Purpose: We examined the total prevalence, trends in prevalence, and age-specific mortality among individuals with anorectal malformation (ARM). Methods: We conducted a retrospective cohort study using data from 24 population- and hospital-based birth defects surveillance programs affiliated with the International Clearinghouse for Birth Defects Surveillance and Research (ICBDSR) from 18 countries and for births from 1974 to 2014. We estimated pooled and program-specific total prevalence per 10,000 total births. Poisson regression was used to assess time trends in prevalence from 2001 to 2012 when most programs contributed data. We calculated selected age-specific proportions of deaths, stratified by case status. Results: The pooled total prevalence of ARM was 3.26 per 10,000 total births (95% Confidence Interval = 3.19, 3.32) for birth years 1974–2014. About 60% of cases were multiple or syndromic. Prevalence of multiple, syndromic, and stillborn cases decreased from 2001 to 2012. The first week mortality proportion was 12.5%, 3.2%, 28.3%, and 18.2% among all, isolated, multiple, and syndromic cases, respectively. Conclusions: ARM is relatively rare, with multiple and syndromic cases showing decreasing prevalence during the study period. Mortality is a concern during the first week of life, and especially among multiple and syndromic cases. Our descriptive epidemiological findings increase our understanding of geographic variation in the prevalence of ARM and can be used to plan needed clinical services. Exploring factors influencing prevalence and mortality among individuals with ARM could inform future studies.

Paediatricians have a long history of championing highly effective public health programmes that have improved the lives of children around the world. Now, paediatricians have another unprecedented opportunity to champion primary prevention of spina bifida and anencephaly (SBA) globally. Thirty years ago, the British Medical Research Council’s landmark randomised control trial established that adequate intake of folic acid consumption by mothers before and during early pregnancy is the most effective public health strategy for preventing the majority of SBA cases, known as SBA-F (F indicates folic acid-preventable SBA).1 As of 2020, 58 countries have mandatory policies for staple food fortification with folic acid, due to which about 22% of global cases of preventable SBA-F are prevented.2 While mandatory fortification benefits these 58 countries, over 100 other countries, many in Europe, Asia and Africa lag behind in SBA-F prevention by not investing in this proven public health intervention.

Background: Congenital diaphragmatic hernia (CDH) occurs when abnormal diaphragm development allows herniation of abdominal organs into the thoracic cavity. Its etiopathogenesis is not well understood, but cigarette smoking and alcohol exposure may impact diaphragm development. Using data from a large, population-based case–control study, we examined associations between maternal cigarette smoking and alcohol consumption and CDH in offspring. Methods: We analyzed maternal interview reports of cigarette smoking and alcohol consumption during early pregnancy for 831 children with CDH and 11,416 children without birth defects with estimated dates of delivery during 1997–2011. Generalized linear mixed effects models with a random intercept for study site were used to estimate associations between measures of exposure to smoking (any, type, frequency, duration) and alcohol (any, quantity, frequency, variability, type) for all CDH combined and selected subtypes (Bochdalek and Morgagni). Results: Mothers of 280 (34.0%) case and 3,451 (30.3%) control children reported early pregnancy exposure to cigarette smoking. Adjusted odds ratios for all CDH were increased for any (1.3; 95% confidence interval 1.1–1.5), active (1.3, 1.0–1.7), and passive (1.4, 1.1–1.7) smoking. Early pregnancy alcohol consumption was reported by mothers of 286 (34.9%) case and 4,200 (37.0%) control children; odds were near the null for any consumption (0.9, 0.8–1.1) and consumption with (0.9, 0.7, 1.1) or without (0.9, 0.8, 1.1) binging. Estimates for smoking and alcohol tended to be higher for Bochdalek CDH and Morgagni CDH than those for all CDH. Conclusions: Findings suggest that maternal early pregnancy exposure to cigarette smoking, but less so to alcohol consumption, contributes to CDH. These findings need to be replicated in additional large studies that use systematic case ascertainment and classification, detailed exposure assessment, and examine subtype-specific associations.

