Search

Copyright of Developmental Medicine & Child Neurology is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background: Partial urorectal septum malformation (pURSM) sequence (or 'persistent cloaca') is a rare congenital anomaly characterised by a joining of the urethral, anal, and genital openings into a single common channel. This study describes the epidemiology of pURSM sequence in England and Wales including prevalence, additional anomalies, and pregnancy outcomes. Methods: All cases of pURSM sequence prospectively notified to seven congenital anomaly registers in England and Wales during 1985-2010, whether delivered as live births, spontaneous fetal deaths (>20 weeks' gestation), or elective terminations of pregnancy for fetal anomaly (TOPFA, any gestation), formed this population-based cohort. The risks of spontaneous fetal and infant death were examined by Kaplan-Meier analysis. Differences in prevalence over time, and between regions, were examined by multilevel Poisson regression. Results: 117 cases were recorded among 4 251 241 total births. Six (5%) pregnancies resulted in spontaneous fetal deaths, 53 (45%) in TOPFA, and 58 (50%) in live births. The prevalence was 2.8 (95% CI 2.3 to 3.4) per 100 000 total births, increasing significantly over time (p=0.002) and differing significantly between regions (p=0.005). 77 cases (66%) had at least one additional major congenital anomaly outside the perineum, including 67 (57%) renal, 29 (25%) musculoskeletal, 26 (23%) digestive system, and 24 (21%) cardiovascular anomalies. The risks of spontaneous fetal and infant death were estimated as 8.9% (95% CI 4.1 to 18.8) and 26.3% (95% CI 15.1 to 43.4) respectively. Conclusions: This is the largest study of the epidemiology of pURSM sequence. The information will be valuable for families and health professionals whenever a case of pURSM sequence is diagnosed. [ABSTRACT FROM AUTHOR]

Objective To provide current population-based prevalence and prenatal diagnosis rates (PND) for specified major congenital anomalies in England and Wales to enable monitoring of the Fetal Anomaly Screening Programme (FASP). Design Secondary analysis of prospectively collected registry data. Setting Seven multiple-source, population-based congenital anomaly registers, members of the British Isles Network of Congenital Anomaly Registers (BINOCAR) in 2005 and 2006. Population 2,883 births with congenital anomalies from a total of 601,545 live and stillbirths. Main outcome measures PND and birth prevalence of selected congenital anomaly groups/ subtypes (anencephaly, spina-bifida, serious cardiac, diaphragmatic hernia, gastroschisis, exomphalos, bilateral renal agenesis, lethal/severe skeletal dysplasia, cleft lip with or without cleft palate [CL + /−P]). Results Of the selected anomaly groups, the most frequently reported were serious cardiac (14.1 per 10,000 births [95% CI 13.0−15.2]) and CL + /−P (9.7 per 10,000 births [8.9-10.5]); the least frequent were bilateral renal agenesis and lethal/severe skeletal dysplasia (<1.5 per 10,000 births). The PND varied for different anomalies from 53.1% (95% CI 43.5-65.2) for serious cardiac anomalies to 99.6% (95% CI 97.9-100.0) for anencephaly. Least variation in PND rates was for anencephaly (range 98.9-100%) and gastroschisis (93.5-100%); greatest variation was for serious cardiac (43.5-65.2%) and lethal/severe skeletal dysplasias (50.0-100%). Conclusions BINOCAR registers can, uniquely, provide contemporary data on PND and birth prevalence rates to enable monitoring of the ultrasound component of FASP at a national and regional level, allowing comparisons between populations to be made, planning of resources facilitated and assistance for parents making informed decisions on whether to enter the screening programme. [ABSTRACT FROM AUTHOR]

There is a growing interest in the history of childhood and child care. This article brings together from disparate sources some of the details and themes concerning the emergence and development of public and voluntary child welfare in Scotland. Particular attention is given to residential and foster care arrangements under the Poor Law and to the influence of ‘philanthropic’ individuals and organisations. Comparative references are made to the contemporary situation in England and Wales and to present day practice. [ABSTRACT FROM AUTHOR]

Objectives: The aim of this study was to assess the relationship between deciles of area-level deprivation and seven adverse pregnancy outcomes in Wales., Design: Cross-sectional analysis., Setting: 64 699 live births in Wales from 31 March 2014 to 16 September 2019., Primary Outcome Variable: We examined each of the following seven adverse pregnancy outcomes: (1) small for gestational age (SGA); (2) large for gestational age; (3) preterm birth; (4) third-degree or fourth-degree perineal tear; (5) major postpartum haemorrhage (MPPH); (6) a lower Apgar score at 5 min and (7) emergency caesarean section., Results: There was no significant association between increasing aggregate measures of area-level deprivation and the adverse pregnancy outcomes we studied. Women living in an area with greater access to services are more likely to have a baby that is SGA (1.27, 95% CI 1.11 to 1.49), have a greater likelihood of a perineal tear (1.74, 95% CI 1.15 to 2.61), are significantly less likely to have MPPH (0.79, 95% CI 0.64 to 0.96), have a baby with an Apgar score of 0.26 higher (95% CI 0.22 to 0.29) and are significantly less likely to have an emergency caesarean section (0.81, 95% CI 0.73 to 0.88). Women living in areas with higher employment (0.26, 95% CI 0.19 to 0.36) and better health (0.26, 95% CI 0.19 to 0.35) were less likely to experience perineal tear., Conclusions: There was no clear social-spatial gradient in area-level deprivation and adverse pregnancy outcomes. We found a stronger association for individual-level behavioural risk factors than area-level factors. These findings support the benefits that accessible and holistic person-centred care may bring through addressing individual behavioural risk factors. There is a need for improved data completeness and further individual-level data on risk factors such as employment and income to better understand the role which may be played by population-level policies and their pathways to affecting outcomes., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Exomphalos occurs in 2.2 per 10,000 births with 76% of these babies surviving to discharge. The aim of this study was to determine the birth prevalence and survival of babies with this anomaly in England and Wales., Methods: Six BINOCAR regional congenital anomaly registers in England and Wales (covering 36% of births) between 2005 and 2011 provided cases for this study. Cases included live births, stillbirths (24+ weeks' gestation), late miscarriages (20-23 weeks' gestation), and terminations of pregnancy with fetal anomaly., Results: The overall birth prevalence was 3.8 (95% confidence interval [CI]: 3.6-4.0) per 10,000 births; 1.4 (1.2-1.6) for isolated cases, 1.2 (1.1-1.4) for cases with multiple anomalies, and 1.2 (1.1-1.4) for cases with chromosomal anomalies. The live birth prevalence was 0.8 (0.7-0.9), 0.5 (0.4-0.6), and 0.1 (0.0-0.1) per 10,000 live births, respectively. Edwards syndrome, congenital heart defects, and nervous system anomalies were the most common anomalies associated with exomphalos. A prenatal diagnosis was made in 83% of isolated, 95% of multiple, and 99% of chromosomal cases. Fifty-five percent of isolated and multiple cases were live born, whereas 85% of cases with chromosomal anomalies resulted in a termination of pregnancy with fetal anomaly. The 1-year survival of live born babies with an isolated exomphalos was 92% compared with 81% in cases with multiple anomalies and 27% in cases with chromosomal anomalies (p < 0.001)., Conclusion: We report a higher birth prevalence than has previously been reported. The proportion of infants surviving with exomphalos remained unchanged over the time period., (© 2014 Wiley Periodicals, Inc.)