Birth Prevalence Of Spina Bifida Research Articles

Birth prevalence and risk factors of neural tube defects in Ethiopia: a systematic review and meta-analysis

ObjectiveThis study aims to estimate the prevalence of neural tube defects (NTDs) and to identify potential risk factors in the Ethiopian context.Study designSystematic review and meta-analysis.Study participantsA total of 611...

Birth prevalence of neural tube defects in eastern Africa: a systematic review and meta-analysis

BackgroundNeural tube defects (NTDs) are associated with high rates of neonatal mortality and morbidity worldwide. The promotion of folic acid fortification and supplementation in pregnant women by the Food and Drug Administration significantly decreased the incidence of NTDs in the United States. This practice is not widely adopted in Eastern Africa countries. We hypothesized that these countries experience a higher burden of NTDs than countries that promote the use of folic acid. We aimed to estimate the birth prevalence of NTDs in the United Nations (UN) Eastern African region.MethodsPubMed (Medline), Embase, and Cochrane Library databases were systematically searched from inception to December 17, 2021. We included randomized controlled trials or observational studies that reported the prevalence estimates of NTDs in Eastern Africa. Random effects model was used to pool the effect estimates. The GRADE (Grading of Recommendations, Assessment, Development and Evaluation) approach was used to assess the certainty of the evidence. Outcome measures were overall and specific (spina bifida, anencephaly, encephalocele) rates of NTDs per 10,000 births, including live and stillborn cases.ResultsThe meta-analysis included 20 studies consisting of 752,936 individuals. The pooled prevalence of all NTDs per 10,000 births in Eastern Africa was 33.30 (95% CI: 21.58 to 51.34). Between-study heterogeneity was high (I2 = 97%, p < 0.0001), The rate was highest in Ethiopia (60 per 10,000). Birth prevalence of spina bifida (20 per 10,000) was higher than anencephaly (9 per 10,000) and encephalocele (2.33 per 10,000). No studies on NTDs were identified in 70% of the UN Eastern Africa region. Birth prevalence increased by 4% per year from 1983 to 2018. The level of evidence as qualified with GRADE was moderate.ConclusionThe birth prevalence of NTDs in the United Nations region of Eastern Africa is 5 times as high as observed in Western countries with mandatory folic acid supplementation in place. Therefore, mandatory folic acid supplementation of stable foods may decrease the risk of NTDs in Eastern Africa.

Prevalence of Spina Bifida among Newborns in Africa: A Systematic Review and Meta-Analysis.

Spina bifida is an abnormal closure of the neural tube during the fourth week of development. It is the major cause of fetal loss and considerable disabilities in newborns. The aim of this review is to determine the pooled prevalence of spina bifida among newborns in Africa. PubMed/Medline, Google Scholar, Science Direct, Joanna Briggs Institute (JBI) Library, Cochrane Library, Web of Science, African Journals Online, and Embase databases were systematically searched. Cochran Q test and I2 test statistics were applied to assess heterogeneity across studies. A random-effect model was applied to calculate the pooled prevalence of spina bifida. Forest plot and Galbraith's plot were used to visualize heterogeneity. Subgroup, sensitivity, meta-regression, and meta-cumulative analyses were performed. All essential data were extracted using a standardized data extraction format, and the JBI quality appraisal checklist was used to assess the quality of studies. Egger's test and Begg's test were used in order to detect the publication bias. In the present systematic review and meta-analysis, 6,587,298 births in twenty-seven studies were included. The pooled birth prevalence of spina bifida in Africa was 0.13% with a range between 0.12% and 0.14%. In Africa, the highest burden of spina bifida was detected in Algeria (0.43%), Ethiopia (0.32%), Tanzania (0.26%), Cameron (0.12%), Egypt (0.10%), and South Africa (0.10%). The lowest burden of spina bifida was detected in Libya (0.006%) and Tunisia (0.009%). The high birth prevalence of spina bifida was detected in Africa. There was a significant variation in the prevalence of spina bifida among study countries in Africa. The authors recommend that special awareness creation with the help of health education intervention should be provided for mothers to focus on prevention in order to reduce the burden of spina bifida.

Language and Latino immigrants living with spina bifida: Social determinants of health - the missing dimension in quality of life research.

