We use cookies to distinguish you from other users and to provide you with a better experience on our websites. Close this message to accept cookies or find out how to manage your cookie settings.
To save content items to your account,
please confirm that you agree to abide by our usage policies.
If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account.
Find out more about saving content to .
To save content items to your Kindle, first ensure no-reply@cambridge.org
is added to your Approved Personal Document E-mail List under your Personal Document Settings
on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part
of your Kindle email address below.
Find out more about saving to your Kindle.
Note you can select to save to either the @free.kindle.com or @kindle.com variations.
‘@free.kindle.com’ emails are free but can only be saved to your device when it is connected to wi-fi.
‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.
The aim of this study is to assess the evolution of respiratory and feeding support in children with spinal muscular atrophy (SMA) type 1 after 24 months of nusinersen treatment.
Methods:
Data on SMA type 1 children treated with nusinersen between 2017 and 2023 from the Canadian Neuromuscular Disease Registry were extracted. The cohort was divided into two groups based on age at treatment initiation: ≤2 years and >2 years. The primary outcome was the (i) time to death or needing full-time (≥16 hours/day) ventilation and (ii) time to needing feeding tube support. The secondary outcomes were differences in respiratory and feeding support requirements between the two groups at 24-month follow-up.
Results:
Thirty-two children were included, and the median age (range) for treatment initiation was 3.2 months (0.8– 13.1) in children who initiated treatment at ≤2 years and 51.2 (28.7–183.8) in those who initiated at >2 years of age. The median age of death or full-time ventilation was 8.6 months (6–22.4) and 10.5 months (4–24) for the two groups, respectively. The median age for initiation of feeding support was 5.1 (1.7–26.4) and 14.5 months (3.9–130.6), respectively. At 24 months (n = 23), there were no significant differences between the need for respiratory or feeding tube support between the two treatment groups.
Conclusion:
Most children with SMA type 1 treated with nusinersen across Canada have continued need for respiratory and feeding support over time when initiated after symptom onset.
Climatic and atmospheric conditions impact mental health, including increased incidents of depression associated with air pollution. A growing body of research considers time-bound ‘snap-shots’ of climatic drivers and mental health outcomes. Less is known about the likely effects of future climate change on mental health. Research is often inhibited by data scarcity, the challenge of synthesising data across multiple geospatial and temporal scales, and the under-representation of hard-to-reach groups. Thus, research methods are needed to integrate and analyse complex environmental and mental health multi-datasets while improving the visibility of under-represented groups. In this methods paper we present a novel approach for investigating the impact of climate change on mental health and addressing some challenges with, a) invisibility of vulnerable groups, and b) integrating complex environmental and mental health multi-datasets. The research aim is to pilot a web-based and smartphone application (Methane Early Warning Network (ME-NET)) for investigating the role of methane as a precursor of on-ground ozone, and the impact of ozone on mental health outcomes to improve civic knowledge and health-protection behaviour in the United Kingdom and Ghana. The methods include exploring the feasibility of using machine learning to develop an ozone early warning system and application co-design.
Bruce Harris has left an indelible mark on public law across the common law world. In this Festschrift, commentators explore key themes including the nature of executive power, issues concerning the judiciary, and the future of unwritten constitutions. This collection of essays conveys a distinctively pragmatic approach to public law, relevant to scholars and practitioners alike.
Bruce Harris is a stalwart of the New Zealand legal community and epitomises the very best of legal academia. His dedication to teaching in his 23 years at the University of Auckland, in addition to his previous tenure at the University of Otago, has inspired hundreds of lawyers, especially those who have completed his legendary public law honours seminar. His research has sparked new fields of scholarly inquiry from republicanism to judicial conduct, and his suggestion of the ‘third source of executive power’ has been cited and developed in judicial decisions across the Commonwealth. His administrative service – as dean, university councillor and work with the New Zealand Vice Chancellors’ Committee – has been influential and prolific.
