Observations on recurrent vomiting with acetonuria in children

It would seem justifiable, therefore, to present the following clinical observations to demonstrate that:(1) The presence of ketosis is the essential feature of recurrent vomiting, the latter being secondary to a high concentration of acetone. This condition is invariably met with in a definite type of child who shows evidence of nervous hyperexcitability even from the first few weeks of life. (3) This physical type may be hereditarily transmitted.A discussion of the findings follows, and the relationship between ketonuria and the neuro-labile character is examined in an attempt to find an explanation for the occurrence of this condition

Bending moments in long spines

While it is unusual to test tank-models with controlled, variable elasticity there are several reasons why we believe that it is im-portant to exert the large effort required. 1. The convenient model-building materials are much too stiff to represent the proper behaviour of concrete and steel at full scale. 2. We believe that, although the close-packed, crest-spanning ter-minator configuration is the best possible arrangement for a wave-energy device, it is not economic to resist bending moments great-er than those which would occur at the power limit. This means that non-destructive yielding to bigger waves must be achieved. This will not only save money on the spine structure but will also produce dramatic reductions in mooring forces. 3. Work in the narrow tank had shown that the correct control of a duck mounting could produce large improvements in duck perfor-mance, doubling the efficiency in waves twenty-five duck diameters in length. We had also discovered that the best values of mounting stiffness were low, less than those that would be provided by post-tensioned concrete at full scale, and that the mounting move-ment could itself be a useful power-generating mechanism. Further-more the hardware needed to provide non-destructive yielding needed very little modification to provide intelligent control and the extra generating capability. We realised that the software require-ments would be formidable - well beyond our present knowledge. But we were confident that the existence of a controlled model would, as so often before, stimulate theoretical work. Furthermore we were quite certain that the progress of computer technology be-tween now and the date of the return of energy shortage would be so enormous that any level of control sophistication could be safe-ly assumed to be available at virtually zero cost. On many occa-sions it has seemed that this view was not shared by the civil and marine engineers who assess our progress. The work reported in this volume covers the measurements of bending moments and joint angles as a function of sea conditions, model lengths and stiffness, for circular spine sections without ducks. Other volumes will contain the observations of shear, axial, torsion and mooring forces, for spines with various appendages

The development, reliability, and validity of the Facilitator Assessment Tool: an implementation fidelity measure used in Parenting for Lifelong Health for Young Children

Background: The Parenting for Lifelong Health for Young Children (PLH-YC) programme aims to reduce violence against children and child behaviour problems among families in low- and middle-income countries (LMICs). Although the programme has been tested in four randomised controlled trials and delivered in over 25 countries, there are gaps in understanding regarding the programme's implementation fidelity and, more generally, concerning the implementation fidelity of parenting programmes in LMICs. Aims: This study aims to address these gaps by examining the psychometric properties of the PLH-YC-Facilitator Assessment Tool (FAT)—an observational tool used to measure the competent adherence of PLH-YC facilitators. Examining the psychometric properties of the FAT is important in order to determine whether there is an association between facilitator competent adherence and programme outcomes and, if correlated, to improve facilitator performance. It is also important to develop the implementation literature among parenting interventions in LMICs. Methods: The study examined the content validity, intra-rater reliability, and inter-rater reliability of the FAT. Revision of the tool was based on consultation with programme trainers, experts, and assessors. A training curriculum and assessment manual was created. Assessors were trained in Southeastern Europe and their assessments of facilitator delivery were analysed as part of a large-scale factorial experiment (N = 79 facilitators). Results: The content validity process with PLH-YC trainers, experts, and assessors resulted in substantial improvements to the tool. Analyses of percentage agreements and intraclass correlations found that, even with practical challenges, assessments were completed with adequate yet not strong intra- and inter-rater reliability. Conclusions: This study contributes to the literature on the implementation of parenting programmes in LMICs. The study found that the FAT appears to capture its intended constructs and can be used with an acceptable degree of consistency. Further research on the tool's reliability and validity—specifically, its internal consistency, construct validity, and predictive validity—is recommended

The Canadian Neuromuscular Disease Registry: Connecting Patients to National and International Research Opportunities

Introduction Patient registries serve an important role in rare disease research, particularly for the recruitment and planning of clinical trials. The Canadian Neuromuscular Disease Registry was established with the primary objective of improving the future for neuromuscular (NM) patients through the enablement and support of research into potential treatments. Methods In this report, we discuss design and utilization of the Canadian Neuromuscular Disease Registry with special reference to the paediatric cohort currently enrolled in the registry. Results As of July 25, 2017, there are 658 paediatric participants enrolled in the registry, 249 are dystrophinopathies (229 are Duchenne muscular dystrophy), 57 are myotonic dystrophy participants, 98 spinal muscular atrophy participants and 65 are limb girdle muscular dystrophy. A total of 175 patients have another NM diagnosis. The registry has facilitated 20 clinical trial inquiries, 5 mail-out survey studies and 5 other studies in the paediatric population. Discussion The strengths of the registry are discussed. The registry has proven to be an invaluable tool to NM disease research and has increased Canada’s visibility as a competitive location for the conduct of clinical trials for NM therapies

Modelling carbonaceous aerosol from residential solid fuel burning with different assumptions for emissions

Evidence is accumulating that emissions of primary particulate matter (PM) from residential wood and coal combustion in the UK may be underestimated and/or spatially misclassified. In this study, different assumptions for the spatial distribution and total emission of PM from solid fuel (wood and coal) burning in the UK were tested using an atmospheric chemical transport model. Modelled concentrations of the PM components were compared with measurements from aerosol mass spectrometers at four sites in central and Greater London (ClearfLo campaign, 2012), as well as with measurements from the UK black carbon network. The two main alternative emission scenarios modelled were Base4x and combRedist. For Base4x, officially reported PM2.5 from the residential and other non-industrial combustion source sector were increased by a factor of four. For the combRedist experiment, half of the baseline emissions from this same source were redistributed by residential population density to simulate the effect of allocating some emissions to the smoke control areas (that are assumed in the national inventory to have no emissions from this source). The Base4x scenario yielded better daily and hourly correlations with measurements than the combRedist scenario for year-long comparisons of the solid fuel organic aerosol (SFOA) component at the two London sites. However, the latter scenario better captured mean measured concentrations across all four sites. A third experiment, Redist – all emissions redistributed linearly to population density, is also presented as an indicator of the maximum concentrations an assumption like this could yield. The modelled elemental carbon (EC) concentrations derived from the combRedist experiments also compared well with seasonal average concentrations of black carbon observed across the network of UK sites. Together, the two model scenario simulations of SFOA and EC suggest both that residential solid fuel emissions may be higher than inventory estimates and that the spatial distribution of residential solid fuel burning emissions, particularly in smoke control areas, needs re-evaluation. The model results also suggest the assumed temporal profiles for residential emissions may require review to place greater emphasis on evening (including “discretionary”) solid fuel burning

The wide tank

Our aim was to construct a tank with complete control of directional distribution of energy as well as spectral shape. This control is important for several types of wave power device but it is of critical importance for the design of the backbone of a duck string