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Long-Term outcome of infantile onset pompe disease patients treated with enzyme replacement therapy
(2024)
Background: Enzyme replacement therapy (ERT) with recombinant human alglucosidase alfa (rhGAA) was approved in Europe in 2006. Nevertheless, data on the long-term outcome of infantile onset Pompe disease (IOPD) patients at school age is still limited.
Objective: We analyzed in detail cardiac, respiratory, motor, and cognitive function of 15 German-speaking patients aged 7 and older who started ERT at a median age of 5 months.
Results: Starting dose was 20 mg/kg biweekly in 12 patients, 20 mg/kg weekly in 2, and 40 mg/kg weekly in one patient. CRIM-status was positive in 13 patients (86.7%) and negative or unknown in one patient each (6.7%). Three patients (20%) received immunomodulation. Median age at last assessment was 9.1 (7.0–19.5) years. At last follow-up 1 patient (6.7%) had mild cardiac hypertrophy, 6 (42.9%) had cardiac arrhythmias, and 7 (46.7%) required assisted ventilation. Seven patients (46.7%) achieved the ability to walk independently and 5 (33.3%) were still ambulatory at last follow-up. Six patients (40%) were able to sit without support, while the remaining 4 (26.7%) were tetraplegic. Eleven patients underwent cognitive testing (Culture Fair Intelligence Test), while 4 were unable to meet the requirements for cognitive testing. Intelligence quotients (IQs) ranged from normal (IQ 117, 102, 96, 94) in 4 patients (36.4%) to mild developmental delay (IQ 81) in one patient (9.1%) to intellectual disability (IQ 69, 63, 61, 3x < 55) in 6 patients (54.5%). White matter abnormalities were present in 10 out of 12 cerebral MRIs from 7 patients.
Measuring what matters
(2023)
Patient reported outcomes (PROs) are generally defined as ‘any report of the status of a patient's health condition that comes directly from the patient, without interpretation of the patient's response by a clinician or anyone else’. A broader definition of PRO also includes ‘any information on the outcomes of health care obtained directly from patients without modification by clinicians or other health care professionals’. Following this approach, PROs encompass subjective perceptions of patients on how they function or feel not only in relation to a health condition but also to its treatment as well as concepts such as health-related quality of life (HrQoL), information on the functional status of a patient, signs and symptoms and symptom burden. PRO measurement instruments (PROMs) are mostly questionnaires and inform about what patients can do and how they feel. PROs and PROMs have not yet found unconditional acceptance and wide use in the field of inborn errors of metabolism. This review summarises the importance and usefulness of PROs in research, drug legislation and clinical care and informs about quality standards, development, and potential methodological shortfalls of PROMs. Inclusion of PROs measured with high-quality, well-selected PROMs into clinical care, drug legislation, and research helps to identify unmet needs, improve quality of care, and define outcomes that are meaningful to patients. The field of IEM should open to new methodological approaches such as the definition of core sets of variables including PROs to be systematically assessed in specific metabolic conditions and new collaborations with PRO experts, such as psychologists to facilitate the systematic collection of meaningful data.
Why do some countries assign a major role to wind energy in decarbonizing their electricity systems, while others are much less committed to this technology? We argue that processes of (de-)legitimation, driven by discourse coalitions who strategically employ certain storylines in public debates, provide part of the answer. To illustrate our approach, we comparatively investigate public discourses surrounding wind energy in Austria and Switzerland, two countries that differ strongly in wind energy deployment. By combining a qualitative content analysis and a discourse network analysis of 808 newspaper articles published 2010–2020, we identify four distinct sets of storylines used to either delegitimize or legitimize the technology. Our study indicates that low deployment rates in Switzerland can be related to the prominence of delegitimizing storylines in the public discourse, which result in a rather low socio-political acceptance of wind energy. In Austria, by contrast, there is more consistent support for wind energy by discourse coalitions using a broad set of legitimizing storylines. By bridging the related but separate literatures of technology legitimacy and social acceptance, our study contributes to a better understanding of socio-political conflict and divergence in low-carbon technological pathways.
A step change is needed in the deployment of renewable energy if the triple challenge of ensuring climate change mitigation, energy security, and energy affordability is to be met. Yet, social acceptance of infrastructure projects and policies remains a key concern. While there has been decades of fruitful research on the social acceptance of wind energy and other renewables, much of the extant research is cross-sectional in nature, failing to capture the important dynamic processes that can make or break renewable energy projects. This paper introduces a Special Issue of Energy Policy which focuses on the neglected topic of the dynamics of social acceptance of renewable energy, drawing on contributions made at an international research conference held in St. Gallen (Switzerland) in June 2022. In addition to introducing these papers and drawing out common themes, we also seek to offer some conceptual clarity on the issue of dynamics in social acceptance, taking into account the influence of time, power, and scale in shaping decision-making processes. We conclude by highlighting a number of avenues of potential future research.
