228 research outputs found
EU-Behörde: Warum wir sie brauchen
No full textJan Cremers (UvT) contributed to the magazine Gute Arbeit (in German), published by BUND-Verlag. Together with co-author Birgit Krämer, he discusses the plans of the European Commission to introduce a European Labour Authority. They reflect on the possible contribution of such a body in checking respect for and compliance with labour legislation and conventional standards
Nota, betreffende de toepassing van gewapend beton voor heipalen: opgemaakt door den ingenieur van den Rijskwaterstaat J.J. Canter Cremers
No full textOnderzoek naar problemen die onstaan door problemen bij het lassen van de wapening van heipalen. Ook blijkt het heiblok voor betonpalen anders te moeten worden uitgevoerd dan voor traditionele houten palen
The slowdown in German bank lending - revisited
No full textThe rate of growth in bank loans to private households and firms in Germany has declined substantially since early 2000 and currently stands at virtually zero. In this article, we analyse whether cyclical factors (demand-side driven) or banks unwillingness and/or inability to lend (supply-side driven) can be held responsible for this trend.Our preliminary results suggest that the slowdown in bank loan expansion is largely driven by a decline in the demand for loans. This result is supported by taking into account the latest tendency of corporates substituting bank loans for the issuance of money and capital market instruments. Although it cannot be ruled out that supply-side restrictions have contributed to the dampening of real bank loan expansion, to date these factors have played only a minor role. --German bank lending,Credit rationing
Genetic Etiology and Clinical Consequences of Cone Disorders
No full textHereditary retinal disorders constitute a large heterogeneous group of diseases in which
the photoreceptors are primarily aff ected. When cone cells are aff ected, one cannot see
details or perceive color. In this thesis, we focused on the three most important diseases
in which the cones are primarily aff ected: achromatopsia (ACHM), cone dystrophy (CD),
and cone-rod dystrophy (CRD). Although cone disorders account for only a small portion
of all retinal disorders, they have a great impact on daily life. This is mainly due to
the early onset of disease, severe visual outcome, and lack of therapeutic options. Cone
disorders can have an autosomal recessive, autosomal dominant or X-linked inheritance,
but unfortunately the genetic etiology is still largely unknown. This hampers clinical and
genetic counseling and the development of therapeutic options. This gap of knowledge
encouraged us to initiate a multicenter study with the aim to investigate the clinical
course of cone disorders and to unravel the genetic causes
Nota, betreffende de bepaling der grootheden, die bekend moeten zijn voor de berekening van den gronddruk tegen grondkerende constructies in de visschershaven te IJmuiden: opgemaakt door den ingenieur van den Rijkswaterstaat J.J. Canter Cremers
No full textBeschouwing over de actieve en passieve gronddruk voor de kademuur van de vissershaven van Ijmuiden. Het probleem was dat er geen standaardwaarden voor duinzand beschikbaar zijn. Prototype metingen over afschuiving van zand
Wrong from the start:The treatment of migrant labour in the EU
No full textThis working paper, based on a discourse by Jan Cremers at a major conference in Berlin on labour recruitment and exploitation, discusses forms of labour recruitment and hiring. The reasoning in political debates on the impact of cross-border mobility is that migrant labour decides to move elsewhere on a free choice and own initiative, with economic drivers such as work and (better) income. Based on research on the diversity of recruitment channels and on mechanisms of recruitment through migration and mobility by the recruitment industry, this can be partly questioned. User undertakings in destination countries drive the demand side of the recruitment market. Migrant workers are recruited as agency workers by, or on the instructions of temporary employment agencies from inside or outside the host country, based on a strong demand of potential user undertakings that developed a categorical preference for migrant labour based on issues of cost and obedience in certain sectors.The paper describes the occurrence and forms of abuses of labour hiring, underlying observations and experiences that demonstrate that a lot goes wrong from the start, and finally the dependency stemming from, among others, debt bondage. In the last paragraph, the author refers to recommendations about ‘ethical’ recruitment, stronger regulations of the access to the recruitment market, and a ban of private agencies in sensitive industries, as formulated by several global and EU labour market institutions.<br/
Wrong from the start:The treatment of migrant labour in the EU
No full textThis working paper, based on a discourse by Jan Cremers at a major conference in Berlin on labour recruitment and exploitation, discusses forms of labour recruitment and hiring. The reasoning in political debates on the impact of cross-border mobility is that migrant labour decides to move elsewhere on a free choice and own initiative, with economic drivers such as work and (better) income. Based on research on the diversity of recruitment channels and on mechanisms of recruitment through migration and mobility by the recruitment industry, this can be partly questioned. User undertakings in destination countries drive the demand side of the recruitment market. Migrant workers are recruited as agency workers by, or on the instructions of temporary employment agencies from inside or outside the host country, based on a strong demand of potential user undertakings that developed a categorical preference for migrant labour based on issues of cost and obedience in certain sectors.The paper describes the occurrence and forms of abuses of labour hiring, underlying observations and experiences that demonstrate that a lot goes wrong from the start, and finally the dependency stemming from, among others, debt bondage. In the last paragraph, the author refers to recommendations about ‘ethical’ recruitment, stronger regulations of the access to the recruitment market, and a ban of private agencies in sensitive industries, as formulated by several global and EU labour market institutions.<br/
