To send 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 sending content to .
To send content items to your Kindle, first ensure firstname.lastname@example.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 sending to your Kindle.
Note you can select to send to either the @free.kindle.com or @kindle.com variations.
‘@free.kindle.com’ emails are free but can only be sent 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.
Background: Mutations of the slow skeletal muscle troponin-T1 (TNNT1) gene are a rare cause of nemaline myopathy. The phenotype is characterized by severe amyotrophy and contractures. Death from respiratory insufficiency occurs in infancy. We report on four French Canadians with a novel congenital TNNT1-related myopathy. Methods: Patients underwent MRI of leg muscles, quadriceps biopsy and genetic testing. Wild type or mutated human TNNT1 mRNAs were co-injected with morpholinos in a zebrafish knockdown model to assess their relative abilities to rescue the morphant phenotype. Results: Three adults and one child shared a novel missense homozygous pathogenic variant in the TNNT1 gene. They developed from childhood slowly progressive limb-girdle weakness with spinal rigidity and contractures. They suffered from restrictive lung disease and recurrent episodes of infection-triggered rhabdomyolysis, which were relieved by dantrolene in one patient. Older patients remained ambulatory into their sixties. MRI of leg muscles showed symmetrical atrophy and fatty infiltration in a proximal-to-distal gradient. Biopsies showed multi-minicores, while nemaline rods were seen in half the patients. Wild type TNNT1 mRNA rescued the zebrafish morphants but mutant transcripts failed to rescue the morphants. Conclusions: This study expands the spectrum of TNNT1-related myopathy to include a milder clinical phenotype caused by a functionally-confirmed novel missense mutation.
Norovirus is the leading cause of acute gastroenteritis in the USA. Although secondary household transmission of norovirus is frequently reported in outbreaks, little is known about specific risk factors for susceptibility and infectiousness in the household. Three norovirus outbreaks were investigated and data were collected on individuals exposed in the primary outbreak setting and their household members. Potential individual- and household-level risk factors for susceptibility and infectiousness were assessed using univariate and multivariate generalised linear mixed models. In the univariate models, the secondary attack rate (SAR) was significantly higher when living in a household with two or more primary cases (incidence rate ratio (IRR) = 2·1; 95% confidence interval (CI) 1·37–3·29), more than one primary case with vomiting (IRR = 1·9; CI 1·11–3·37), and at least one primary case with diarrhoea (IRR = 3·0; CI 1·46–6·01). After controlling for other risk factors in the multivariate models, the SAR was significantly higher among those living in a household with two or more primary cases (adjusted IRR = 2·0; CI 1·17–3·47) and at least one primary case with diarrhoea (adjusted IRR = 2·8; CI 1·35–5·93). These findings underscore the importance of maintaining proper hygiene and isolating ill household members to prevent norovirus transmission in the household.
Climate change has been identified as the biggest global health threat of the twenty-first century. Hundreds of millions of people around the world currently suffer from allergic diseases such as asthma and allergic rhinitis (hay fever), and the prevalence of these diseases is increasing. This book is the first authoritative and comprehensive assessment of the many impacts of climate change on allergens, such as pollen and mould spores, and allergic diseases. The international authorship team of leaders in this field explore the topic to a breadth and depth far beyond any previous work. This book will be of value to anyone with an interest in climate change, environmental allergens, and related allergic diseases. It is written at a level that is accessible for those working in related physical, biological, and health and medical sciences, including researchers, academics, clinicians, and advanced students.
Using physical experiments as oracles for algorithms, we can characterise the computational power of classes of physical systems. Here we show that two different physical models of the apparatus for a single experiment can have different computational power. The experiment is the scatter machine experiment (SME), which was first presented in Beggs and Tucker (2007b). Our first physical model contained a wedge with a sharp vertex that made the experiment non-deterministic with constant runtime. We showed that Turing machines with polynomial time and an oracle based on a sharp wedge computed the non-uniform complexity class P/poly. Here we reconsider the experiment with a refined physical model where the sharp vertex of the wedge is replaced by any suitable smooth curve with vertex at the same point. These smooth models of the experimental apparatus are deterministic. We show that no matter what shape is chosen for the apparatus:
(i)the time of detection of the scattered particles increases at least exponentially with the size of the query; and
(ii)Turing machines with polynomial time and an oracle based on a smooth wedge compute the non-uniform complexity class P/log* ⫋ P/poly.
We discuss evidence that many experiments that measure quantities have exponential runtimes and a computational power of P/log*.
We pose the following question: If a physical experiment were to be completely controlled by an algorithm, what effect would the algorithm have on the physical measurements made possible by the experiment?
In a programme to study the nature of computation possible by physical systems, and by algorithms coupled with physical systems, we have begun to analyse:
(i)the algorithmic nature of experimental procedures; and
(ii)the idea of using a physical experiment as an oracle to Turing Machines.
To answer the question, we will extend our theory of experimental oracles so that we can use Turing machines to model the experimental procedures that govern the conduct of physical experiments. First, we specify an experiment that measures mass via collisions in Newtonian dynamics and examine its properties in preparation for its use as an oracle. We begin the classification of the computational power of polynomial time Turing machines with this experimental oracle using non-uniform complexity classes. Second, we show that modelling an experimenter and experimental procedure algorithmically imposes a limit on what can be measured using equipment. Indeed, the theorems suggest a new form of uncertainty principle for our knowledge of physical quantities measured in simple physical experiments. We argue that the results established here are representative of a huge class of experiments.
Trapping experiments have been performed at the Idaho National Laboratory to assess the performance of AgX sorbent media in capturing volatile iodine during the oxidation of irradiated oxide fuel. The demonstration of iodine release and capture from the used fuel has been accomplished with laboratory-scale equipment in a hot cell environment. Iodine loadings as high as 6 ug/g media have been achieved via chemical adsorption with filter efficiencies in excess of 90%. In addition to iodine, significant quantities of tritium have also been collected on the AgX filter media. Filter media loaded with radioactive iodine has been sequestered in a tin matrix by hot isostatic pressing at 200°C. The placement and encapsulation of the sorbent media was examined by neutron radiography, thus confirming the sequestration of radioactive iodine.
Five large outbreaks of food poisoning are described in which clinical, epidemiological or laboratory data indicated Clostridium perfringens as the causative organism. The foodstuff common to all incidents was boiled salmon served cold as an hors d'oeuvre. In all cases the fish had been subject to a long period of cooling or storage between boiling and consumption. It is thought that multiplication of the organism occurred during this time.
Recommendations are made for the avoidance of further similar incidents.