Make it so! Jean-Luc Picard, Bart Simpson and the design of e-public services

In this paper, we report on a project applying participatory design methods to include people who have experience of social exclusion (in one form or another) in designing possible technologies for e-(local)-government services. The work was part of a project for the Office of the Deputy Prime Minister in the UK, and was concerned with ‘access tokens’ that can provide personal identification for individuals accessing public services, based on technologies such as multi-functional smartcards, flash memory sticks, mobile phone SIMs or similar devices. In particular we report on our experience using the ‘pastiche scenarios’ technique recently developed by Mark Blythe. Our findings indicate that the technique can be effective and engaging in helping people to create realistic scenarios of future technology use and highlight some possible pitfalls to consider when using this technique.</p

Disseminated Fusarium oxysporum Infection in Hemophagocytic Lymphohistiocytosis

Abstract : The portal of entry of disseminated Fusarium spp. infections is still not clearly defined. We report on a disseminated Fusarium oxysporum infection occurring during a long period of severe neutropenia in a child with hemophagocytic lymphohistiocytosis. A nasogastric feeding tube was the possible source of entry of the fungu

CRIM-negative infantile Pompe disease: 42-month treatment outcome

Pompe disease is a rare lysosomal glycogen storage disorder characterized by deficiency of acid α-glucosidase enzyme (GAA) and caused by mutations in the GAA gene. Infantile-type Pompe disease is a multiorgan disorder presenting with cardiomyopathy, hypotonia, and muscular weakness, which is usually fatal. Enzyme replacement therapy (ERT) with recombinant human GAA (rhGAA) has recently been shown to be effective and subsequently yielded promising results in cross-reactive immunologic material (CRIM)-positive patients. CRIM-negative patients showed a limited response to ERT and died or were ventilator dependant. Over a period of 44 months, we monitored cognitive and motor development, behavior, auditory function, and brain imaging of a CRIM-negative infantile Pompe disease patient on rhGAA and monoclonal anti-immunoglobulin E (anti-IgE) antibody (omalizumab) treatment due to severe allergic reaction. Cardiorespiratory and skeletal muscle response was significant, with almost normal motor development. Cognitive development-in particular, speech and language-deviated increasingly from normal age-appropriate development and was markedly delayed at 44 months, unexplained by moderate sensorineural hearing impairment. Brain magnetic resonance imaging (MRI) at 18, 30, and 44 months of age revealed symmetrical signal alteration of the deep white matter. Titer values of IgG antibodies to rhGAA always remained <1:800. The potential role of omalizumab in immune modulation remains to be elucidated; however, this is the first report presenting a ventilator-free survival of a CRIM-negative patient beyond the age of 36 months. The central nervous system (CNS) findings are hypothesized to be part of a yet not fully described CNS phenotype in treated patients with longer survival

Clinical data for paediatric research: the Swiss approach : Proceedings of the National Symposium in Bern, Switzerland, Dec 5-6, 2019.

Continuous improvement of health and healthcare system is hampered by inefficient processes of generating new evidence, particularly in the case of rare diseases and paediatrics. Currently, most evidence is generated through specific research projects, which typically require extra encounters with patients, are costly and entail long delays between the recognition of specific needs in healthcare and the generation of necessary evidence to address those needs. The Swiss Personalised Health Network (SPHN) aims to improve the use of data obtained during routine healthcare encounters by harmonizing data across Switzerland and facilitating accessibility for research. The project "Harmonising the collection of health-related data and biospecimens in paediatric hospitals throughout Switzerland (SwissPedData)" was an infrastructure development project funded by the SPHN, which aimed to identify and describe available data on child health in Switzerland and to agree on a standardised core dataset for electronic health records across all paediatric teaching hospitals. Here, we describe the results of a two-day symposium that aimed to summarise what had been achieved in the SwissPedData project, to put it in an international context, and to discuss the next steps for a sustainable future. The target audience included clinicians and researchers who produce and use health-related data on children in Switzerland. The symposium consisted of state-of-the-art lectures from national and international keynote speakers, workshops and plenary discussions. This manuscript summarises the talks and discussions in four sections: (I) a description of the Swiss Personalized Health Network and the results of the SwissPedData project; (II) examples of similar initiatives from other countries; (III) an overview of existing health-related datasets and projects in Switzerland; and (IV) a summary of the lessons learned and future prospective from workshops and plenary discussions. Streamlined processes linking initial collection of information during routine healthcare encounters, standardised recording of this information in electronic health records and fast accessibility for research are essential to accelerate research in child health and make it affordable. Ongoing projects prove that this is feasible in Switzerland and elsewhere. International collaboration is vital to success. The next steps include the implementation of the SwissPedData core dataset in the clinical information systems of Swiss hospitals, the use of this data to address priority research questions, and the acquisition of sustainable funding to support a slim central infrastructure and local support in each hospital. This will lay the foundation for a national paediatric learning health system in Switzerland

Die Allergiekarriere : Grundlage für Frühdiagnostik, Prävention und Frühtherapie allergischer Erkrankungen

Allergic diseases have a peculiar characteristic: at different ages of the child they present under different forms. The typical time course, where atopic children 'grow out' of one allergic disease (e.g. atopic dermatitis) but then suffer from the next form of allergic disease (e.g. allergic asthma) has been termed 'atopic march', or 'allergic march'. Various information can help to identify early in life those children at risk for engaging in this atopic march. Sensitization to hen's egg and mites during the first year of life, long-lasting sensitization to food allergens during the first two years of age and atopic dermatitis in early infancy have been found to be predictors of allergic sensitizations and/or allergic airway disease later on in childhood. Although today no measures are available to completely prevent any manifestation of atopy before disease onset, some measures can contribute to stop or at least slow down the progression of the allergic march. Breast feeding for a least 4 to 6 months, stepwise introduction of solid food after this age only and avoidance of highly allergenic food during the first year of life can help to achieve this goal, as do measures to reduce indoor allergen exposure (especially house dust mites). For selected patients suffering from atopic dermatitis who are sensitized to grass pollen and/or house dust mites and who have highly motivated parents an early treatment with an antihistamine (cetirizine) can be considered

Wheezing und Asthma bei Kindern: Prognose und Stufentherapie

Rezidivierende Wheezingsymptome bei Kindern im Vorschulalter sind häufig nicht mit einem Asthma bronchiale im Schulalter assoziiert. Was gibt es Neues zu Abschätzung der Prognose und Therapie im Vorschulalter

A boy with a one-sided red rash.

We report on a boy with a sudden onset of unilateral skin lesions following tonsillar infection with fever 2 weeks before. The lesions consisted of erythematous macules with scaling affecting trunk, axillar, as well as inguinal region. CRP, blood differential, serum IgG and IgM antibodies (coxsackievirus, cytomegalovirus, parvovirus, herpes virus, varicella zoster virus, human herpesvirus-6/-7), and lesional swabs (bacteria, dermatophytes, yeasts) were uneventful