Survival of infants and children born with severe microcephaly, Texas, 1999-2015.

BACKGROUND: Severe microcephaly is a brain reduction defect where the delivery head circumference is <3rd percentile for gestational age and sex with subsequent lifelong morbidities. Our objective was to evaluate survival among 2,704 Texas infants with severe microcephaly delivered 1999-2015. METHODS: Infants with severe microcephaly from the Texas Birth Defects Registry were linked to death certificates and the national death index. Survival estimates, hazard ratios (HR) and confidence intervals (CI) were calculated using the Kaplan-Meier method and Cox proportional hazards models stratified by presence versus absence of co-occurring defects. RESULTS: We identified 496 deaths by age 4 years; most (42.9%) occurred in the neonatal period, and another 39.9% died by 1 year of age. Overall infant survival was 84.8%. Lowest infant survival subgroups included those with chromosomal/syndromic conditions (66.1%), very preterm deliveries (63.9%), or co-occurring critical congenital heart defects (44.0%). Among infants with severe microcephaly and a chromosomal/syndromic co-occurring defect, the risk of death was nearly three-fold higher among those with: proportionate microcephaly (i.e., small baby overall), relative to non-proportionate (HR = 2.84, 95% CI = 2.17-3.71); low-birthweight relative to normal (HR = 2.72, 95% CI = 1.92-3.85); critical congenital heart defects (CCHD) relative to no CCHD (HR = 2.90, 95% CI = 2.20-3.80). Trisomies were a leading underlying cause of death (27.5%). CONCLUSIONS: Overall, infants with severe microcephaly had high 4-year survival rates which varied by the presence of co-occurring defects. Infants with co-occurring chromosomal/syndromic anomalies have a higher risk of death by age one than those without any co-occurring birth defects.

Factors associated with Medicaid participation among infants born with birth defects in Texas, 2010-2014.

BACKGROUND: Birth defects are major contributors to healthcare resource use, disability, and mortality, particularly during the perinatal period. As the nation's public insurance program for low-income individuals, Medicaid funds a large proportion of healthcare costs associated with birth defects. Here, we explore birth defect-related factors associated with Medicaid participation in the first year of life. METHODS: Infants born with birth defects between 2010 and 2014 were linked from the Texas Birth Defects Registry to the state's Medicaid claims database. Variation in Medicaid participation was examined by individual birth defect and by birth defect characteristics. The associations between covariates and Medicaid participation are described using percentages and adjusted prevalence ratios (APR). RESULTS: Of the 107,968 infants included in this study, 55,172 (51.1%) participated in Medicaid. Medicaid participation ranged from 12.1% for anencephaly to 77.8% for total anomalous pulmonary venous connection. An indicator of defect severity was associated with an increased likelihood of participation (APR = 1.22, 95% CI: 1.20-1.23). Medicaid participation was 60.8% for individuals with multiple major defects, compared with 45.4% for those without (APR = 1.24, 95% CI: 1.22-1.25). Among individual birth defects, Medicaid participation was almost universally higher for those co-occurring with other major defects. CONCLUSIONS: We detected large variations in Medicaid participation by individual birth defect. Infants participating in Medicaid tended to have more severe defects and were more likely to have multiple major defects. Medicaid claims databases can serve as valuable sources of data for surveillance efforts and observational studies, but care should be taken when generalizing findings.

Acculturation and selected birth defects among non-Hispanic Blacks in a population-based case-control study.