Aware of the higher birth prevalence of spina bifida (SB) among Hispanics/Latinos, we aimed to appraise the literature as it relates to cultural context through a review of quality of life (QOL) studies conducted among individuals with SB in order to improve care among immigrant families. A systematic review was conducted consistent with the Preferred Reporting Items for Systematic Review and Meta-Analyses (PRISMA) guidelines. The inclusion criteria were: (1) children and adolescents (5-21years of age) with SB and/or myelomeningocele; (2) quantitative studies; (3) health-related QOL outcome measured by validated instrument determinants; and (4) US-based studies. Articles meeting inclusion criteria were assessed using the focused conceptual framework informing the study (i.e., social determinants of health). Eighteen studies met inclusion criteria, with eight different QOL instruments represented. The majority of studies used generic assessments of QOL (72%), two reported the use of both a generic and a SB-specific QOL measure (11%), and three (17%) documented QOL utilizing a SB-specific validated instrument. Only seven (39%) of the studies stated that they included Hispanics/Latinos and only six (33%) reported including Spanish-speaking individuals. QOL in individuals with SB is mediated by a wide-range of interrelated factors. In order to better serve this vulnerable population as they transition across the lifespan, multilingual condition-specific QOL measures need to be further developed and implemented among Hispanic/Latino individuals with SB, especially those who are recent immigrants.

Association of maternal risk factors with the recent rise of neural tube defects in Canada.

We sought to assess the recent trend in NTD prevalence at birth in the post-folic acid food fortification era and to identify the maternal risk factors associated with that trend. We carried out a population-based study of all livebirths and stillbirths (including late pregnancy terminations) delivered in hospitals in Canada (excluding Quebec) from 2004 to 2015 (n=3439330). We examined NTD birth prevalence by year, multiple pregnancy, maternal age, parity, pregestational diabetes, chronic illness, and problematic substance use. Poisson regression was used to quantify the association between spina bifida and cranial defects and maternal characteristics and other risk factors. We identified 1517 non-chromosomal NTDs, yielding a birth prevalence of 4.4 per 10000 total births. NTD prevalence rose from 3.6 in 2004 to 4.6 per 10000 in 2015 (Ptrend =0.03). Among NTD subtypes, only spina bifida showed a temporal increase (Ptrend =0.03). Birth prevalence of spina bifida was higher among younger mothers, those with type 2 diabetes (rate ratio (RR) 3.74, 95% confidence interval (CI) 2.21, 6.35), chronic illness (RR 3.16, 95% CI 1.97, 5.07), and problematic substance use (RR 1.88, 95% CI 1.31, 2.71). Adjusting for risk factors attenuated the significant temporal trend in spina bifida (unadjusted average annual prevalence ratio (aAAPR) 1.016, 95% CI 1.001, 1.032; adjusted AAPR 1.014, 95% CI 0.998, 1.029). Increases in the frequency of maternal risk factors such as pregestational diabetes mellitus, substance use, and chronic illness may be partly responsible for the recent rise in NTDs, particularly spina bifida.

Scorecard for spina bifida research, prevention, and policy: Score analysis by Human Development Index and WHO region

The People and Organizations United for Spina Bifida and Hydrocephalus (PUSH!) Global Alliance created scorecards to rate country-level performance on spina bifida, including folate status among women of reproductive age; birth prevalence and mortality associated with spina bifida; prevention strategies; access to care; and the country's engagement in the UN Convention on the Rights of Persons with Disabilities. A total of 187 countries were examined based on a criteria-based scoring system represented by stars (http://www.pu-sh.org). This paper summarized scores by Human Development Index (HDI) classification spanning six World Health Organization (WHO) regions. For every indicator stratified by HDI classification, a greater proportion of ‘Very High’ and ‘High’ HDI countries scored a full star (highest performance) compared to countries in the ‘Medium’ and ‘Low’ HDI strata. A majority of countries in the South East Asia (SEARO) region received full stars for availability of published studies on folate status among women of reproductive age, and most countries in the European region scored full stars for surveying birth prevalence of spina bifida. Very few countries in the European (EURO) and SEARO region had full stars for prevention strategy for spina bifida through fortification. Overall, 90% of countries did not have published studies on spina bifida mortality. This is the first time country-level performance for spina bifida research, prevention and care, grouped by HDI and WHO region has been examined. Policy-makers and stakeholders can use our analyses as benchmarks in their efforts to improve spina bifida surveillance, prevention and care and to close gaps.

