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Introduction: Cigarette affordability, the price of tobacco relative to consumer income, is a key determinant of tobacco consumption. This study examined trends over 12 years in individualised factory-made cigarette affordability in the Netherlands, and whether these trends differed by sex, age, and education.Methods: Data from 10 waves (2008-2020) of the International Tobacco Control (ITC) Netherlands Surveys were used to estimate individualised affordability, measured as the percentage of income required to buy 100 cigarette packs (Relative Income Price, RIP), using self-reported prices and income. The higher the RIP, the less affordable cigarettes are. Generalised estimating equation regression models assessed trends in individualised affordability over time and by sex, age, and education.Results: Affordability decreased significantly between 2008 and 2020, with RIP increasing from 1.89% (2008) to 2.64% (2020) (p≤.001), Except for 2008-2010, no significant year-on-year changes in affordability were found. Lower affordability was found among subgroups who have a lower income level: females (versus males), 18-24 and 25-39 year olds (versus 55 and over) and low or moderate educated individuals (versus high educated). Interactions between wave and education (p=.007) were found, but not with sex (p=.653) or age (p=.295). A decreasing linear trend in affordability was found for moderately (p=.041) and high-educated (p=.025), but not for low-educated individuals (p=.149).Conclusions: Cigarettes in the Netherlands have become less affordable between 2008 and 2020, yet this was mostly due to the decrease in affordability between 2008 and 2010. There is a need for more significant increases in tax to further decrease affordability.Implications: Our findings suggest that cigarettes have become less affordable in the Netherlands between 2008 and 2020. But, this appears to be the result of a steep decrease in affordability between 2008 and 2010. Affordability was lower among groups who have on average lower incomes (females, young adults, low- and moderate educated individuals), and differences in trends across education levels could be explained by per capita income changes. Our individualised measure indicated lower affordability than published aggregate affordability estimations. Future tax increases should be large enough to result in a lower affordability. [ABSTRACT FROM AUTHOR]

Ataxia telangiectasia (A-T) is a severe neurodegenerative disorder with variable immunodeficiency. Together with the Dutch A-T community, we investigated the opinion of A-T parents on an early A-T diagnosis in the asymptomatic phase of the disease. During an annual national meeting for A-T patients and families, the topic of an early A-T diagnosis was discussed in relation to the recent introduction of neonatal screening for severe combined immunodeficiency (SCID) in the Netherlands. Based on the discussion, individual arguments were identified and processed into a questionnaire, which was sent out to 64 A-T parents (32 families). Arguments included were insecurity to diagnosis, possible medical advantages, appropriate genetic counseling and family planning, loss of "golden" year(s), and early cancer screening for parents. The response rate was 55% (n = 35 parents). Twenty-six (74%) parents felt that the advantages of an early diagnosis outweighed the disadvantages, five parents thought that the disadvantages would outweigh the advantages (14%), and four parents did not indicate a preference.Conclusion: The majority of parents of a child with A-T would have preferred an early diagnosis during the asymptomatic phase of the disease, because the uncertainty during the diagnostic process had had a major impact on their lives. In addition, the knowledge of being carriers of an ATM gene mutation influenced decisions about family planning. Parents who opposed against an early diagnosis emphasized the joy of having a seemingly healthy child until diagnosis.What is Known:• Ataxia telangiectasia (A-T) is a devastating DNA repair disorder with a huge impact on quality of life of patients and their parents.• Patients with A-T may incidentally be identified at birth as the consequence of neonatal screening for severe combined immunodeficiency (SCID).What is New:• The majority of Dutch parents of A-T patients (74%) would have preferred an early diagnosis of their child in the asymptomatic phase of the disease.• Major arguments for an early A-T diagnosis were (1) the experienced insecurity in diagnostic trajectories and its impact on families and (2) the knowledge of being ATM mutation carriers when deciding about family planning. An argument against an early diagnosis is losing the joy of having a seemingly healthy child until diagnosis. [ABSTRACT FROM AUTHOR]

There is no heritage without emotional sharing and clashing. This article explores the involvement of divergent emotions in heritage making by discussing the debate series of Imagine IC and the Reinwardt Academy and zooming in on the commemoration of slavery and imagery of ‘Black Pete’ in the Netherlands. We introduce ‘emotion networking’ as a methodology to approach present-day heritage production, aiming for a novel approach to engage with ‘the collective’. [ABSTRACT FROM PUBLISHER]

