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      Age-specific global epidemiology of hydrocephalus: Systematic review, metanalysis and global birth surveillance

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          Abstract

          Background

          Hydrocephalus is a debilitating disorder, affecting all age groups. Evaluation of its global epidemiology is required for healthcare planning and resource allocation.

          Objectives

          To define age-specific global prevalence and incidence of hydrocephalus.

          Methods

          Population-based studies reporting prevalence of hydrocephalus were identified (MEDLINE, EMBASE, Cochrane, and Google Scholar (1985–2017)). Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines were followed. Two authors reviewed abstracts, full text articles and abstracted data. Metanalysis and meta-regressions were used to assess associations between key variables. Heterogeneity and publication bias were assessed. Main outcome of interest was hydrocephalus prevalence among pediatric (≤ 18 years), adults (19–64 years), and elderly (≥ 65) patients. Annual hydrocephalus incidence stratified by country income level and folate fortification requirements were obtained (2003–2014) from the International Clearinghouse for Birth Defects Surveillance and Research (ICBDSR).

          Results

          Of 2,460 abstracts, 52 met review eligibility criteria (aggregate population 171,558,651). Mean hydrocephalus prevalence was 85/100,000 [95% CI 62, 116]. The prevalence was 88/100,000 [95% CI 72, 107] in pediatrics; 11/100,000 [95% CI 5, 25] in adults; and 175/100,000 [95% CI 67, 458] in the elderly. The ICBDSR-based incidence of hydrocephalus diagnosed at birth remained stable over 11 years: 81/100,000 [95% CI 69, 96]. A significantly lower incidence was identified in high-income countries.

          Conclusion

          This systematic review established age-specific global hydrocephalus prevalence. While high-income countries had a lower hydrocephalus incidence according to the ICBDSR registry, folate fortification status was not associated with incidence. Our findings may inform future healthcare resource allocation and study.

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          Most cited references97

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          Prevention of neural tube defects: results of the Medical Research Council Vitamin Study. MRC Vitamin Study Research Group.

          A randomised double-blind prevention trial with a factorial design was conducted at 33 centres in seven countries to determine whether supplementation with folic acid (one of the vitamins in the B group) or a mixture of seven other vitamins (A,D,B1,B2,B6,C and nicotinamide) around the time of conception can prevent neural tube defects (anencephaly, spina bifida, encephalocele). A total of 1817 women at high risk of having a pregnancy with a neural tube defect, because of a previous affected pregnancy, were allocated at random to one of four groups--namely, folic acid, other vitamins, both, or neither. 1195 had a completed pregnancy in which the fetus or infant was known to have or not have a neural tube defect; 27 of these had a known neural tube defect, 6 in the folic acid groups and 21 in the two other groups, a 72% protective effect (relative risk 0.28, 95% confidence interval 0.12-0.71). The other vitamins showed no significant protective effect (relative risk 0.80, 95% Cl 0.32-1.72). There was no demonstrable harm from the folic acid supplementation, though the ability of the study to detect rare or slight adverse effects was limited. Folic acid supplementation starting before pregnancy can now be firmly recommended for all women who have had an affected pregnancy, and public health measures should be taken to ensure that the diet of all women who may bear children contains an adequate amount of folic acid.
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            Hydrocephalus in children.

            Hydrocephalus is a common disorder of cerebral spinal fluid (CSF) physiology resulting in abnormal expansion of the cerebral ventricles. Infants commonly present with progressive macrocephaly whereas children older than 2 years generally present with signs and symptoms of intracranial hypertension. The classic understanding of hydrocephalus as the result of obstruction to bulk flow of CSF is evolving to models that incorporate dysfunctional cerebral pulsations, brain compliance, and newly characterised water-transport mechanisms. Hydrocephalus has many causes. Congenital hydrocephalus, most commonly involving aqueduct stenosis, has been linked to genes that regulate brain growth and development. Hydrocephalus can also be acquired, mostly from pathological processes that affect ventricular outflow, subarachnoid space function, or cerebral venous compliance. Treatment options include shunt and endoscopic approaches, which should be individualised to the child. The long-term outcome for children that have received treatment for hydrocephalus varies. Advances in brain imaging, technology, and understanding of the pathophysiology should ultimately lead to improved treatment of the disorder.
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              Effects and safety of periconceptional oral folate supplementation for preventing birth defects

              Cochrane Database of Systematic Reviews
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                Author and article information