Objective: The United Nations' Sustainable Development Goals (SDGs) require population-based data on children with disabilities to inform global policies and intervention programs. We set out to compare the prevalence estimates of disabilities among children and adolescents younger than 20 years as reported by the world's leading organizations for global health statistics. Methods: We purposively searched the disability reports and databases of the United Nations Children's Fund (UNICEF), the World Health Organization (WHO), the World Bank and the Global Burden of Diseases (GBD) Study. We analyzed the latest disability data reported by these organizations since 2015. We examined the methodologies adopted in generating the reported prevalence estimates and evaluated the degree of agreement among the data sources using Welch's test of statistical difference, and the two one-sided t-test (TOST) for statistical equivalence. Results: Only UNICEF and GBD provided the most comprehensive prevalence estimates of disabilities in children and adolescents. Globally, UNICEF estimated that 28.9 million (4.3%) children aged 0–4 years, 207.4 million (12.5%) children aged 5–17 years and 236.4 million (10.1%) children aged 0–17 years have moderate-to-severe disabilities based on household surveys of child functional status. Using the UNICEF estimated prevalence of 10.1%, approximately 266 million children aged 0–19 years are expected to have moderate-to-severe disabilities. In contrast, GBD 2019 estimated that 49.8 million (7.5%) children aged under 5 years, 241.5 million (12.6%) children aged 5–19 years and 291.3 million (11.3%) children younger than 20 years have mild-to-severe disabilities. In both databases, Sub-Saharan Africa and South Asia accounted for more than half of children with disabilities. A comparison of the UNICEF and GBD estimates showed that the overall mean prevalence estimates for children under 5 years were statistically different and not statistically equivalent based on ±3 percentage-point margin. However, the prevalence estimates for children 5–19 years and < 20 years were not statistically different and were statistically equivalent. Conclusion: Prevalence estimates of disabilities among children and adolescents generated using either functional approach or statistical modeling appear to be comparable and complementary. Improved alignment of the age-groups, thresholds of disability and the estimation process across databases, particularly among children under 5 years should be considered. Children and adolescents with disabilities will be well-served by a variety of complementary data sources to optimize their health and well-being as envisioned in the SDGs.

Objective: Children with developmental disabilities are associated with a high risk of poor school enrollment and educational attainment without timely and appropriate support. Epidemiological data on cerebral palsy and associated comorbidities required for policy intervention in global health are lacking. This paper set out to report the best available evidence on the global and regional prevalence of cerebral palsy (CP) and developmental intellectual disability and the associated “years lived with disability” (YLDs) among children under 5 years of age in 2019. Methods: We analyzed the collaborative 2019 Rehabilitation Database of the Global Burden of Disease (GBD) Study and World Health Organization for neurological and mental disorders available for 204 countries and territories. Point prevalence and YLDs with 95% uncertainty intervals (UI) are presented. Results: Globally, 8.1 million (7.1–9.2) or 1.2% of children under 5 years are estimated to have CP with 16.1 million (11.5–21.0) or 2.4% having intellectual disability. Over 98% resided in low-income and middle-income countries (LMICs). CP and intellectual disability accounted for 6.5% and 4.5% of the aggregate YLDs from all causes of adverse health outcomes respectively. African Region recorded the highest prevalence of CP (1.6%) while South-East Asia Region had the highest prevalence of intellectual disability. The top 10 countries accounted for 57.2% of the global prevalence of CP and 62.0% of the global prevalence of intellectual disability. Conclusion: Based on this Database, CP and intellectual disability are highly prevalent and associated with substantial YLDs among children under 5 years worldwide. Universal early detection and support services are warranted, particularly in LMICs to optimize school readiness for these children toward inclusive education as envisioned by the United Nations' Sustainable Development Goals.