Acquired demyelinating syndromes (ADS) can be a first presentation of multiple sclerosis (MS) in children. The incidence of these disorders in Europe is currently unknown. Children (<18 years old) living in the Netherlands who presented with ADS were included from January 1, 2007 to December 31, 2010 by the Dutch pediatric MS study group and the Dutch surveillance of rare pediatric disorders. Demographic and clinical data were collected. Eighty-six patients were identified over 4 years, resulting in an incidence of 0.66/1,00,000 per year. Most patients presented with polyfocal ADS without encephalopathy (30%), followed by polyfocal ADS with encephalopathy (24%), optic neuritis (ON, 22%), monofocal ADS (16%), transverse myelitis (3%), and neuromyelitis optica (3%). Patients with polyfocal ADS with encephalopathy were younger (median 3.9 years) than patients with ON (median 14.6 years, p < 0.001) or monofocal ADS (median 16.0 years, p < 0.001). Patients with polyfocal ADS without encephalopathy (median 9.2 years) were also younger than monofocal ADS patients (median 16.0 years, p < 0.001). There was a slight female preponderance in all groups except the ON group, and a relatively large number of ADS patients (29%) reported a non-European ancestry. Familial autoimmune diseases were reported in 23%, more often in patients with relapsing disease than monophasic disease (46 vs. 15%, p = 0.002) and occurring most often in the maternal family (84%, p < 0.001). During the study period, 23% of patients were subsequently diagnosed with MS. The annual incidence of ADS in the Netherlands is 0.66/1,00,000 children/year. A polyfocal disease onset of ADS was most common. [ABSTRACT FROM AUTHOR]

Background: Immunodeficiency in ataxia telangiectasia (A-T) is less severe in patients expressing some mutant or normal ATM kinase activity. We, therefore, determined whether expression of residual ATM kinase activity also protected against tumour development in A-T.Methods: From a total of 296 consecutive genetically confirmed A-T patients from the British Isles and the Netherlands, we identified 66 patients who developed a malignant tumour; 47 lymphoid tumours and 19 non-lymphoid tumours were diagnosed. We determined their ATM mutations, and whether cells from these patients expressed any ATM with residual ATM kinase activity.Results: In childhood, total absence of ATM kinase activity was associated, almost exclusively, with development of lymphoid tumours. There was an overwhelming preponderance of tumours in patients <16 years without kinase activity compared with those with some residual activity, consistent with a substantial protective effect of residual ATM kinase activity against tumour development in childhood. In addition, the presence of eight breast cancers in A-T patients, a 30-fold increased risk, establishes breast cancer as part of the A-T phenotype.Conclusion: Overall, a spectrum of tumour types is associated with A-T, consistent with involvement of ATM in different mechanisms of tumour formation. Tumour type was influenced by ATM allelic heterogeneity, residual ATM kinase activity and age. [ABSTRACT FROM AUTHOR]

Few smokers who try to quit smoking use smoking cessation treatment (SCT), and cost could be one factor. To increase the number of successful quitters, we assessed whether financial reimbursement for SCT would encourage the use of SCT and would as a result increase the 6-month point abstinence rate. We recruited smokers aged over 18 years from a random sample of Dutch inhabitants insured by one health insurance company. The smokers were assigned randomly to the intervention group ( n = 632) or control group ( n = 634). Respondents in the intervention group received an offer of reimbursement for nicotine replacement therapy, bupropion and behavioural counselling. No reimbursement was offered to the control group. To preclude a change of behaviour due to disappointment in the control group, we used a randomized consent design. During the reimbursement period, 10.8% smokers in the intervention group reported having used SCT compared with 4.1% in the control group (OR = 2.9, 95% CI 1.8–4.7). In the intervention group, 23.4% smokers tried to stop compared with 20.8% in the control group (OR = 1.2, 95% CI 0.9–2.4). After 6 months, the biochemically validated 7-day point prevalence abstinence rate was 5.5% in the intervention group and 2.8% in the control group (OR = 2.3, 95% CI 1.2–4.1). Reimbursement for SCT seems efficacious in increasing the use of SCT and may double the number of successful quitters. [ABSTRACT FROM AUTHOR]

