This study provides prevalence estimates of DS among children and adolescents from 10 US regions. These estimates varied according to region, race/ethnicity, and gender, suggesting possible variation in prevalence at birth or in survival rates on the basis of these characteristics.

OBJECTIVE To identify trends in the prevalence and epidemiologic correlates of gastroschisis using a large population-based sample with cases identified by the National Birth Defects Prevention Network over the course of an 11-year period. METHODS This study examined 4,713 cases of gastroschisis occurring in 15 states during 1995–2005, using public use natality data sets for denominators. Multivariable Poisson regression was used to identify statistically significant risk factors, and Joinpoint regression analyses were conducted to assess temporal trends in gastroschisis prevalence by maternal age and race and ethnicity. RESULTS Results show an increasing temporal trend for gastroschisis (from 2.32 per 10,000 to 4.42 per 10,000 live births). Increasing prevalence of gastroschisis has occurred primarily among younger mothers (11.45 per 10,000 live births among mothers younger than age 20 years compared with 5.35 per 10,000 among women aged 20 to 24 years). In the multivariable analysis, using non-Hispanic whites as the referent group, non-Hispanic black women had the lowest risk of having a gastroschisis-affected pregnancy (prevalence ratio 0.42, 95% confidence interval [CI] 0.37–0.48), followed by Hispanics (prevalence ratio 0.86, 95% CI 0.81–0.92). Gastroschisis prevalence did not differ by newborn sex. CONCLUSIONS Our findings demonstrate that the prevalence of gastroschisis has been increasing since 1995 among 15 states in the United States, and that higher rates of gastroschisis are associated with non-Hispanic white maternal race and ethnicity, and maternal age younger than 25 years (particularly younger than 20 years of age).

Background Because of advancements in care, there has been a decline in mortality from congenital heart defects (CHD) over the last several decades. However, there are no current empirical data documenting the number of people living with CHD in the United States (US). Our aim was to estimate the CHD prevalence across all age groups in the US in the year 2010. Methods The age-, sex-, and severity-specific observed prevalence of CHD in Québec, Canada in the year 2010 was assumed to equal the CHD prevalence in the non-Hispanic white population in the US in 2010. A race-ethnicity adjustment factor, reflecting differential survival between racial-ethnic groups through age 5 for persons with a CHD and that in the general US population, was applied to the estimated non-Hispanic white rates to derive CHD prevalence estimates among US non-Hispanic blacks and Hispanics. Confidence intervals for the estimated CHD prevalence rates and case counts were derived using a combination of Taylor series approximations and Monte Carlo simulation. Results We estimated that approximately 2.4 million people (1.4 million adults, 1 million children) were living with CHD in the US in 2010. Nearly 300,000 of these individuals had severe CHD. Conclusions Our estimates highlight the need for two important efforts: (1) planning for health services delivery to meet the needs of the growing population of adults with CHD and; (2) the development of surveillance data across the lifespan to provide empirical estimates of the prevalence of CHD across all age groups in the US.

The survival of infants born with spina bifida has improved; however, improvements in survival varied by race/ethnicity, and blacks and Hispanics continued to have poorer survival than whites in the most recent birth cohort from 1998-2002. Further studies are warranted to elucidate possible reasons for the observed differences in survival.

An increase in the rate of gastroschisis was observed in the mid-1970s, but no temporal trend has been observed since that time. In light of recent reports of an increasing prevalence of gastroschisis in the United States, continued monitoring of this birth defect is warranted.

Objectives To examine racial/ethnic-specific survival of children with major birth defects in the US. Study design We pooled data on live births delivered during 1999-2007 with any of 21 birth defects from 12 population-based birth defects surveillance programs. We used the Kaplan-Meier method to calculate cumulative survival probabilities and Cox proportional hazards models to estimate mortality risk. Results For most birth defects, there were small-to-moderate differences in neonatal (<28 days) survival among racial/ethnic groups. However, compared with children born to non-Hispanic white mothers, postneonatal infant (28 days to <1 year) mortality risk was significantly greater among children born to non-Hispanic black mothers for 13 of 21 defects (hazard ratios [HRs] 1.3-2.8) and among children born to Hispanic mothers for 10 of 21 defects (HRs 1.3-1.7). Compared with children born to non-Hispanic white mothers, a significantly increased childhood (≤8 years) mortality risk was found among children born to Asian/Pacific Islander mothers for encephalocele (HR 2.6), tetralogy of Fallot, and atrioventricular septal defect (HRs 1.6-1.8) and among children born to American Indian/Alaska Native mothers for encephalocele (HR 2.8), whereas a significantly decreased childhood mortality risk was found among children born to Asian/Pacific Islander mothers for cleft lip with or without cleft palate (HR 0.6). Conclusion Children with birth defects born to non-Hispanic black and Hispanic mothers carry a greater risk of mortality well into childhood, especially children with congenital heart defect. Understanding survival differences among racial/ethnic groups provides important information for policy development and service planning.

Since its inception, MACDP has served as a resource for the development of uniform methods and approaches to birth defect surveillance across the United States and in many other countries, monitoring birth defects rates, and as a case registry for various descriptive, etiologic, and survival studies of birth defects. MACDP has also served as a training ground for a large number of professionals active in birth defects epidemiology.

Objective To assess the association between genitourinary infections in the month before conception to the end of the first trimesterand gastroschisis. Design Case-control study with self reported infections from a computer assisted telephone interview. Setting National birth defects prevention study, a multisite, population based study including 10 surveillance systems for birth defects in the United States. Participants Mothers of 505 offspring with gastroschisis and 4924 healthy liveborn infants as controls. Main outcome measure Adjusted odds ratios for gastroschisis with 95% confidence intervals. Results About 16% (n=81) of case mothers and 9% (n=425) of control mothers reported a genitourinary infection in the relevant time period; 4% (n=21) and 2% (n=98) reported a sexually transmitted infection and 13% (n=67) and 7% (n=338) reported a urinary tract infection, respectively. Case mothers aged <25 years reported higher rates of urinary tract infection alone and in combination with a sexually transmitted infection compared with control mothers. In women who reported both types of infection, there was a greater risk of gastroschisis in offspring (adjusted odds ratio 4.0, 95% confidence interval 1.4 to 11.6). Conclusion There is a significant association between self reported urinary tract infection plus sexually transmitted infection just before conception and in early pregnancy and gastroschisis.