BACKGROUND: Estimates of children and adolescents with disabilities worldwide are needed to inform global intervention under the disability-inclusive provisions of the Sustainable Development Goals. We sought to update the most widely reported estimate of 93 million children,15 years with disabilities from the Global Burden of Disease Study 2004. METHODS: We analyzed Global Burden of Disease Study 2017 data on the prevalence of childhood epilepsy, intellectual disability, and vision or hearing loss and on years lived with disability (YLD) derived from systematic reviews, health surveys, hospital and claims databases, cohort studies, and disease-specific registries. Point estimates of the prevalence and YLD and the 95% uncertainty intervals (UIs) around the estimates were assessed. RESULTS: Globally, 291.2 million (11.2%) of the 2.6 billion children and adolescents (95% UI: 249.9–335.4 million) were estimated to have 1 of the 4 specified disabilities in 2017. The prevalence of these disabilities increased with age from 6.1% among children aged,1 year to 13.9% among adolescents aged 15 to 19 years. A total of 275.2 million (94.5%) lived in low- and middle-income countries, predominantly in South Asia and sub-Saharan Africa. The top 10 countries accounted for 62.3% of all children and adolescents with disabilities. These disabilities accounted for 28.9 million YLD or 19.9% of the overall 145.3 million (95% UI: 106.9–189.7) YLD from all causes among children and adolescents. CONCLUSIONS: The number of children and adolescents with these 4 disabilities is far higher than the 2004 estimate, increases from infancy to adolescence, and accounts for a substantial proportion of all-cause YLD.

Background: Neural tube defects continue to be one of the main congenital malformations affecting the development of the nervous system and a significant cause of disability and disease burden to individuals living with these conditions. Mandatory food fortification with folic acid is, by far, one of the most efficacious, safe, and cost-effective interventions to prevent neural tube defects. However, most countries fail to effectively fortify staple foods with folic acid, impacting public health and healthcare systems and generating dismal disparities. Aim: This article discusses the main barriers and facilitators for implementing mandatory food fortification as an evidence-based policy to prevent neural tube defects worldwide. Methods: A comprehensive review of the scientific literature allowed the identification of the determinant factors acting as barriers or facilitators for the reach, adoption, implementation, and scaling up of mandatory food fortification with folic acid as an evidence-based policy. Results: We identified eight barriers and seven facilitators as determinant factors for food fortification policies. The identified factors were classified as individual, contextual, and external, inspired by the Consolidated Framework for Implementation of Research (CFIR). We discuss mechanisms to overcome obstacles and seize the opportunities to approach this public health intervention safely and effectively. Conclusions: Several determinant factors acting as barriers or facilitators influence the implementation of mandatory food fortification as an evidence-based policy worldwide. Notoriously, policymakers in many countries may lack knowledge of the benefits of scaling up their policies to prevent folic acid-sensitive neural tube defects, improve the health status of their communities, and promote the protection of many children from these disabling but preventable conditions. Not addressing this problem negatively affects four levels: public health, society, family, and individuals. Science-driven advocacy and partnerships with essential stakeholders can help overcome the barriers and leverage the facilitators for safe and effective food fortification.