Objectives: Female-specific issues, such as breast injuries, pelvic floor dysfunctions, saddle sores, and menstrual symptoms, can significantly impact female athletes. This study examined the prevalence, perceived impact on performance, and role of the support staff in addressing and managing these issues in elite athletes based at a multisport training centre., Design: Anonymous online questionnaire., Methods: A total of 180 female athletes from various sport disciplines at the Dutch Olympic Training Centre were invited to participate in the study. An anonymous, 39-item questionnaire was developed and hosted on Qualtrics collecting data on respondents' characteristics, breast injuries, pelvic floor dysfunctions, saddle sores, menstrual symptoms, contraceptive use, and communication about these issues., Results: A total of 105 valid responses were analysed. The findings revealed that athletes regularly experience breast injuries (15.2 %), urinary incontinence (29.5 %), other pelvic floor problems (21.0 %), saddle sores (65.7 %), and menstrual cycle-related symptoms, with negative performance effects. Communication with support staff remained limited, with only 23.8 % of athletes discussing any of these issues, often due to concerns about understanding, shame, or a lack of opportunity for easy communication., Conclusions: The findings underscore the crucial role of creating a supportive environment, promoting early intervention, and utilising multisport centres to comprehensively address these concerns, emphasising the need for open communication, education, and support to enhance female athletes' performance and well-being., Competing Interests: Declaration of interest statement The authors have no competing interest to declare., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Invasive group A streptococcal (iGAS) disease cases increased in the first half of 2022 in the Netherlands, with a remarkably high proportion of emm 4 isolates. Whole-genome sequence analysis of 66 emm 4 isolates, 40 isolates from the pre-coronavirus disease 2019 (COVID-19) pandemic period 2009-2019 and 26 contemporary isolates from 2022, identified a novel Streptococcus pyogenes lineage (M4 NL22 ), which accounted for 85 % of emm 4 iGAS cases in 2022. Surprisingly, we detected few isolates of the emm 4 hypervirulent clone, which has replaced nearly all other emm 4 in the USA and the UK. M4 NL22 displayed genetic differences compared to other emm 4 strains, although these were of unclear biological significance. In publicly available data, we identified a single Norwegian isolate belonging to M4 NL22 , which was sampled after the isolates from this study, possibly suggesting export of M4 NL22 to Norway. In conclusion, our study identified a novel S. pyogenes emm 4 lineage underlying an increase of iGAS disease in early 2022 in the Netherlands and the results have been promptly communicated to public health officials.

Background: The EUREST-PLUS ITC Europe surveys aim to evaluate the impact of the European Union's Tobacco Products Directive (EU TPD) implementation within the context of the WHO FCTC. This article describes the methodology of the 2016 (Wave 1) and 2018 (Wave 2) International Tobacco Control 6 European (6E) Country Survey in Germany, Greece, Hungary, Poland, Romania and Spain; the England arm of the 2016 (Wave 1) and 2018 (Wave 2) ITC 4 Country Smoking and Vaping (4CV) Survey; and the 2016 (Wave 10) and 2017 (Wave 11) ITC Netherlands (NL) Survey. All three ITC surveys covering a total of eight countries are prospective cohort studies with nationally representative samples of smokers., Methods: In the three surveys across the eight countries, the recruited respondents were cigarette smokers who smoked at least monthly, and were aged 18 and older. At each survey wave, eligible cohort members from the previous waves were retained, regardless of smoking status, and dropouts were replaced by a replenishment sample., Results: Retention rates between the two waves of the ITC 6E Survey by country were 70.5% for Germany, 41.3% for Greece, 35.7% for Hungary, 45.6% for Poland, 54.4% for Romania and 71.3% for Spain. The retention rate for England between ITC 4CV1 and ITC 4CV2 was 39.1%; the retention rates for the ITC Netherlands Survey were 76.6% at Wave 10 (2016) and 80.9% at Wave 11 (2017)., Conclusion: The ITC sampling design and data collection methods in these three ITC surveys allow analyses to examine prospectively the impact of policy environment changes on the use of cigarettes and other tobacco products in each country, to make comparisons across the eight countries., (© The Author(s) 2020. Published by Oxford University Press on behalf of the European Public Health Association. All rights reserved.)

