Dataset Information

Analysis of Mortality among Neonates and Children with Spina Bifida: An International Registry-Based Study, 2001-2012.

ABSTRACT: BACKGROUND:Medical advancements have resulted in better survival and life expectancy among those with spina bifida, but a significantly increased risk of perinatal and postnatal mortality for individuals with spina bifida remains. OBJECTIVES:To examine stillbirth and infant and child mortality among those affected by spina bifida using data from multiple countries. METHODS:We conducted an observational study, using data from 24 population- and hospital-based surveillance registries in 18 countries contributing as members of the International Clearinghouse for Birth Defects Surveillance and Research (ICBDSR). Cases of spina bifida that resulted in livebirths or stillbirths from 20 weeks' gestation or elective termination of pregnancy for fetal anomaly (ETOPFA) were included. Among liveborn spina bifida cases, we calculated mortality at different ages as number of deaths among liveborn cases divided by total number of liveborn cases with spina bifida. As a secondary outcome measure, we estimated the prevalence of spina bifida per 10 000 total births. The 95% confidence interval for the prevalence estimate was estimated using the Poisson approximation of binomial distribution. RESULTS:Between years 2001 and 2012, the overall first-week mortality proportion was 6.9% (95% CI 6.3, 7.7) and was lower in programmes operating in countries with policies that allowed ETOPFA compared with their counterparts (5.9% vs. 8.4%). The majority of first-week mortality occurred on the first day of life. In programmes where information on long-term mortality was available through linkage to administrative databases, survival at 5 years of age was 90%-96% in Europe, and 86%-96% in North America. CONCLUSIONS:Our multi-country study showed a high proportion of stillbirth and infant and child deaths among those with spina bifida. Effective folic acid interventions could prevent many cases of spina bifida, thereby preventing associated childhood morbidity and mortality.

SUBMITTER: Bakker MK

PROVIDER: S-EPMC6899817 | biostudies-literature | 2019 Nov

REPOSITORIES: biostudies-literature

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Publications

Analysis of Mortality among Neonates and Children with Spina Bifida: An International Registry-Based Study, 2001-2012.

Bakker Marian K MK Kancherla Vijaya V Canfield Mark A MA Bermejo-Sanchez Eva E Cragan Janet D JD Dastgiri Saeed S De Walle Hermien E K HEK Feldkamp Marcia L ML Groisman Boris B Gatt Miriam M Hurtado-Villa Paula P Kallen Karin K Landau Daniella D Lelong Nathalie N Lopez Camelo Jorge S JS Martínez Laura L Morgan Margery M Mutchinick Osvaldo M OM Nembhard Wendy N WN Pierini Anna A Rissmann Anke A Sipek Antonin A Szabova Elena E Tagliabue Giovanna G Wertelecki Wladimir W Zarante Ignacio I Mastroiacovo Pierpaolo P

Paediatric and perinatal epidemiology 20191021 6

<h4>Background</h4>Medical advancements have resulted in better survival and life expectancy among those with spina bifida, but a significantly increased risk of perinatal and postnatal mortality for individuals with spina bifida remains.<h4>Objectives</h4>To examine stillbirth and infant and child mortality among those affected by spina bifida using data from multiple countries.<h4>Methods</h4>We conducted an observational study, using data from 24 population- and hospital-based surveillance re ...[more]

PMID: 31637749

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Project description:BackgroundChildren with the severe form of spina bifida (SBM: spina bifida with myelomeningocele with accompanying hydrocephalus) may manifest attention deficits, and have a similar psychological profile to children with hydrocephalus due to other etiologies. It is unclear to what extent tests to assess attention in SBM are confounded by the accompanying cognitive or visual-motor impairments. The aim of this study was to analyse attention functions by administering two different types of attention tests, one with high and the other with low cognitive and motor requirements. This enabled the possible interaction between attention and cognitive and motor impairment to be assessed.MethodsThe study group comprised 31 children with SBM with shunted hydrocephalus. Twenty children with SB-only formed a closely matched comparison group. Of these, 19 children with SBM and 18 with SB had a full-scale IQ (FSIQ) higher than 70. All had undergone spinal surgery and all children with SBM had been shunted within the first months of life. Between 6 and 15 years of age, the children were assessed on focused and sustained attention, encoding, and distractibility/impulsivity, using both traditional tests and computerized attention tests.ResultsCompared to the SB group, attention scores of children with SBM were lower on the traditional tests, but when interfering cognitive and visual-motor requirements were eliminated using the computerised tasks, most differences disappeared. Furthermore, in contrast to traditional attention tasks, computerized tests showed no significant correlations with IQ-scores and visual-motor skills.ConclusionAssessment of attention functions in children with SBM by traditional tests may be misleading, because this paediatric population with complex cerebral malformations has difficulty with the cognitive and visual-motor requirements. To control for these interactions, the use of both traditional and computerized attention tests is recommended.

