Fetal alcohol spectrum disorders: Prevalence rates in South Africa

Background. Fetal alcohol spectrum disorder (FASD) is an under-diagnosed condition in South Africa (SA). Fetal alcohol syndrome and FASD community prevalence studies were undertaken in 17 towns in three of the nine provinces in SA.Objective. The objective for all the studies was to determine the FASD prevalence rates by assessing the grade 1 learners in all the studies, using international FASD diagnostic criteria.Methods. The same methodology was used for all the studies in Gauteng, Western and Northern Cape provinces. Consenting grade 1 learners received anthropometric screening, clinical examinations and neurodevelopmental assessments. Structured interviews were used to assess maternal alcohol consumption during pregnancy.Results. Reported prevalence rates ranged from 29 to 290 per 1 000 live births.Conclusion. FASD rates from studies conducted in SA are among the highest worldwide. FASD affects all communities in SA and is therefore a major public health concern in SA. Multidisciplinary and intersectoral interventions are urgently required to raise awareness about the dangers of prenatal alcohol exposure and the devastating effect of FASD on the lives of children, families and communities

"Because of His Intellectual Disability, He Couldn't Cope." Is Euthanasia the Answer?

In 2018, the authors published their analysis of nine online case reports by the Euthanasia Review Committee in the Netherlands, involving people with intellectual disability and/or autism spectrum disorder who were given euthanasia. In this commentary, they reflect further on the challenges of assessing “unbearable suffering without prospect of improvement,” which is one of the Dutch legal due care criteria. Two more recent case reports are presented in detail, where doctors struggled to assess and sometimes came to divergent conclusions. In both cases, limitations resulting from the intellectual disability and autism spectrum disorder were seen by physicians as causes of unbearable suffering, leading them to agreeing to the patient's euthanasia request. The authors discuss their concern about the implications of accepting the effects of lifelong disability as reasons for euthanasia, not only for individuals but for society as a whole

Erratum to: Worldwide Prevalence of Fetal Alcohol Spectrum Disorders: A Systematic Literature Review Including Meta-Analysis

BACKGROUND: Although fetal alcohol spectrum disorders (FASD) affect communities worldwide, little is known about its prevalence. The objective of this study was to provide an overview of the global FASD prevalence. METHODS: We performed a search in multiple electronic bibliographic databases up to August 2015, supplemented with the ascendancy and descendancy approach. Studies were considered when published in English, included human participants, and reported empirical data on prevalence or incidence estimates of FASD. Raw prevalence estimates were transformed using the Freeman-Tukey double arcsine transformation so that the data followed an approximately normal distribution. Once the pooled prevalence estimates, 95% confidence intervals and prediction intervals were calculated based on multiple meta-analyses with transformed proportions using random effects models, these estimates were transformed back to regular prevalence rates. Heterogeneity was tested using Cochran's Q and described using the I(2) statistic. RESULTS: Among studies that estimated prevalence in general population samples, considerable differences in prevalence rates between countries were found and therefore separate meta-analyses for country were conducted. Particularly high-prevalence rates were observed in South Africa for fetal alcohol syndrome (55.42 per 1,000), for alcohol-related neurodevelopmental disorder (20.25 per 1,000), and FASD (113.22 per 1,000), For partial fetal alcohol syndrome high rates were found in Croatia (43.01 per 1,000), Italy (36.89 per 1,000), and South Africa (28.29 per 1,000). In the case of alcohol-related birth defects, a prevalence of 10.82 per 1,000 was found in Australia. However, studies into FASD exhibited substantial heterogeneity, which could only partly be explained by moderators, most notably geography and descent, in meta-regressions. In addition, the moderators were confounded, making conclusions as to each moderator's relevance tentative at best. CONCLUSIONS: The worldwide pooled prevalence estimates are higher than assumed so far, but this was largely explained by geography and descent. Furthermore, prevalence studies varied considerably in terms of used methodology and methodological quality. The pooled estimates must therefore be interpreted with caution and for future research it is highly recommended to report methodology in a more comprehensive way. Finally, clear guidelines on assessing FASD prevalence are urgently needed, and a first step toward these guidelines is presented