Global Birth Prevalence of Spina Bifida by Folic Acid Fortification Status: A Systematic Review and Meta-Analysis

Background. Birth defects remain a significant source of worldwide morbidity and mortality. Strong scientific evidence shows that folic acid fortification of a region’s food supply leads to a decrease in spina bifida (a birth defect of the spine). Still, many countries around the world have yet to approve mandatory fortification through government legislation. Objectives. We sought to perform a systematic review and meta-analysis of period prevalence of spina bifida by folic acid fortification status, geographic region, and study population. Search methods. An expert research librarian used terms related to neural tube defects and epidemiology from primary research from 1985 to 2010 to search in EMBASE and MEDLINE. We searched the reference lists of included articles and key review articles identified by experts. Selection criteria. Inclusion criteria included studies in English or French reporting on prevalence published between January 1985 and December 2010 that (1) were primary research, (2) were population-based, and (3) reported a point or period prevalence estimate of spina bifida (i.e., prevalence estimate with confidence intervals or case numerator and population denominator). Two independent reviewers screened titles and abstracts for eligible articles, then 2 authors screened full texts in duplicate for final inclusion. Disagreements were resolved through consensus or a third party. Data collection and analysis. We followed Preferred Reporting Items for Systematic Reviews and Meta-Analyses, or PRISMA, abstracting data related to case ascertainment, study population, folic acid fortification status, geographic region, and prevalence estimate independently and in duplicate. We extracted overall data and any subgroups reported by age, gender, time period, or type of spina bifida. We classified each period prevalence estimate as “mandatory” or “voluntary” folic acid fortification according to each country’s folic acid fortification status at the time data were collected (as determined by a well-recognized fortification monitoring body, Food Fortification Initiative). We determined study quality on the basis of sample representativeness, standardization of data collection and birth defect assessment, and statistical analyses. We analyzed study-level period prevalence estimates by using a random effects model (α level of &lt; 0.05) for all meta-analyses. We stratified pooled period prevalence estimates by birth population, fortification status, and continent. Results. Of 4078 studies identified, we included 179 studies in the systematic review and 123 in a meta-analysis. In studies of live births (LBs) alone, period prevalences of spina bifida were (1) lower in geographical regions with mandatory (33.86 per 100 000 LBs) versus voluntary (48.35 per 100 000 LBs) folic acid fortification, and (2) lower in studies of LBs, stillbirths, and terminations of pregnancy in regions with mandatory (35.22 per 100 000 LBs) versus voluntary (52.29 per 100 000 LBs) fortification. In LBs, stillbirths, and terminations of pregnancy studies, the lowest pooled prevalence estimate was in North America (38.70 per 100 000). Case ascertainment, surveillance methods, and reporting varied across these population-based studies. Conclusions. Mandatory legislation enforcing folic acid fortification of the food supply lags behind the evidence, particularly in Asian and European countries. This extensive literature review shows that spina bifida is significantly more common in world regions without government legislation regulating full-coverage folic acid fortification of the food supply (i.e., Asia, Europe) and that mandatory folic acid fortification resulted in a lower prevalence of spina bifida regardless of the type of birth cohort. African data were scarce, but needed, as many African nations are beginning to adopt folic acid legislation.

Global Birth Prevalence of Spina Bifida by Folic Acid Fortification Status: A Systematic Review and Meta-Analysis.