Background: We examined quit attempts, use of cessation assistance, quitting beliefs and intentions among smokers who participated in the 2018 International Tobacco Control (ITC) Europe Surveys in eight European Union Member States (England, Germany, Greece, Hungary, the Netherlands, Poland, Romania and Spain)., Methods: Cross-sectional data from 11 543 smokers were collected from Wave 2 of the ITC Six European Country (6E) Survey (Germany, Greece, Hungary, Poland, Romania and Spain-2018), the ITC Netherlands Survey (the Netherlands-late 2017) and the Four Countries Smoking and Vaping (4CV1) Survey (England-2018). Logistic regression was used to examine associations between smokers' characteristics and recent quit attempts., Results: Quit attempts in the past 12 months were more frequently reported by respondents in the Netherlands (33.0%) and England (29.3%) and least frequently in Hungary (11.5%), Greece (14.7%), Poland (16.7%) and Germany (16.7%). With the exception of England (35.9%), the majority (56-84%) of recent quit attempts was unaided. Making a quit attempt was associated with younger age, higher education and income, having a smoking-related illness and living in England. In all countries, the majority of continuing smokers did not intend to quit in the next 6 months, had moderate to high levels of nicotine dependence and perceived quitting to be difficult., Conclusions: Apart from England and the Netherlands, smokers made few quit attempts in the past year and had low intentions to quit in the near future. The use of cessation assistance was sub-optimal. There is a need to examine approaches to supporting quitting among the significant proportion of tobacco users in Europe and increase the use of cessation support as part of quit attempts., (© The Author(s) 2020. Published by Oxford University Press on behalf of the European Public Health Association. All rights reserved.)

Background: Patients experiencing severe mental illnesses (SMI) need continuing support and remain vulnerable in many domains. Crisis interventions and compulsory admissions are common, causing a huge burden on police, health workers, the community and patients. The aim of this retrospective case-file study is to determine profiles of SMI-patients and their pathways through care among those experiencing multiple public crisis events., Methods: Data from a larger study of 323 SMI-patients in Amsterdam were used. These data were linked to data of the public mental health care (PMHC) in order to identify persons that experienced crisis interventions (CI's) between January 2004 and November 2012. The cut-off point for inclusion in the study population was set on three CI's, resulting in a group of 47 SMI-patients. PMHC and mental health care (MHC) data were linked in order to identify profiles in patterns of care. Qualitative content analysis was used to gather and analyze chronological timelines., Results: Three profiles were identified: SMI-patients with CI's during continuous MHC, SMI-patients with CI's after discharge and SMI-patients with CI's during unstable MHC. For each profile events prior to, during and after a CI were identified., Conclusions: PMHC and MHC can possibly identify cases with a high risk of CI's and predict these events based on the results of this study. CI's seem inevitable for a group of SMI-patients in care but they do not only require acute psychiatric care. The collaboration between MHC, PMHC and police could be further developed in a quick and effective triage in order to tackle the complexity of problems of the SMI-patients.

Until recently, the cause of intellectual disability (ID) remained unexplained in at least 50% of affected individuals. Recent advances in genetic technologies led to great new opportunities to elucidate genetic defects implicated in ID. The introduction of genome-wide technologies that are able to detect small chromosomal copy number variations led to the identification of several microdeletion/duplication syndromes and to the subsequent identification of single causative genes. By the recent implementation of whole exome sequencing (WES) in research and diagnostics, with the potential to identify disease causing variants throughout the human exome at the base-pair level, a new revolution has started. Several studies showed that WES is effective in the identification of ID genes. Here we provide an historical overview of the advances in diagnostics of ID and illustrate the high diagnostic potential of current technologies by presenting the diagnostic survey that we performed in a series of 253 individuals with previously unexplained ID. This is the first study that systematically evaluated the diagnostic yield of the currently available and rapidly developing genetic diagnostic arsenal. The results of our study indicate that application of present-day genetic diagnostic technologies lead to a significant increase in the number of patients that can be diagnosed., (© 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Background: In the clinically and genetically heterogeneous group of the hereditary spastic paraplegias (HSPs), mutations in the SPAST gene are most frequently found and cause a pure autosomal dominant form., Objective: To provide the clinical and genetic characteristics of Dutch patients with HSP due to a SPAST mutation (SPG4)., Methods: SPAST mutation carriers were identified through a comprehensive national database search. Available medical records were reviewed., Results: 151 mutation carriers carried 60 different changes in the SPAST gene, of which one was a known polymorphism, and 27 were novel. Missense mutations were most frequently found (39%). Clinical information was available from 72 mutation carriers. Age at onset ranged from 1 to 63 years with a bimodal peak distribution in the first decade and above age 30. The predominantly pure spastic paraplegia was accompanied by deep sensory disturbances and sphincter problems in almost 50%. An additional hand tremor was found in 10%. Patients with missense mutations and exon deletions did not reveal a distinctive phenotype., Conclusions: Dutch SPAST mutation carriers show a broad mutation spectrum, with 27 novel mutations in the present series. A bimodal peak distribution in age at onset was found and an accompanying tremor as peculiar feature of SPG4. The pathogenicity of S44L, the first exon 4 mutation, and a possible autosomal recessive mode of inheritance are discussed.