Do-Not-Attempt-Resuscitation orders for people with intellectual disabilities : dilemmas and uncertainties for ID physicians and trainees. The importance of the deliberation process

Item does not contain fulltextBACKGROUND: Not much is known about Do-Not-Attempt-Resuscitation (DNAR) decision-making for people with intellectual disabilities (IDs). The aim of this study was to clarify the problems and pitfalls of non-emergency DNAR decision-making for people with IDs, from the perspective of ID physicians. METHODS: This qualitative study was based on semi-structured individual interviews, focus group interviews and an expert meeting, all recorded digitally and transcribed verbatim. Forty ID physicians and trainees were interviewed about problems, pitfalls and dilemmas of DNAR decision-making for people with IDs in the Netherlands. Data were analysed using Grounded Theory procedures. RESULTS: The core category identified was 'Patient-related considerations when issuing DNAR orders'. Within this category, medical considerations were the main contributory factor for the ID physicians. Evaluation of quality of life was left to the relatives and was sometimes a cause of conflicts between physicians and relatives. The category of 'The decision-maker role' was as important as that of 'The decision procedure in an organisational context'. The procedure of issuing a non-emergency DNAR order and the embedding of this procedure in the health care organisation were important for the ID physicians. CONCLUSION: The theory we developed clarifies that DNAR decision-making for people with IDs is complex and causes uncertainties. This theory offers a sound basis for training courses for physicians to deal with uncertainties regarding DNAR decision-making, as well as a method for advance care planning. Health care organisations are strongly advised to implement a procedure regarding DNAR decision-making

Psychometric properties of the revised Developmental Behaviour Checklist scales in Dutch children with intellectual disability

The present study assessed the reliability and validity of the revised scales of the Developmental Behaviour Checklist (DBC) in a Dutch sample of children with intellectual disability (ID). The psychometric properties of the parent and teacher versions of the DBC were assessed in various subsamples derived from a sample of 1057 Dutch children (age range = 6-18 years) with ID or borderline intellectual functioning. Good test-retest reliability was shown both for the parent and teacher versions. Moderate inter-parent agreement and high one-year stability was found for the scale scores. Construct validity was satisfactory, although limited by high informant variance. The DBC scales showed good criterion-related validity, as indicated by significant mean differences between referred and non-referred children, and between children with and without a corresponding DSM-IV diagnosis. The reliability and validity of the revised DBC scales are satisfactory, and the checklist is recommended for clinical and research purposes

Effect of parental and ART treatment characteristics on perinatal outcomes

Funding This study was funded by Foreest Medical School, Alkmaar, the Netherlands (grants: FIO 1307 and FIO 1505). Acknowledgements We thank the Foundation of the Netherlands Perinatal Registry for permission to use their registry data (approval number 12.43). We thank G.P. Kroon and H.W.W. van Leeuwen for their assistance in collecting the necessary IVF data. Furthermore, we thank the medical informatics students A. Wong for the first deterministic data linkage and S. Wortel for assisting in the database validation process. In addition, we thank all care providers for the registration of the perinatal data as well as the IVF laboratory data.Peer reviewedPublisher PD

Uncommon genetic syndromes and narrative production - Case Studies with Williams, Smith-Magenis and Prader- Willi Syndromes

This study compares narrative production among three syndromes with genetic microdeletions: Williams syndrome (WS), Smith-Magenis syndrome (SMS), and Prader-Willi syndrome (PWS), characterized by intellectual disabilities and relatively spared language abilities. Our objective is to study the quality of narrative production in the context of a common intellectual disability. To elicit a narrative production, the task Frog! Where Are You was used. Then, structure, process, and content of the narrative process were analysed in the three genetic disorders:WS (n52), SMS (n52), and PWS (n52). Data show evidence of an overall low narrative quality in these syndromes, despite a high variability within different measures of narrative production. Results support the hypothesis that narrative is a highly complex cognitive process and that, in a context of intellectual disability, there is no evidence of particular ‘hypernarrativity’ in these syndromes.This research was supported by the grants FEDER –