Birth defects remain a significant source of worldwide morbidity and mortality. Strong scientific evidence shows that folic acid fortification of a region's food supply leads to a decrease in spina bifida (a birth defect of the spine). Still, many countries around the world have yet to approve mandatory fortification through government legislation. We sought to perform a systematic review and meta-analysis of period prevalence of spina bifida by folic acid fortification status, geographic region, and study population. An expert research librarian used terms related to neural tube defects and epidemiology from primary research from 1985 to 2010 to search in EMBASE and MEDLINE. We searched the reference lists of included articles and key review articles identified by experts. Inclusion criteria included studies in English or French reporting on prevalence published between January 1985 and December 2010 that (1) were primary research, (2) were population-based, and (3) reported a point or period prevalence estimate of spina bifida (i.e., prevalence estimate with confidence intervals or case numerator and population denominator). Two independent reviewers screened titles and abstracts for eligible articles, then 2 authors screened full texts in duplicate for final inclusion. Disagreements were resolved through consensus or a third party. We followed Preferred Reporting Items for Systematic Reviews and Meta-Analyses, or PRISMA, abstracting data related to case ascertainment, study population, folic acid fortification status, geographic region, and prevalence estimate independently and in duplicate. We extracted overall data and any subgroups reported by age, gender, time period, or type of spina bifida. We classified each period prevalence estimate as "mandatory" or "voluntary" folic acid fortification according to each country's folic acid fortification status at the time data were collected (as determined by a well-recognized fortification monitoring body, Food Fortification Initiative). We determined study quality on the basis of sample representativeness, standardization of data collection and birth defect assessment, and statistical analyses. We analyzed study-level period prevalence estimates by using a random effects model (α level of < 0.05) for all meta-analyses. We stratified pooled period prevalence estimates by birth population, fortification status, and continent. Of 4078 studies identified, we included 179 studies in the systematic review and 123 in a meta-analysis. In studies of live births (LBs) alone, period prevalences of spina bifida were (1) lower in geographical regions with mandatory (33.86 per 100,000 LBs) versus voluntary (48.35 per 100,000 LBs) folic acid fortification, and (2) lower in studies of LBs, stillbirths, and terminations of pregnancy in regions with mandatory (35.22 per 100,000 LBs) versus voluntary (52.29 per 100,000 LBs) fortification. In LBs, stillbirths, and terminations of pregnancy studies, the lowest pooled prevalence estimate was in North America (38.70 per 100,000). Case ascertainment, surveillance methods, and reporting varied across these population-based studies. Mandatory legislation enforcing folic acid fortification of the food supply lags behind the evidence, particularly in Asian and European countries. This extensive literature review shows that spina bifida is significantly more common in world regions without government legislation regulating full-coverage folic acid fortification of the food supply (i.e., Asia, Europe) and that mandatory folic acid fortification resulted in a lower prevalence of spina bifida regardless of the type of birth cohort. African data were scarce, but needed, as many African nations are beginning to adopt folic acid legislation.

Contemporary Epidemiological Trends in Complex Congenital Genitourinary Anomalies

Anecdotal evidence suggests that complex congenital genitourinary anomalies are occurring less frequently. However, few epidemiological studies are available to confirm or refute this suggestion. The Kids' Inpatient Database (KID) is a national, all payer database of several million inpatient pediatric hospitalizations per year, including complicated and uncomplicated in-hospital births. We reviewed the 1997 to 2009 KID to determine the birth prevalence of spina bifida, posterior urethral valves, bladder exstrophy, epispadias, prune belly syndrome, ambiguous genitalia and imperforate anus. For posterior urethral valves and prune belly syndrome we limited our search to newborn males only. During the study period, there was a diagnosis of spina bifida in 3,413 neonates, bladder exstrophy in 214, epispadias in 1,127, ambiguous genitalia in 726, prune belly syndrome in 180, posterior urethral valves in 578 and imperforate anus in 4,040. We identified no significant change in the birth prevalence of spina bifida (from 33.9 new spina bifida births of 100,000 uncomplicated births to 29.0/100,000, p = 0.08), posterior urethral valves (from 10.4/100,000 to 11.0/100,000, p = 0.51), prune belly syndrome (from 4.8/100,000 to 3.3/100,000, p = 0.44) or ambiguous genitalia (from 5.82/100,000 to 5.87/100,000, p = 0.38). There was a significant decrease in the birth prevalence of bladder exstrophy (from 2.4/100,000 to 1.6/100,000 uncomplicated births, p = 0.01) and a significant increase in epispadias (from 8.0/100,000 to 11.6/100,000) and imperforate anus (from 33.6/100,000 to 35.0/100,000, each p = 0.04) during the study period. The birth prevalence of spina bifida, posterior urethral valves and prune belly syndrome appears to have been stable in the last 12 years. Epispadias, ambiguous genitalia and imperforate anus diagnoses in newborns became more common in the same period, while bladder exstrophy diagnoses became less common.

Neural tube defects in 21st century: is Northern Ireland changing?