Background: Epidermolytic ichthyosis (EI), previously termed bullous congenital ichthyosiform erythroderma or epidermolytic hyperkeratosis, is a clinically heterogeneous genodermatosis caused by mutations in the genes encoding the suprabasal keratins 1 and 10. Classical EI is clinically characterized by severe neonatal erythroderma, blistering and fragile skin in infancy, quickly subsiding with subsequent development of generalized scaling hyperkeratosis. We report three Dutch families with palmoplantar keratoderma and mild blistering, but without neonatal erythroderma and generalized scaling. A novel heterozygous missense mutation in the linker L12 domain of KRT1:c.1019A>G, p.Asp340Gly was found associated with this phenotype in these families., Objectives: To investigate the effects of the novel KRT1:p.Asp340Gly and the one other previously reported KRT1:p.Asp340Val mutations on keratinocyte cytoskeleton formation and stress resistance., Methods: Wild-type and mutant pEGFP-KRT1 fusion constructs were transfected into HaCaT cells and exposed to hypo-osmotic shock. Haplotyping and genealogical studies were performed to investigate the possibility of a common founder for p.Asp340Gly., Results: Cells transfected with either one of the keratin 1 L12 domain mutations showed significantly increased tonofilament aggregation. The haplotype around the KRT1 gene was shared in all affected family members of two families and a common founder was traced., Conclusions: Our study supports the pathogenicity of the keratin 1 L12 domain mutations in vitro. These mutations are associated with a milder EI phenotype with pronounced palmoplantar keratoderma, and without neonatal erythroderma and scaling. The KRT1:p.Asp340Gly mutation in the Dutch families is likely to have arisen from a common founder.

Adoption of potentially effective preventive interventions often fails. This study aimed to identify factors that determine why midwifery practices decide to use a smoking cessation protocol, using a comprehensive model of both organizational and psychosocial factors. A cross-sectional survey was conducted among representatives of all 446 Dutch midwifery practices, of whom 251 (56%) responded. The results show that adoption of the protocol was facilitated by the presence of practice assistants and impeded by a large proportion of clients of foreign ethnic origin. The most successful information channel was the midwives' professional association. A consistent positive attitude (perceiving a lot of advantages, few disadvantages and a low level of ambivalence) and positive social norms toward using the protocol, a perceived large proportion of midwives who use the protocol and knowledge about the protocol significantly increased the likelihood of adoption. The decision to use the protocol was better explained by personal awareness and motivation factors than by organizational factors.

This study assessed the antecedents of continued use of an education program to prevent passive smoking in infants. It consists of a booklet for parents and a manual for health professionals describing a five-step procedure for discussing passive smoking. A questionnaire was sent to 67 managers, 670 nurses, and 335 physicians working in well-baby clinics (response rate: 70%, 53%, 47% respectively). Questions concerned the completeness of use, level of institutionalization, and characteristics of the organization, the user, and the dissemination strategy. Seventy-one percent of nurses and 42% of physicians worked with the program. They foremost provided the first three steps of the five-step procedure. Physicians' completeness of use was related to their perceived responsibility in providing this education, and nurses' use was related to their perceived self-efficacy, responsibility, training attendance, participation in the adoption decision, and level of institutionalization. Diffusion efforts should focus on improving the completeness of use and level of institutionalization.