Common ABCA4 mutations in South Africans: frequencies, pathogenicity and genotype-phenotype correlations
No full textStargardt disease (STGD), a juvenile-onset form of macular dystrophy resulting in a severe reduction of central vision, may be inherited in either an autosomal recessive or autosomal dominant manner. To date the only gene found to be involved with the autosomal recessive form is ABCA4. Mutations in this gene are associated not only with STGD, but with other autosomal recessive retinal diseases. Due to the numerous mutations detected in ABCA4 and their associated phenotypic heterogeneity, a genotype-phenotype model has been proposed based on the amount of ABCA4 protein activity. Research in the Division of Human Genetics at the University of Cape Town (UCT) has suggested possible ABCA4 founder mutations underlying STGD in the South African Caucasian Afrikaner population and has identified seven (C1490Y, R602W, IVS38-10T>C, L2027F, V256V, G863A, and R152X) common mutations. In a cohort of patients affected with an ABCA4-associated retinopathy (AAR) a total of 36% were identified as having various bi-allelic combinations of the seven mutations.In the current study, SNaPshot PCR, allele-specific PCR (AS-PCR) and denaturing high performance liquid chromatography (dHPLC) analysis were used to screen for the seven mutations in a patient cohort and a control cohort. A high detection rate of bi-allelic disease-causing mutations in total of 28/72 patients (i.e. 38.89% were fully characterised) confirmed the designed assay to be a viable screening tool, which could be employed in a diagnostic setting. The detection of 12 heterozygotes in the Caucasian control samples (n = 269; 169 of which were specifically Afrikaner) resulted in an estimated background frequency of 4.46 per 100 individuals. This could be used by counsellors to discuss carrier risks with patients and their family members. Bioinformatic tools (PolyPhen, SIFT, PMUT, PANTHER PSEC, ESEfinder, and the BDGP Splice Site Prediction programme) revealed the predicted pathogenicity of the seven mutations to be as follows (in order of decreasing pathogenicity): C1490Y, R602W, V256V, R152X, G863A, L2027F, and IVS38-10T>C. Statistical analysis (using the Kruskal-Wallis test and the Wilcoxon Rank Sum test) showed no significant12effect of mutation combination on phenotype (i.e. AOO/severity as a measure of clinical outcome).To improve the understanding of the genotype-phenotype correlation a larger cohort of South African STGD patients with the same common mutations in various combinations and the availability of sufficient clinical data, is required. Further investigations into the genotype-phenotype correlation, combined with the information on the pathogenicity of the mutations, could result in increased understanding regarding the impact of each mutation, thus enhancing the clinical utility of identifying ABCA4 mutations
Retinal gene therapy in patients with choroideremia: initial findings from a phase 1/2 clinical trial
No full textBackground: Choroideremia is an X-linked recessive disease that leads to blindness due to mutations in the CHM gene, which encodes the Rab escort protein 1 (REP1). We assessed the effects of retinal gene therapy with an adeno-associated viral (AAV) vector encoding REP1 (AAV.REP1) in patients with this disease.Methods: In a multicentre clinical trial, six male patients (aged 35-63 years) with choroideremia were administered AAV.REP1 (0·6-1·0×10(10) genome particles, subfoveal injection). Visual function tests included best corrected visual acuity, microperimetry, and retinal sensitivity tests for comparison of baseline values with 6 months after surgery. This study is registered with ClinicalTrials.gov, number NCT01461213.Findings: Despite undergoing retinal detachment, which normally reduces vision, two patients with advanced choroideremia who had low baseline best corrected visual acuity gained 21 letters and 11 letters (more than two and four lines of vision). Four other patients with near normal best corrected visual acuity at baseline recovered to within one to three letters. Mean gain in visual acuity overall was 3·8 letters (SE 4·1). Maximal sensitivity measured with dark-adapted microperimetry increased in the treated eyes from 23·0 dB (SE 1·1) at baseline to 25·3 dB (1·3) after treatment (increase 2·3 dB [95% CI 0·8-3·8]). In all patients, over the 6 months, the increase in retinal sensitivity in the treated eyes (mean 1·7 [SE 1·0]) was correlated with the vector dose administered per mm(2) of surviving retina (r=0·82, p=0·04). By contrast, small non-significant reductions (p>0·05) were noted in the control eyes in both maximal sensitivity (-0·8 dB [1·5]) and mean sensitivity (-1·6 dB [0·9]). One patient in whom the vector was not administered to the fovea re-established variable eccentric fixation that included the ectopic island of surviving retinal pigment epithelium that had been exposed to vector.Interpretation: The initial results of this retinal gene therapy trial are consistent with improved rod and cone function that overcome any negative effects of retinal detachment. These findings lend support to further assessment of gene therapy in the treatment of choroideremia and other diseases, such as age-related macular degeneration, for which intervention should ideally be applied before the onset of retinal thinning<br/
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