteries were elevated. RESULTS: A total of 61 cases were ascertained yielding a birth prevalence of 3.7 per 10,000 births, which was greater than the estimated population risk of 2.79. The rate for the southeast region also exceeded the expected rate. The most urban and industrialized areas had the highest rates. Rates for the other two diagnoses evaluated were not different from estimated population risks. CONCLUSIONS: Eastern Wisconsin, particularly the urban southeast region, had elevated rates of HLHS. Because of the geographic clustering of high rates, environmental factors may be associated with this finding.

Completeness of state administrative databases for surveillance of congenital heart disease.

BACKGROUND: Tracking birth prevalence of cardiac defects is essential to determining time and space clusters, and identifying potential associated factors. Resource limitations on state birth defects surveillance programs sometimes require that databases already available be used for ascertaining such defects. This study evaluated the data quality of state administrative databases for ascertaining congenital heart defects (CHD) and specific diagnoses of CHD. METHODS: Children's Hospital of Wisconsin (CHW) medical records for infants born 1997-1999 and treated for CHD (n = 373) were abstracted and each case assigned CHD diagnoses based on definitive diagnostic reports (echocardiograms, catheterizations, surgical or autopsy reports). These data were linked to state birth and death records, and birth and postnatal (< 1 year of age) hospital discharge summaries at the Wisconsin Bureau of Health Information (WBHI). Presence of any code/checkbox indicating CHD (generic CHD) and exact matches to abstracted diagnoses were evaluated. RESULTS: Fifty-eight percent of cases with generic CHD were identified by state databases. Postnatal hospital discharge summaries identified 48%, birth hospital discharge summaries 27%, birth certificates 9% and death records 4% of these cases. Exact matches were found for 52% of 633 specific diagnoses. Postnatal hospital discharge summaries provided most matches. CONCLUSION: State databases identified 60% of generic CHD and exactly matched about half of specific CHD diagnoses. The postnatal hospital discharge summaries performed best in both in identifying generic CHD and matching specific CHD diagnoses. Vital records had limited value in ascertaining CHD.

Growth of postmenarcheal girls from three ethnic groups.

Documentation of normal growth in late adolescence has been limited to a few studies using largely white participants. Annual growth rates of 668 high school girls who had already achieved menarche were determined for stature, sitting height, and knee height measured using the Knee Height Measuring Device (KHMD), an instrument with superior reliability. The sample was 61.4% white, 16.8% Puerto Rican, 15.7% African American, and 6.1% girls of other ethnic backgrounds. Median growth rate was 1.5 cm/year for stature, 1.1 cm/year for sitting height, and 2.7 mm/year for knee height in the first full year after menarche, and >80% of all girls grew in at least one dimension. Growth in stature (≥1 cm) continued for 64% of girls 1 full year after menarche and for 31% of girls 2 years after menarche, and growth in knee height (≥1 mm) continued in >45% of girls up to 5 completed years past menarche. Whites, African Americans, and Puerto Ricans showed small but significant differences in amounts of postmenarcheal growth in the dimensions measured. African-American girls grew less in stature and sitting height than other groups, whereas Puerto Rican girls grew significantly more in sitting height and significantly less in knee height than other ethnic groups. These findings demonstrate that, as documented in other studies of postmenarcheal growth, there is substantial growth after menarche in most girls. The most sensitive measures indicate that small amounts of growth persist >5 years after menarche. Appreciation of this phenomenon needs to be communicated to clinicians who generally assume that growth ceases at/or just after menarche. © 1996 Wiley-Liss, Inc.

Longitudinal principal components analysis of patterns and predictors of growth in Guatemalan children.