                Contributors
                Role: Data curationRole: Formal analysisRole: InvestigationRole: MethodologyRole: Project administrationRole: ResourcesRole: SupervisionRole: ValidationRole: Writing – original draftRole: Writing – review & editing
                Role: ConceptualizationRole: Formal analysisRole: MethodologyRole: Project administrationRole: ResourcesRole: SupervisionRole: Writing – review & editing
                Role: Data curationRole: Formal analysisRole: InvestigationRole: MethodologyRole: ValidationRole: Writing – review & editing
                Role: Data curationRole: Formal analysisRole: InvestigationRole: MethodologyRole: ValidationRole: Writing – review & editing
                Role: ConceptualizationRole: Formal analysisRole: Funding acquisitionRole: InvestigationRole: MethodologyRole: ResourcesRole: Writing – review & editing
                Role: ConceptualizationRole: Formal analysisRole: Funding acquisitionRole: InvestigationRole: MethodologyRole: ResourcesRole: SupervisionRole: ValidationRole: Writing – review & editing
                Role: Data curationRole: Formal analysisRole: MethodologyRole: Project administrationRole: SupervisionRole: ValidationRole: Writing – review & editing
                Role: ConceptualizationRole: Formal analysisRole: Funding acquisitionRole: MethodologyRole: SupervisionRole: Writing – review & editing
                Role: Data curationRole: Formal analysisRole: InvestigationRole: MethodologyRole: Writing – review & editing
                Role: ConceptualizationRole: Data curationRole: Formal analysisRole: Funding acquisitionRole: InvestigationRole: MethodologyRole: Project administrationRole: SupervisionRole: Writing – review & editing
                Role: Editor
                Journal
                PLoS One
                PLoS ONE
                plos
                plosone
                PLoS ONE
                Public Library of Science (San Francisco, CA USA )
                1932-6203
                1 October 2018
                2018
                : 13
                : 10
                : e0204926
                Affiliations
                [1 ] Division of Neurosurgery, Department of Clinical Neuroscience, University of Calgary, Calgary, Alberta, Canada
                [2 ] Department of Neuroscience, Washington University School of Medicine, St. Louis, Missouri, United States of America
                [3 ] Department of Neurosurgery, Alberta Children’s Hospital, University of Calgary, Calgary, Alberta, Canada
                [4 ] Department of Orthopedic Surgery, New York University, New York, New York, United States of America
                [5 ] Division of Neurology, Department of Clinical Neuroscience, University of Calgary, Calgary, Alberta, Canada
                [6 ] Department of Neurology, Alberta Children’s Hospital, University of Calgary, Calgary, Alberta, Canada
                [7 ] Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, New York, United States of America
                [8 ] Clinical Research Unit, University of Calgary, Calgary, Alberta, Canada
                University of Mississippi Medical Center, UNITED STATES
                Author notes

                Competing Interests: The authors have declared that no competing interests exist.

                Author information
                http://orcid.org/0000-0001-8405-2454
                http://orcid.org/0000-0002-9348-503X
                Article
                PONE-D-18-18531
                10.1371/journal.pone.0204926
                6166961
                30273390
                777aa053-068b-40ec-9637-788fb022ad7a
                © 2018 Isaacs et al

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                : 21 June 2018
                : 17 September 2018
                Page count
                Figures: 7, Tables: 2, Pages: 24
                Funding
                Funded by: funder-id http://dx.doi.org/10.13039/100011094, Public Health Agency of Canada;
                Award Recipient :
                This study has been sponsored by the Public Health Agency of Canada. Funding was received by Drs. Mark Hamilton, Tamara Pringsheim and Nathalie Jette. However, the funder had no role in study design, data collection, data analysis, data interpretation, writing of the report, or decision to submit the findings for publication.
                Categories
                Research Article
                Medicine and Health Sciences
                Neurology
                Hydrocephalus
                Medicine and Health Sciences
                Pediatrics
                Computer and Information Sciences
                Data Visualization
                Infographics
                Charts
                People and Places
                Population Groupings
                Age Groups
                Elderly
                Medicine and Health Sciences
                Geriatrics
                Research and Analysis Methods
                Research Assessment
                Systematic Reviews
                Medicine and Health Sciences
                Congenital Disorders
                Birth Defects
                Neural Tube Defects
                Spina Bifida
                Biology and Life Sciences
                Developmental Biology
                Morphogenesis
                Birth Defects
                Neural Tube Defects
                Spina Bifida
                Medicine and Health Sciences
                Neurology
                Spinal Cord Diseases
                Spina Bifida
                Medicine and Health Sciences
                Surgical and Invasive Medical Procedures
                Custom metadata
                All relevant data are within the paper and its Supporting Information files.

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                Uncategorized

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