Parents play an important role in the development of young people's smoking behavior, through the modeling effects of their own smoking status, through the ways they raise their children and through the ways they deal with smoking at home. The present study focused on anti-smoking socialization by, first, comparing the perspectives of both parents and an adolescent on eight indicators of anti-smoking socialization. In addition, we aimed to examine how these indicators of anti-smoking socialization are related to adolescent smoking-related cognitions (e.g. attitudes, self-efficacy, intentions to smoke). Data were collected from 116 Dutch families with fathers, mothers and adolescents (10-19 years old) included. Self-reports of these family members were used by means of questionnaires that were sent through the Internet. The findings showed that parents and adolescents differ in their reports on anti-smoking socialization. In general, mothers are more positive about anti-smoking socialization than adolescents and fathers. Furthermore, the results demonstrate that aspects of anti-smoking socialization, such as parental monitoring, norms on adolescents smoking and reactions on adolescent smoking, are related to smoking-related cognitions, such as negative attitudes to smoking, lower intentions to start smoking and higher self-efficacy. These findings are a first step in research on smoking-specific socialization that is considered to be of importance for the development of effective smoking prevention programmes focusing on parents. Nonetheless, longitudinal studies on large samples of families are needed to replicate the findings in this study.

Objective: To measure environmental tobacco smoke (ETS) exposure in psychiatric settings and to assess determinants of support for complete smoking bans., Design: Cross sectional study, Setting: Dutch psychiatric hospitals, outpatient care institutions, and sheltered home facilities., Subjects: A random sample of 540 treatment staff, 306 attendants/nurses, and 93 patients., Main Outcome Measures: Self reported ETS exposure, current smoking policy, compliance with smoking policy, beliefs about smoking bans., Results: 87% of respondents were exposed to tobacco smoke in psychiatric institutions; 29% said that on an average day they were exposed to "a lot of smoke". Although ETS originates mainly from smoking patients, both non-compliance from patients and employees with existing bans resulted in non-smokers being exposed to ETS. Due to non-compliance, ETS exposure was quite high when there is a general smoking ban (designated areas option). Only with a complete ban was compliance good and employees sufficiently protected from ETS exposure. Psychiatrists, psychologists, physicians, attendants, and nurses were most concerned about resistance from patients, partly because of the fear of infringing on patients' freedom to smoke., Conclusions: Complete smoking bans are the only way to fully protect those working in psychiatry from ETS exposure, mainly because general smoking bans are not sufficiently complied with. Communication strategies to improve compliance with complete bans are crucial to protect those working in psychiatry from ETS. Compliance could be improved by addressing the belief that the ban will effectively result in less ETS exposure and the issue of patients' freedom to smoke versus employees' right to work in a smoke-free environment.

Background: Passive smoking is harmful to young children. A protocol has been developed to allow health care workers to communicate with parents about preventing passive smoking. The main message was to refrain from smoking in the presence of the child. The aim of the study was to assess the effectiveness of this education programme., Method: The prevalence of smoking in the presence of infants aged 0-10 months was compared before and after the implementation of the education programme. National samples of mothers completed questionnaires in 1996 (n = 1,129) and in 1999 (n = 2,534). Questions were asked about smoking in the living room in the presence of infants, and about parental smoking, and background characteristics., Results: The prevalence of passive infant smoking decreased from 41% to 18%. The adjusted odds ratio for passive infant smoking in 1999 compared to 1996 was 0.34 (0.26-0.44) when none of the parents smoked, 0.19 (0.14-0.27) when one of the parents smoked, and 0.30 (0.20-0.44) when both parents smoked., Conclusion: The implementation of this health education programme seems to have been very successful in reducing passive smoking in children. Implementation of similar health education programmes in other countries is recommended.