X-ray microtomography is a nondestructive, three-dimensional inspection technique applied across a vast range of fields and disciplines, ranging from research to industrial, encompassing engineering, biology, and medical research. Phasecontrast imaging extends the domain of application of x-ray microtomography to classes of samples that exhibit weak attenuation, thus appearing with poor contrast in standard x-ray imaging. Notable examples are low-atomic-number materials, like carbon-fiber composites, soft matter, and biological soft tissues.We report on a compact and cost-effective system for x-ray phase-contrast microtomography. The system features high sensitivity to phase gradients and high resolution, requires a low-power sealed x-ray tube, a single optical element, and fits in a small footprint. It is compatible with standard x-ray detector technologies: in our experiments, we have observed that single-photon counting offered higher angular sensitivity, whereas flat panels provided a larger field of view. The system is benchmarked against knownmaterial phantoms, and its potential for soft-tissue three-dimensional imaging is demonstrated on small-animal organs: a piglet esophagus and a rat heart.We believe that the simplicity of the setupwe are proposing, combined with its robustness and sensitivity, will facilitate accessing quantitative x-ray phase-contrast microtomography as a research tool across disciplines, including tissue engineering, materials science, and nondestructive testing in general.
In this work, parametric excitation is introduced in a fully balanced flexible rotor mounted on two identical active gas foil bearings. The active gas foil bearings change the top foil shape harmonically with a specific amplitude and frequency. The deformable foil shape is approximated by an analytical function, while the gas pressure distribution is evaluated by the numerical solution of the Reynolds equation for compressible flow. The harmonic variation of the foil shape generates a respective variation in the bearings’ stiffness and damping properties and the system experiences parametric resonances and antiresonances in specific excitation frequencies. The nonlinear gas bearing forces generate bifurcations in the solutions of the system at certain rotating speeds and excitation frequencies; period doubling and Neimark-Sacker bifurcations are noticed in the examined system, and their progress is evaluated as the two bifurcation parameters (rotating speed and parametric excitation frequency) are changed, though a codimension-2 numerical continuation of limit cycles. It is found that at specific range of excitation frequency there are parametric anti-resonances and the bifurcations collide and vanish. Therefore, a bifurcation-free operating range is established and the system can operate stable at a wide speed range.
Pooled data from published reports on infants with clinically diagnosed vitamin B12 (B12) deficiency were analyzed with the purpose of describing the presentation, diagnostic approaches, and risk factors for the condition to inform prevention strategies. An electronic (PubMed database) and manual literature search following the PRISMA approach was conducted (preregistration with the Open Science Framework, accessed on 15 February 2023). Data were described and analyzed using correlation analyses, Chi-square tests, ANOVAs, and regression analyses, and 102 publications (292 cases) were analyzed. The mean age at first symptoms (anemia, various neurological symptoms) was four months; the mean time to diagnosis was 2.6 months. Maternal B12 at diagnosis, exclusive breastfeeding, and a maternal diet low in B12 predicted infant B12, methylmalonic acid, and total homocysteine. Infant B12 deficiency is still not easily diagnosed. Methylmalonic acid and total homocysteine are useful diagnostic parameters in addition to B12 levels. Since maternal B12 status predicts infant B12 status, it would probably be advantageous to target women in early pregnancy or even preconceptionally to prevent infant B12 deficiency, rather than to rely on newborn screening that often does not reliably identify high-risk children.
Purpose – The purpose of this study is to explore the exogenous and endogenous drivers of the high-growth of Unicorn start-ups along their life cycle, with a particular focus on Unicorns in the fintech industry.
Design/methodology/approach – The study employs an explorative longitudinal analysis with a matched pair of two cases of Unicorns start-ups with similar antecedent features to understand holistically drivers over the longer term.
Findings – High-growth patterns over the longer term are the result of a combined industry- and company-life cycle perspective. Drivers and growth patterns vary significantly according to the time of entry in the industry and
its development status. The findings are systematised within a set of propositions to be tested in future research.
Research limitations/implications – The limitations lie in empirical evidence, as the analysis is limited to one matched-pair. The revealed Unicorns’ drivers for long-term growth might encourage future research to further investigate these drivers on a larger scale.
Practical implications – The study offers practical recommendations for start-ups with high-growth ambitions and advice to policy makers regarding the development of tailor-made support programs.
Originality/value – The study significantly extends extant work on growth and high-growth by examining endogenous and exogenous triggers over time and by linking the Unicorn-life cycle to the industry life cycle, an approach which has, to the best of the authors’ knowledge, not yet been applied.
Background: Cardiovascular disease is the major cause of death worldwide. Although knowledge regarding diagnosing and treating cardiovascular disease has increased dramatically, secondary prevention remains insufficiently implemented due to failure among affected individuals to adhere to guideline recommendations. This has continued to lead to high morbidity and mortality rates. Involving patients in their healthcare and facilitating their active roles in their chronic disease management is an opportunity to meet the needs of the increasing number of cardio-vascular patients. However, simple recall of advice regarding a more preventive lifestyle does not affect sustainable behavioral lifestyle changes. We investigate the effect of plaque visualization combined with low-threshold daily lifestyle tasks using the smartphone app PreventiPlaque to evaluate change in cardiovascular risk profile. Methods: and study design: This randomized, controlled clinical trial includes 240 participants with ultrasound evidence of atherosclerotic plaque in one or both carotid arteries, defined as focal thickening of the vessel wall measuring 50% more than the regular vessel wall. A criterion for participation is access to a smartphone suitable for app usage. The participants are randomly assigned to an intervention or a control group. While both groups receive the standard of care, the intervention group has additional access to the PreventiPlaque app during the 12-month follow-up. The app includes daily tasks that promote a healthier lifestyle in the areas of smoking cessation, medication adherence, physical activity, and diet. The impact of plaque visualization and app use on the change in cardiovascular risk profile is assessed by SCORE2. Feasibility and effectiveness of the PreventiPlaque app are evaluated using standardized and validated measures for patient feedback.