Background Neural tube defects (NTD), which include spina bifida, anencephaly, and encephalocoele, are an important group of severe birth defects whose prevalence has fallen significantly in past few decades in many countries. Northern Ireland (NI) is known to have the highest prevalence of neural tube defects (NTD) in Europe. The prevalence rate of 60 and 40 per 10,000 births was reported in 1970's and 1980's, respectively. However no NI data has been published since then to assess the trend of decline in NTD prevalence as noted in other previously high prevalent regions of UK and Ireland. The aims of the study were (1) To describe the current epidemiology of NTD in Northern Ireland (2) To assess the impact of prenatal diagnosis on the birth prevalence of spina bifida and anencephaly (3) To compare the current prevalence, antenatal diagnosis and termination trend with previously published reports from the province (3) To compare the current NI data with rest of UK and Europe for the same period.

Trends in the postfortification prevalence of spina bifida and anencephaly in the United States.

The prevalence of NTDs in the US declined significantly after mandatory folic acid fortification; however, it is not known if the prevalence of NTDs has continued to decrease in recent years relative to the period immediately following the fortification mandate. Population-based data from 21 birth defects surveillance systems were used to examine trends in the birth prevalence of spina bifida and anencephaly during 1999-2000, 2001-2002, and 2003-2004. Prevalence data were stratified by non-Hispanic White, non-Hispanic Black, and Hispanic race or ethnicity. Prevalence ratios were calculated by dividing the birth prevalences during the later time periods (2001-2002 and 2003-2004) by the birth prevalences during 1999-2000. During 1999-2004, 3,311 cases of spina bifida and 2,116 cases of anencephaly were reported. Hispanic infants had the highest prevalences of NTDs for all years. For all infants, the combined birth prevalences of spina bifida and anencephaly decreased 10% from the 1999-2000 period to the 2003-2004 period. The decline in spina bifida (3%) was not significant; however the decline in anencephaly (20%) was statistically significant. While the prevalences of spina bifida and anencephaly in the United States have declined since folic acid fortification in the food supply began, these data suggest that reductions in the prevalence of anencephaly continued during 2001-2004 and that racial and ethnic and other disparities remain.

Audit of prenatal and postnatal diagnosis of isolated open spina bifida in three university hospitals in The Netherlands.

To audit the current Dutch policy of prenatal detection of isolated open spina bifida based on offering detailed ultrasound examination only on indication. A retrospective analysis of prenatally diagnosed isolated spina bifida cases and of newborns diagnosed with this condition was carried out in three university hospitals. The data were collected from databases and clinical records of the departments of prenatal diagnosis, obstetrics, neonatology, child neurology and neurosurgery of the three centers. Between January 1996 and December 1999, 88 cases of isolated open spina bifida were diagnosed prenatally by ultrasound investigation. Thirty-eight cases (43%) were diagnosed before the 24th week of gestation. Of these, 35 (92%) ended in termination of the pregnancy at the parents' request. Of the remaining 50 cases (57%) diagnosed after the 24th week of gestation, eight (16%) pregnancies were terminated beyond the legal limit for termination due to the severity of the condition. Of the 88 cases of isolated spina bifida, 25 infants (28%) were still alive at the age of 4 years. In the same audit period 112 newborn infants with isolated open spina bifida were admitted to the neonatology, child neurology, or neurosurgery ward of the three centers. Of these cases, 47 (42%) had been diagnosed prenatally and 65 (58%) were an unexpected finding at birth. In 24 infants (21%) surgical treatment was withheld because of the severity of the condition and predicted poor outcome, whereas the remaining 88 infants (79%) underwent surgical repair. The current practice in The Netherlands of offering ultrasound screening to high-risk patients only leads to the early detection of a minority of cases of spina bifida. Most cases are diagnosed either after the 24th week of gestation or they remain undiagnosed until after birth. When spina bifida is diagnosed before the 24th week of gestation the vast majority of parents opt for termination. In order to reduce the birth prevalence of spina bifida in The Netherlands the introduction of a policy of routine ultrasound screening should be considered.