Background: Neural tube defects are common birth defects resulting in severe morbidity and mortality; they can largely be prevented with periconceptional maternal intake of folic acid. Understanding the occurrence of neural tube defects and their contribution to mortality in settings where their burden is highest could inform prevention and health-care policy. We aimed to estimate the mortality attributed to neural tube defects in seven countries in sub-Saharan Africa and southeast Asia. Methods: This analysis used data from the Child Health and Mortality Prevention Surveillance (CHAMPS) network and health and demographic surveillance systems from South Africa, Mozambique, Bangladesh, Kenya, Mali, Ethiopia, and Sierra Leone. All stillbirths and infants and children younger than 5 years who died, who were enrolled in CHAMPS, whose families consented to post-mortem minimally invasive tissue sampling (MITS) between Jan 1, 2017, and Dec 31, 2021, and who were assigned a cause of death by a determination of cause of death panel as of May 24, 2022, were included in this analysis, regardless the cause of death. MITS and advanced diagnostic methods were used to describe the frequency and characteristics of neural tube defects among eligible deaths, identify risk factors, and estimate the mortality fraction and mortality rate (per 10 000 births) by CHAMPS site. Findings: Causes of death were determined for 3232 stillbirths, infants, and children younger than 5 years, of whom 69 (2%) died with a neural tube defect. Most deaths with a neural tube defect were stillbirths (51 [74%]); 46 (67%) were neural tube defects incompatible with life (ie, anencephaly, craniorachischisis, or iniencephaly) and 22 (32%) were spina bifida. Deaths with a neural tube defect were more common in Ethiopia (adjusted odds ratio 8·09 [95% CI 2·84–23·02]), among female individuals (4·40 [2·44–7·93]), and among those whose mothers had no antenatal care (2·48 [1·12–5·51]). Ethiopia had the highest adjusted mortality fraction of deaths with neural tube defects (7·5% [6·7–8·4]) and the highest adjusted mortality rate attributed to neural tube defects (104·0 per 10 000 births [92·9–116·4]), 4–23 times greater than in any other site. Interpretation: CHAMPS identified neural tube defects, a largely preventable condition, as a common cause of death among stillbirths and neonatal deaths, especially in Ethiopia. Implementing interventions such as mandatory folic acid fortification could reduce mortality due to neural tube defects. Funding: Bill & Melinda Gates Foundation.

July 20, 2021 marked the 30th anniversary of the publication of the landmark trial by the British Medical Research Council showing unequivocally that maternal intake of folic acid (vitamin B9) starting before pregnancy prevents most cases of infant spina bifida and anencephaly—two major neural tube defects that are severe, disabling, and often fatal. Mandatory food fortification with folic acid is a safe, cost-effective, and sustainable intervention to prevent spina bifida and anencephaly. Yet few countries implement fortification with folic acid; only a quarter of all preventable spina bifida and anencephaly cases worldwide are currently avoided by food fortification. We summarise scientific evidence supporting immediate, mandatory fortification with folic acid to prevent the development of spina bifida and anencephaly. We make an urgent call to action for the World Health Assembly to pass a resolution for universal mandatory folic acid fortification. Such a resolution could accelerate the slow pace of spina bifida and anencephaly prevention globally, and will assist countries to reach their 2030 Sustainable Development Goals on child mortality and health equity. The cost of inaction is profound, and disproportionately impacts susceptible populations in low-income and middle-income countries.

Background: Breastfeeding duration is an important indicator commonly measured in maternal and child health and nutrition research. Maternal short-term recall for both initiation and duration of breastfeeding has been shown to be valid; however, validity of long-term recall is not well understood. Research aim: This study aims to assess the validity of maternal recall of breastfeeding duration 6 years after childbirth and its association with sociodemographic factors. Methods: Among 635 mother-child pairs, breastfeeding duration data collected monthly throughout the 1st year after childbirth in the Infant Feeding Practices Study II (IFPS II) were compared to recall data obtained 6 years later during the Year 6 Follow-Up. The intraclass correlation coefficient (ICC) and Bland-Altman plots were examined to study the agreement between the two data sets. Sociodemographic factors associated with accurate recall to within 1 month of the IFPS II breastfeeding duration were assessed using multivariable logistic regression modeling. Results: Maternal recall of breastfeeding duration was found to be valid 6 years after childbirth with a small median overall bias (1 week) toward overestimation. The overall concordance was high (ICC = 0.84), except for high school graduates (ICC = 0.63) and smokers (ICC = 0.61). Smokers (adjusted odds ratio = 0.52; 95% confidence interval [0.4, 0.8]) and multiparous women (adjusted odds ratio = 0.57; 95% confidence interval [0.4, 0.9]) were also less likely to give an accurate recall of their breastfeeding duration to within 1 month. Conclusion: Our study found that maternal recall of breastfeeding duration varies by sociodemographic factors but is accurate 6 years after childbirth.