BACKGROUND: There are noted birth defects prevalence differences between race/ethnicity groups. For instance, non-Hispanic (NH) Black mothers are more likely to have an infant with encephalocele, although less likely to have an infant with anotia/microtia compared to NH Whites. When stratifying by nativity and years lived within the United States, additional variations become apparent. METHODS: Data from the National Birth Defects Prevention Study were used to calculate descriptive statistics and estimate crude/adjusted odds ratios (aORs) and 95% confidence intervals (95%CIs) among NH Blacks with one of 30 major defects and non-malformed controls. Total case/controls were as follows: U.S.- (2,773/1101); Foreign- (343/151); African-born (161/64). Study participants were also examined by number of years lived in the U.S. (≤5 vs. 6+ years). RESULTS: Compared to U.S.-born, foreign-born NH Black controls tended to be older, had more years of education, and were more likely to have a higher household income. They also had fewer previous livebirths and were less likely to be obese. In the adjusted analyses, two defect groups were significantly attenuated: limb deficiencies, aORs/95%CIs = (0.44 [0.20-0.97]) and septal defects (0.69 [0.48-0.99]). After stratifying by years lived in the United States, the risk for hydrocephaly (2.43 [1.03-5.74]) became apparent among those having lived 6+ years in the United States. When restricting to African-born mothers, none of the findings were statistically significant. CONCLUSIONS: Foreign-born NH Blacks were at a reduced risk for a few selected defects. Results were consistent after restricting to African-born mothers and did not change considerably when stratifying by years lived in the United States.

The prevalence of birth defects among non-Hispanic Asian/Pacific Islanders and American Indians/Alaska Natives in Texas, 1999-2015.

BACKGROUND: There is considerable variability in the prevalence of birth defects among racial/ethnic groups. This study estimated birth defect prevalence among the less studied non-Hispanic (NH) Asian/Pacific Islander (PI) and American Indian/Alaska Native (AI/AN) populations in Texas relative to NH Whites. METHODS: Data were obtained from the Texas Birth Defect Registry from 1999 to 2015 for deliveries to Texas-resident women who were NH White, NH Asian/PI, or AI/AN. This covers a live birth population of 2.6 million. Prevalence ratios and 95% confidence intervals (95% CIs) were calculated for NH Asian/PIs and AI/ANs (relative to NH Whites) for 44 birth defects using Poisson regression adjusting for maternal age. RESULTS: After adjustment there were 33 statistically significant prevalence ratios (aPRs). Among NH Asian/PIs, 23 defects had a lower aPR (0.38-0.86) and three defects had a higher aPR (1.19-2.50). AI/ANs had one defect with a significantly lower aPR (0.64) and six with a higher aPR (1.36-4.63). CONCLUSIONS: Non-Hispanic Asian/PIs generally have a lower prevalence ratio for many birth defects while AI/ANs have a higher prevalence ratio compared to NH Whites. These findings update the limited literature on this topic and warrant additional research to identify the true associations across a range of birth defects among these understudied racial/ethnic groups.

Selected acculturation factors and birth defects in the National Birth Defects Prevention Study, 1997-2011.

BACKGROUND: Acculturation has been examined with respect to various pregnancy adverse outcomes, including birth defects. Given the mixed and limited findings on the association between nativity and birth defects, we sought to further explore parental nativity and years lived in the U.S. across a range of defects. METHODS: Data from the National Birth Defects Prevention Study were used for this analysis. Infants with one of 46 major isolated birth defects (30 noncardiac/16 cardiac conditions) and infants without birth defects (controls) born during 1997-2011 were included. We examined parental nativity (foreign-born mothers, fathers, and both parents combined compared to a referent of both U.S.-born parents) and the number of years lived in the U.S. (≤5/6+ years). Descriptive statistics and logistic regression analyses were performed to estimate crude/adjusted odds ratios and 95% confidence intervals. RESULTS: Compared to U.S.-born mothers, foreign-born mothers tended to be older (25+ years), of Hispanic or Other race/ethnicity and were less likely to have reported drinking, smoking, illicit drug use, or having taken folic acid. In the adjusted analysis, seven findings among both parents reporting a foreign-birth were significant, including an increased association with spina bifida, anotia/microtia, and diaphragmatic hernia (aORs range: 1.3-1.7), and a reduced association with craniosynostosis and gastroschisis (aORs = 0.7). A generally protective effect was observed among foreign-born subjects living in the U.S. ≤5 years. CONCLUSIONS: We found that nativity was associated with some selected isolated defects, although the direction of effect varied by phenotype and by a number of years residing in the U.S.