Longitudinal principal components (LPC) analysis was used to assess growth patterns in children from rural Guatemala in order to determine if this methodology could provide additional information regarding correlates of growth compared to more traditionally used methods based on attained size and increments. LPC analysis reduces measures at many points in time into a few parameters. However, LPC analysis requires complete data, and many cases may be lost due to missing values. Thus the potentially greater sensitivity of LPC analysis should be weighed against the reduced power resulting from smaller sample sizes. Component indices representing centile level and centile shift, attained size, and 3 to 36 month increments of growth in length and weight were used as the dependent variables in multiple regression models in order to examine the effects of environmental variables, such as home dietary intake, supplementation, and prevalence of diarrhea on growth. Regardless of which growth index, i.e., attained size, incremental change, or principal component, was used, regression results were similar; higher nutritional intakes were generally associated with greater and more rapid growth from birth to age 3 years. The possible advantages of LPC analysis over more traditional methods were not great; therefore, LPC analysis is not recommended as the method of choice in this population.

Measurement of short-term growth with a new knee height measuring device.

Information on short-term growth (growth over periods of 1 month or less) has potential use in the clinic for treatment of children with growth disorders, and knowledge about patterns of short-term growth may also contribute to an understanding of growth control mechanisms. Limitations of measurement reliability for most measurements of linear growth have generally confined us to evaluation of growth over periods 3 month or longer. This report introduces the Knee Height Measuring Device (KHMD) The Knee Height Measuring Device is available from Intersciences Development Associates, 3508 Market St., Philadelphia, PA 19104 (215-662-0700), patent pending. , which can measure changes in size of the lower leg of approximately 0.5 mm and thus assist in evaluation of short-term growth. It is smaller, more portable, and less expensive than the Valk lower leg measuring device (knemometer) and uses a less subjectively biased measurement technique. Preliminary comparisons using a sample of 15 children measured twice at a 28-day interval of the validity and reliability of a prototype KHMD and the Valk knemometer. Yielded an intraobserver error of 0.295 for the KHMD and 0.206 for the knemometer. Agreement of the two instruments on the presence of detectable growth was 90%. Pearson correlation of growth rates detected by the two instruments was 0.73. After additional alterations improving the design and measurement methodology of the KHMD, 103 children 6-10 years old were measured at 28-day intervals from January to June and then at 3-month intervals from June to December of 1988 using the improved KHMD alone. Intraobserver error for these measurements was 0.22 mm. This error was modestly associated with the weight of the child (r=.31) but unrelated to sex, height, or growth in any dimension. Interobserver error for 22 children measured by two observers was 0.33 mm. We analyzed the knee height growth data primarily to test whether monthly growth could be reliably detected by this instrument. Average 28-day knee height growth was 1.76±0.3 mm, which is three times greater than the 0.5 mm growth detectable with the KHMD. Over any given 1-month interval, fewer than 10% of the children had growth less than that which could be detected with the instrument. We conclude that the KHMD can reliably detect growth over a 1-month interval and has potential clinical use for monitoring growth and for evaluating the characteristics and associations of short-term growth.

Relationship of disease characteristics and nutritional status to patterns of growth in children with Crohn disease.

Crohn disease is one type of inflammatory bowel disease with frequent occurrence before maturity. Many children with Crohn disease experience linear growth retardation. However, the prevalence and contribution of various factors to growth failure have not been adequately investigated. The purpose of the present study was to estimate the prevalence of growth deficiency by using multiple criteria based on longitudinal data and to simulatneously evaluate the contribution of disease, demographic, and treatment characteristics to the growth failure in this group of children. A sample of 78 patients assessed retrospectively and 41 patients followed prospectively were drawn from the Children's Hospital of Philadelphia Division of Gastroenterology and Nutrition. Data on growth, nutritional status (hematocrit and albumin levels), disease characteristics (severity, site, duration, and age of onset), and steroid dosage were collected for both groups. For the prospective sample, measurements of height, weight, and four skinfold thicknesses were taken. A 24 hour recall and 3 day food record estimated kcal intake. Parental size was taken by report from the accompanying caregiver. When three criteria were used, prevalence of growth retardation was 67% in the retrospective group, higher than most available figures. In the prospective sample, reductions in height adjusted for midparental size were greater than those for weight, and depletion of lower body fat was greater than that of upper body fat. Multiple regression analyses showed that degree of growth retardation as measured by the parent adjusted z score for height was associated with higher steroid dosage and later age of disease onset, but not disease severity or the anatomical site of the disease. Of the nutritional status variables, degree of growth retardation was most closely associated with degree of reduction in lower body fat stores.