Prevalence of Spina Bifida at Birth — United States, 1983–1990: a Comparison of Two Surveillance Systems

Problem/Condition Spina bifida is a birth defect of the spinal column that is a substantial contributor to serious developmental disabilities in the United States. The risk for spina bifida and other neural tube defects (NTDs) can be reduced if women consume 0.4 mg of folic acid before and during the first trimester of pregnancy. Public health programs are being developed to prevent many NTDs by increasing the consumption of folic acid by women of childbearing age. To assess the national impact of these programs on the prevalence of NTDs at birth, multistate surveillance is needed to monitor secular trends in birth-prevalence rates. This report summarizes a collaborative effort by CDC and state birth defect surveillance programs in 16 states to a) obtain multistate, population-based data concerning the birth prevalence and descriptive epidemiology of spina bifida and b) determine the usefulness of combining state surveillance data to monitor national trends in the birth prevalence of NTDs. Reporting Period This report presents data from birth defects surveillance systems in 16 states for the period 1983–1990 (specific periods covered varied by state). These findings are compared with CDC's Birth Defects Monitoring Program (BDMP) for the same period. Description of Systems Population-based data about live-born and stillborn infants who have spina bifida were analyzed from 16 state programs. These 16 programs differed in size and racial/ethnic composition of the populations, surveillance methods, and completeness of case ascertainment. Hospital-based data about live-born and stillborn infants who have spina bifida also were analyzed from BDMP, a passive case ascertainment surveillance system that obtains data from participating hospitals in 50 states. Results and Interpretation From 1983 through 1990, the birth-prevalence rate for spina bifida for the 16 states was 4.6 cases per 10,000 births; the BDMP rate was nearly identical (4.4 cases). State-specific rates varied substantially, ranging from 3.0 (Washington) to 7.8 (Arkansas). Both state-based and BDMP rates varied among racial/ethnic groups; in both systems, the rates were highest for Hispanics and lowest for Asians/Pacific Islanders. In both the state-based surveillance systems and BDMP, the annual rate of spina bifida for the total population declined during the period 1983–1990. Much of this decline can be attributed to increased prenatal diagnosis in the 1980s. However, because the decline in the rates of spina bifida and other NTDs in the United States began before the widespread availability of prenatal diagnostic services, an environmental component may have contributed substantially to the etiologies of these defects. The birth-prevalence rate of spina bifida was slightly higher among females than males. The ratio of female-to-male prevalence rates was 1.2 for both the state-based surveillance systems and BDMP. This ratio varied considerably among racial/ethnic groups and among states. The similarities of rates and trends in the birth prevalence of spina bifida between the state-based surveillance data and the BDMP data indicate that both types of surveillance systems can provide reliable information concerning national trends in the birth prevalence of spina bifida. Actions Taken CDC and state birth defects surveillance programs will use results from this analysis to monitor national trends in the birth prevalence of spina bifida in the United States. Aggregated state-based surveillance data about spina bifida, anencephaly, and other NTDs will facilitate the monitoring of changes in NTDs after implementation of programs to increase folic acid consumption by women of child-bearing age.

Prenatal screening and diagnosis of neural tube defects in England and Wales in 1985.

A survey was carried out to determine the effect of prenatal screening and therapeutic abortion on births in 1985 with anencephaly and spina bifida in England and Wales. Maternal serum alpha-fetoprotein tests were done on 399,288 women (60 per cent of pregnant women): 4 per cent were reported as being screen-positive and 1 per cent had an amniocentesis. An estimated 534 pregnancies associated with anencephaly were terminated and an estimated 445 pregnancies associated with spina bifida (but without anencephaly) were terminated. Most (63 per cent) of the anencephalic pregnancies were first suspected from an ultrasound examination; 57 per cent of the spina bifida pregnancies were first suspected from a positive maternal serum alpha-fetoprotein test, 35 per cent by ultrasound, and the remaining 8 per cent by other means. The birth prevalence of anencephaly declined by 94 per cent between 1964-1972 and 1985, but when the terminations of pregnancy on account of having a fetus with anencephaly are added to the births the decline in prevalence was only 50 per cent. The birth prevalence of spina bifida declined by 68 per cent over the same period but when the terminations were added to the births the decline in prevalence was only 32 per cent. Among births with anencephaly 66 per cent had had no screening or diagnostic tests in early pregnancy, but in those that did nearly all were positive--usually in twin pregnancies where one fetus was affected but not the other. Among births with spina bifida, 48 per cent had no tests and in those that did the results were mainly negative.(ABSTRACT TRUNCATED AT 250 WORDS)