Evidence-Based Elements of Child Welfare In-Home Services

In this article we discuss evidence-based elements of effective in-home child welfare services as a foundation for advancing the evidence base for family-centered child welfare practice. In 2009 the U.S. Children’s Bureau established the National Resource Center for In-Home Services to build the capacity of state and tribal child welfare agencies to ensure the safety and well-being of children and youth in their homes, prevent their initial placement or re-entry into out-of-home care, and to support families in their role as primary caregivers. Through a nationwide assessment of in-home services conducted over four years of research and technical assistance, we developed a set of core elements for in-home services. These core elements are supported by empirical research and are congruent with evidence-based practices and programs. We review each of the elements with its underlying research base. We also discuss five evidence-supported in-home services interventions that share many of the elements. We conclude with a discussion of how evidence-based elements can be implemented to strengthen family centered child welfare practice

Guide for Automation of Low Volume Production

There is a remaining need from both academia and practitioners, to gain further knowledge about the decision making process for automation of low volume production. This paper includes insights of drivers for automation, the development of a guide for low volume production and the outcome of using the guide. The research in this study is based on both empirical data and theoretical considerations. The empirical data was collected in five case studies and a questionnaire. This paper is part of a research project with the main objective to develop knowledge about how flexible automation may contribute to improvements in efficiency, ergonomics, quality and production economics in different industries with low volume production. One of the results in the project was a comprehensive guide, developed, refined and improved in an iterative collaborative process, where tools and parts ofthe guide were tested and verified by five manufacturing case companies. The paper describes briefly the development process of the guide and content. The requirements of the guide derived from literature, case companies, questionnaire as well as industrial experts. The resulting guide can be used in several ways, depending on the requirements of the application. The guide includes guiding principles, a decision model for the analysis of the company, choice of automation and facts about automation. In the end of the project, four companies had invested or decided to invest in different types of automation

POLG2 deficiency causes adult-onset syndromic sensory neuropathy, ataxia and parkinsonism

Objective: Mitochondrial dysfunction plays a key role in the pathophysiology of neurodegenerative disorders such as ataxia and Parkinson's disease. We describe an extended Belgian pedigree where seven individuals presented with adult-onset cerebellar ataxia, axonal peripheral ataxic neuropathy, and tremor, in variable combination with parkinsonism, seizures, cognitive decline, and ophthalmoplegia. We sought to identify the underlying molecular etiology and characterize the mitochondrial pathophysiology of this neurological syndrome. Methods: Clinical, neurophysiological, and neuroradiological evaluations were conducted. Patient muscle and cultured fibroblasts underwent extensive analyses to assess mitochondrial function. Genetic studies including genome-wide sequencing were conducted. Results: Hallmarks of mitochondrial dysfunction were present in patients' tissues including ultrastructural anomalies of mitochondria, mosaic cytochrome c oxidase deficiency, and multiple mtDNA deletions. We identified a splice acceptor variant in POLG2, c.970-1G>C, segregating with disease in this family and associated with a concomitant decrease in levels of POLG2 protein in patient cells. Interpretation: This work extends the clinical spectrum of POLG2 deficiency to include an overwhelming, adult-onset neurological syndrome that includes cerebellar syndrome, peripheral neuropathy, tremor, and parkinsonism. We therefore suggest to include POLG2 sequencing in the evaluation of ataxia and sensory neuropathy in adults, especially when it is accompanied by tremor or parkinsonism with white matter disease. The demonstration that deletions of mtDNA resulting from autosomal-dominant POLG2 variant lead to a monogenic neurodegenerative multicomponent syndrome provides further evidence for a major role of mitochondrial dysfunction in the pathomechanism of nonsyndromic forms of the component neurodegenerative disorders

Genetic background may contribute to the latitude-dependent prevalence of dermatomyositis and anti-TIF1-γ autoantibodies in adult patients with myositis

Background: The prevalence of dermatomyositis (DM) versus DM and polymyositis (PM) combined has been shown to be negatively associated with latitude. This observation has been attributed to increasing exposure to ultraviolet (UV) light towards the equator. In this study, we investigated whether differing genetic background in populations could contribute to this distribution of DM. Methods: Case data derived from the MYOGEN (Myositis Genetics Consortium) Immunochip study (n = 1769) were used to model the association of DM prevalence and DM-specific autoantibodies with latitude. Control data (n = 9911) were used to model the relationship of human leucocyte antigen (HLA) associated with DM autoantibodies and DM or PM single-nucleotide polymorphisms (suggestive significance in the Immunochip project, P < 2.25 x 10(-5)) in healthy control subjects with latitude. All variables were analysed against latitude using ordered logistic regression, adjusted for sex. Results: The prevalence of DM, as a proportion of DM and PM combined, and the presence of anti-transcription intermediary factor 1 (anti-TIF1-gamma) autoantibodies were both significantly negatively associated with latitude (OR 0.96, 95% CI 0.95-0.98, P < 0.001; and OR 0.95, 95% CI 0.92-0.99, P = 0.004, respectively). HLA alleles significantly associated with anti-Mi-2 and anti-TIF1-gamma autoantibodies also were strongly negatively associated with latitude (OR 0.97, 95% CI 0.96-0.98, P < 0.001 and OR 0.98, 95% CI 0.97-0.99, P < 0.001, respectively). The frequency of five PM- or DM-associated SNPs showed a significant association with latitude (P < 0.05), and the direction of four of these associations was consistent with the latitude associations of the clinical phenotypes. Conclusions: These results lend some support to the hypothesis that genetic background, in addition to UV exposure, may contribute to the distribution of DM