Project description:OBJECTIVEAlthough the majority of patients with myelomeningocele have hydrocephalus, reported rates of hydrocephalus treatment vary widely. The purpose of this study was to determine the rate of surgical treatment for hydrocephalus in patients with myelomeningocele in the National Spina Bifida Patient Registry (NSBPR). In addition, the authors explored the variation in shunting rates across NSBPR institutions, examined the relationship between hydrocephalus, and the functional lesion level of the myelomeningocele, and evaluated for temporal trends in rates of treated hydrocephalus.METHODSThe authors queried the NSBPR to identify all patients with myelomeningoceles. Individuals were identified as having been treated for hydrocephalus if they had undergone at least 1 hydrocephalus-related operation. For each participating NSBPR institution, the authors calculated the proportion of patients with treated hydrocephalus who were enrolled at that site. Logistic regression was performed to analyze the relationship between hydrocephalus and the functional lesion level of the myelomeningocele and to compare the rate of treated hydrocephalus in children born before 2005 with those born in 2005 or later.RESULTSA total of 4448 patients with myelomeningocele were identified from 26 institutions, of whom 3558 patients (79.99%) had undergone at least 1 hydrocephalus-related operation. The rate of treated hydrocephalus ranged from 72% to 96% among institutions enrolling more than 10 patients. This difference in treatment rates between centers was statistically significant (p < 0.001). Insufficient data were available in the NSBPR to analyze reasons for the different rates of hydrocephalus treatment between sites. Multivariate logistic regression demonstrated that more rostral functional lesion levels were associated with higher rates of treated hydrocephalus (p < 0.001) but demonstrated no significant difference in hydrocephalus treatment rates between children born before versus after 2005.CONCLUSIONSThe rate of hydrocephalus treatment in patients with myelomeningocele in the NSBPR is 79.99%, which is consistent with the rates in previously published literature. The authors' data demonstrate a clear association between functional lesion level of the myelomeningocele and the need for hydrocephalus treatment.

Project description:BackgroundCancer is a major cause of death in children worldwide, and the recorded incidence tends to increase with time. Internationally comparable data on childhood cancer incidence in the past two decades are scarce. This study aimed to provide internationally comparable local data on the incidence of childhood cancer to promote research of causes and implementation of childhood cancer control.MethodsThis population-based registry study, devised by the International Agency for Research on Cancer in collaboration with the International Association of Cancer Registries, collected data on all malignancies and non-malignant neoplasms of the CNS diagnosed before age 20 years in populations covered by high-quality cancer registries with complete data for 2001-10. Incidence rates per million person-years for the 0-14 years and 0-19 years age groups were age-adjusted using the world standard population to provide age-standardised incidence rates (WSRs), using the age-specific incidence rates (ASR) for individual age groups (0-4 years, 5-9 years, 10-14 years, and 15-19 years). All rates were reported for 19 geographical areas or ethnicities by sex, age group, and cancer type. The regional WSRs for children aged 0-14 years were compared with comparable data obtained in the 1980s.FindingsOf 532 invited cancer registries, 153 registries from 62 countries, departments, and territories met quality standards, and contributed data for the entire decade of 2001-10. 385 509 incident cases in children aged 0-19 years occurring in 2·64 billion person-years were included. The overall WSR was 140·6 per million person-years in children aged 0-14 years (based on 284 649 cases), and the most common cancers were leukaemia (WSR 46·4), followed by CNS tumours (WSR 28·2), and lymphomas (WSR 15·2). In children aged 15-19 years (based on 100 860 cases), the ASR was 185·3 per million person-years, the most common being lymphomas (ASR 41·8) and the group of epithelial tumours and melanoma (ASR 39·5). Incidence varied considerably between and within the described regions, and by cancer type, sex, age, and racial and ethnic group. Since the 1980s, the global WSR of registered cancers in children aged 0-14 years has increased from 124·0 (95% CI 123·3-124·7) to 140·6 (140·1-141·1) per million person-years.InterpretationThis unique global source of childhood cancer incidence will be used for aetiological research and to inform public health policy, potentially contributing towards attaining several targets of the Sustainable Development Goals. The observed geographical, racial and ethnic, age, sex, and temporal variations require constant monitoring and research.FundingInternational Agency for Research on Cancer and the Union for International Cancer Control.

Dataset Information

Analysis of Mortality among Neonates and Children with Spina Bifida: An International Registry-Based Study, 2001-2012.

Publications

Analysis of Mortality among Neonates and Children with Spina Bifida: An International Registry-Based Study, 2001-2012.

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