Sjögren-Larsson syndrome (SLS) is an autosomal recessively inherited neurocutaneous disorder caused by a deficiency of the microsomal enzyme fatty aldehyde dehydrogenase (FALDH). We report the clinical characteristics and the results of molecular studies in 19 SLS patients. Patients 1-17 show the classical triad of severe clinical abnormalities including ichthyosis, mental retardation and spasticity. Most patients were born preterm, and all patients exhibit ocular abnormalities and pruritus. Electro-encephalography shows a slow background activity, without other abnormalities. MRI of the brain shows an arrest of myelination, periventricular signal abnormalities of white matter and mild ventricular enlargement. Cerebral (1)H-MR spectroscopy reveals a characteristic, abnormal lipid peak. The degree of white matter abnormality in the MRIs and the height of the lipid peak in (1)H-MR spectra do not correlate with the severity of the neurological signs. The clinical presentation and the clinical course is strikingly similar in these patients. Patient 18 shows a mild phenotype that essentially contains the same, but less severe, clinical features. Patient 19 exhibits the typical, but very mild, dermatological and ocular abnormalities, without any clinical neurological involvement. The diagnosis of SLS was confirmed by demonstration of the enzyme defect in cultured skin fibroblasts. Furthermore, as might be predicted from the essential role of FALDH in leucotriene B(4) (LTB(4)) metabolism, elevated urinary concentrations of LTB(4) and 20-OH-LTB(4) were found in all patients studied. Molecular studies of the FALDH gene revealed eight different mutations, including three new ones: a large 26-base pair deletion (21-46del), a missense mutation (80C-->T) and an insertion mutation (487-488insA). The vast majority of SLS patients seem to be severely affected independent of their genotype.

This article reports on one of the few experimental studies in Europe to examine work site smoking cessation. The study examined whether a comprehensive intervention (self-help manuals, group courses, a mass media campaign, smoking policies, and a second-year program) is more effective than a minimal intervention (self-help manuals only). Eight work sites participated in the study. The effect of treatment on smoking cessation depended on nicotine dependency levels: Heavy smokers had more success with the comprehensive smoking cessation intervention than with the minimal intervention (with respect to both 14-month quit rate and 6-month prolonged abstinence). For heavy smokers, exposure to mass media exhibitions or to group courses had a beneficial effect on prolonged abstinence. Comprehensive programs may be most appropriate in Dutch work sites with large proportions of heavily addicted smokers.

Environmental tobacco smoke (ETS) is an occupational carcinogen. Large companies often examine ETS exposure by employee surveys. However, reliable and valid self-report measures have been lacking. This study compared validity and reliability of various self-report measures. One hundred and seven nonsmokers from 11 Dutch worksites were interviewed. Three self-report measures were correlated with nicotine concentrations collected with area monitors in nonsmokers' offices. Nicotine concentrations averaged 12.7 micrograms/m. The item "How much tobacco smoke, on average, is there during a day in your work office?" correlated highest with nicotine concentrations (r = 0.65; P < 0.001) and produced the lowest standard error of measurement. It was concluded that this simple self-report item may be a valid measure to assess the reach and effectiveness of worksite smoking policies.

Background: Nonsmokers' assertiveness can help regulate smoking in worksites by enhancing the salience of nonsmoking social norms. This study examined determinants of employees' assertiveness toward smoking colleagues., Methods: Cross-sectional data were collected from 898 nonsmoking Dutch employees. Potential determinants were chosen using the attitude-social influence-efficacy model., Results: Fifty-one percent of nonsmoking employees asked co-workers not to smoke. Assertive respondents had colleagues who more often acted assertively toward smokers at work, had a more positive attitude to asking colleagues not to smoke, and had a higher perceived self-efficacy. Moreover, assertive employees more often perceived hindrance from environmental tobacco smoke (ETS) and had more negative beliefs about ETS at work., Conclusions: What matters is whether ETS is perceived as bothersome and harmful. It is likely that both conditions must be met for nonsmokers to behave assertively. Worksite educational programs could focus more on increasing nonsmokers' awareness of the harmfulness of regular exposure to ETS at work to increase social pressure on employees who smoke in places that are shared by smokers and nonsmokers. However, the effectiveness of such strategies is yet unknown.

Background: This study examines the influence of the social environment in worksites on employees' intention to quit smoking by applying the attitude-social influence-efficacy model., Methods: Regression analyses were conducted with 509 smoking employees from eight Dutch worksites. Variables of interest were attitudes toward smoking, perceived social pressure to quit, perceived smoking behavior of others, self-efficacy, demographics, smoking history variables, company type, and smoking tolerance., Results: Attitude, social pressure from people out-side the worksetting (partner, children), and self-efficacy explained 23% of the variance for intention. Past behavior increased the adjusted R2 from 0.23 to 0.29. Social influences in the worksite were unrelated to employees' intention to quit smoking., Conclusions: It was concluded that, in Dutch work-sites, social influences stemming from people at work have less influence on employees' intention to quit smoking than other important people outside the work situation. Implications for smoking control strategies in worksites are briefly discussed.