But more than a list of achievements, Bruce's career has been marked by remarkable kindness and dedication to his students and colleagues. Anyone who has met Bruce will know of his extraordinarily calm, patient and inclusive approach. Colleagues at both Auckland and Otago universities credit him with making the faculty lounge a warmer place to be. In emails and in person, he insisted on referring to his undergraduate honours students as his ‘colleagues’. But his calm and cordial manner has never been a barrier to passion when necessary. Bruce was known as a champion of student and staffwelfare in boThthe law school and the university at large and was never afraid to speak out against corporatising governance reforms. In class there were certain topics – the Kerr/Whitlam saga, the Saxmere cases, and Henry VIII clauses – which sparked his righteous indignation. and Bruce's career has also been marked by a quiet but mischievous humour. His public law honours seminars were full of quips and witty critiques, and he encouraged a spirit of jovial camaraderie. WiTha glint in his eye and deadpan demeanour, he was known for bringing a box of fried chicken to class to illustrate aspects of contract law, or an antique model of the Queen to explain his theory of the third source. He seems to take joy in eliciting surprised reactions to the unexpected.
Bruce's academic work is impressive in both substance and depth. But the breadth and method of his scholarship is equally impressive. Bruce sees public law as a system and avoids the tendency to think too narrowly in terms of administrative law, human rights, or Te Tiriti o Waitangi: for Bruce, no subfield can make sense detached from another.
No doubt there are many different markers of success for an academic career. If pressed to articulate some of them, many of us might point to one or more of these common standards for intellectual achievement: breadth and depth of scholarship, engagement from critics and interlocutors, development of novel ideas and avenues for research, and perhaps the everyday usefulness to society of one's intellectual contribution.
Bruce Harris's academic career has more than seen these standards met. He is expert in New Zealand public law, having taught and published in the field for more than 40 years. Harris graduated with a Bachelor of Laws degree (with Honours) from the University of Otago in 1974 and a Master of Laws degree from Harvard University in 1975. He was admitted as a barrister and solicitor the following year, and spent two years in practice with a focus on resource management law (a focus reflected in his early academic work). Harris then returned to the University of Otago Faculty of Law where he would teach and research for the next 17 years, before moving to join the Faculty of Law at the University of Auckland in 1994. His commitment to faculty life saw him appointed Dean of Law at each of those two proud universities during his tenure. and, of course, a number of academic accolades have followed, including the Sir Ian Barker Prize for Best Published Article in 2007, and the conferral of a Doctor of Laws degree from the University of Otago in 2012.
In terms of scholastic output, the culmination of Harris's expertise and experience was the publication of New Zealand Constitution: An Analysis in Terms of Principles, which provides a comprehensive yet highly accessible account of how New Zealand's constitution operates. It also reflects Harris's highly pragmatic style of public law scholarship, which strips the law of pretence while somehow losing very little of its nuance. One colleague noted not long after its publication that it deserved to sit alongside the Cabinet Manual as an essential resource for all new Members of Parliament. Experience already tells us that a number of law students also now rely on it as a first port of call when examining constitutional issues for the first time.
Created in London c. 1340, the Auchinleck manuscript (Edinburgh, National Library of Scotland Advocates MS 19.2.1) is of crucial importance as the first book designed to convey in the English language an ambitious range of secular romance and chronicle. Evidently made in London by professional scribes for a secular patron, this tantalizing volume embodies a massive amount of material evidence as to London commercial book production and the demand for vernacular texts in the early fourteenth century. But its origins are mysterious: who were its makers? its users? how was it made? what end did it serve? The essays in this collection define the parameters of present-day Auchinleck studies. They scrutinize the manuscript's rich and varied contents; reopen theories and controversies regarding the book's making; trace the operations and interworkings of the scribes, compiler, and illuminators; tease out matters of patron and audience; interpret the contested signs of linguisticand national identity; and assess Auchinleck's implied literary values beside those of Chaucer. Geography, politics, international relations and multilingualism become pressing subjects, too, alongside critical analyses of literary substance.
Susanna Fein is Professor of English at Kent State University (Kent, Ohio) and editor of The Chaucer Review.
Contributors: Venetia Bridges, Patrick Butler, Siobhain Bly Calkin, A. S. G. Edwards, Ralph Hanna, Ann Higgins, Cathy Hume, Marisa Libbon, Derek Pearsall, Helen Phillips, Emily Runde, Timothy A. Shonk, M-l F. Vaughan.
The first demonstration of laser action in ruby was made in 1960 by T. H. Maiman of Hughes Research Laboratories, USA. Many laboratories worldwide began the search for lasers using different materials, operating at different wavelengths. In the UK, academia, industry and the central laboratories took up the challenge from the earliest days to develop these systems for a broad range of applications. This historical review looks at the contribution the UK has made to the advancement of the technology, the development of systems and components and their exploitation over the last 60 years.