Pompe disease results from lysosomal acid α-glucosidase deficiency, which leads to cardiomyopathy in all infantile-onset and occasional late-onset patients. Cardiac assessment is important for its diagnosis and management. This article presents unpublished cardiac findings, concomitant medications, and cardiac efficacy and safety outcomes from the ADVANCE study; trajectories of patients with abnormal left ventricular mass z score at enrolment; and post hoc analyses of on-treatment left ventricular mass and systolic blood pressure z scores by disease phenotype, GAA genotype, and “fraction of life” (defined as the fraction of life on pre-study 160 L production-scale alglucosidase alfa). ADVANCE evaluated 52 weeks’ treatment with 4000 L production-scale alglucosidase alfa in ≥1-year-old United States of America patients with Pompe disease previously receiving 160 L production-scale alglucosidase alfa. M-mode echocardiography and 12-lead electrocardiography were performed at enrolment and Week 52. Sixty-seven patients had complete left ventricular mass z scores, decreasing at Week 52 (infantile-onset patients, change −0.8 ± 1.83; 95% confidence interval −1.3 to −0.2; all patients, change −0.5 ± 1.71; 95% confidence interval −1.0 to −0.1). Patients with “fraction of life” <0.79 had left ventricular mass z score decreasing (enrolment: +0.1 ± 3.0; Week 52: −1.1 ± 2.0); those with “fraction of life” ≥0.79 remained stable (enrolment: −0.9 ± 1.5; Week 52: −0.9 ± 1.4). Systolic blood pressure z scores were stable from enrolment to Week 52, and no cohort developed systemic hypertension. Eight patients had Wolff–Parkinson–White syndrome. Cardiac hypertrophy and dysrhythmia in ADVANCE patients at or before enrolment were typical of Pompe disease. Four-thousand L alglucosidase alfa therapy maintained fractional shortening, left ventricular posterior and septal end-diastolic thicknesses, and improved left ventricular mass z score.

Trial registry: ClinicalTrials.gov Identifier: NCT01526785 https://clinicaltrials.gov/ct2/show/NCT01526785.

Social Media Statement: Post hoc analyses of the ADVANCE study cohort of 113 children support ongoing cardiac monitoring and concomitant management of children with Pompe disease on long-term alglucosidase alfa to functionally improve cardiomyopathy and/or dysrhythmia.

At present, analysis of diet and bladder cancer (BC) is mostly based on the intake of individual foods. The examination of food combinations provides a scope to deal with the complexity and unpredictability of the diet and aims to overcome the limitations of the study of nutrients and foods in isolation. This article aims to demonstrate the usability of supervised data mining methods to extract the food groups related to BC. In order to derive key food groups associated with BC risk, we applied the data mining technique C5.0 with 10-fold cross-validation in the BLadder cancer Epidemiology and Nutritional Determinants study, including data from eighteen case–control and one nested case–cohort study, compromising 8320 BC cases out of 31 551 participants. Dietary data, on the eleven main food groups of the Eurocode 2 Core classification codebook, and relevant non-diet data (i.e. sex, age and smoking status) were available. Primarily, five key food groups were extracted; in order of importance, beverages (non-milk); grains and grain products; vegetables and vegetable products; fats, oils and their products; meats and meat products were associated with BC risk. Since these food groups are corresponded with previously proposed BC-related dietary factors, data mining seems to be a promising technique in the field of nutritional epidemiology and deserves further examination.

The Numeniini is a tribe of 13 wader species (Scolopacidae, Charadriiformes) of which seven are Near Threatened or globally threatened, including two Critically Endangered. To help inform conservation management and policy responses, we present the results of an expert assessment of the threats that members of this taxonomic group face across migratory flyways. Most threats are increasing in intensity, particularly in non-breeding areas, where habitat loss resulting from residential and commercial development, aquaculture, mining, transport, disturbance, problematic invasive species, pollution and climate change were regarded as having the greatest detrimental impact. Fewer threats (mining, disturbance, problematic native species and climate change) were identified as widely affecting breeding areas. Numeniini populations face the greatest number of non-breeding threats in the East Asian-Australasian Flyway, especially those associated with coastal reclamation; related threats were also identified across the Central and Atlantic Americas, and East Atlantic flyways. Threats on the breeding grounds were greatest in Central and Atlantic Americas, East Atlantic and West Asian flyways. Three priority actions were associated with monitoring and research: to monitor breeding population trends (which for species breeding in remote areas may best be achieved through surveys at key non-breeding sites), to deploy tracking technologies to identify migratory connectivity, and to monitor land-cover change across breeding and non-breeding areas. Two priority actions were focused on conservation and policy responses: to identify and effectively protect key non-breeding sites across all flyways (particularly in the East Asian- Australasian Flyway), and to implement successful conservation interventions at a sufficient scale across human-dominated landscapes for species’ recovery to be achieved. If implemented urgently, these measures in combination have the potential to alter the current population declines of many Numeniini species and provide a template for the conservation of other groups of threatened species.

Condors and vultures comprise the only group of terrestrial vertebrates in the world that are obligate scavengers, and these species move widely to locate ephemeral, unpredictable, and patchily-distributed food resources. In this study, we used high-resolution GPS location data to quantify monthly home range size of the critically endangered California Condor Gymnogyps californianus throughout the annual cycle in California. We assessed whether individual-level characteristics (age, sex and breeding status) and factors related to endangered species recovery program efforts (rearing method, release site) were linked to variation in monthly home range size. We found that monthly home range size varied across the annual cycle, with the largest monthly home ranges observed during late summer and early fall (July–October), a pattern that may be linked to seasonal changes in thermals that facilitate movement. Monthly home ranges of adults were significantly larger than those of immatures, but males and females used monthly home ranges of similar size throughout the year and breeding adults did not differ from non-breeding adults in their average monthly home range size. Individuals from each of three release sites differed significantly in the size of their monthly home ranges, and no differences in monthly home range size were detected between condors reared under captive conditions relative to those reared in the wild. Our study provides an important foundation for understanding the movement ecology of the California Condor and it highlights the importance of seasonal variation in space use for effective conservation planning for this critically endangered species.

Cantaloupe and watermelon growers in the southeastern United States use a system of hybrid transplants grown on narrow low-density polyethylene (LDPE) mulch-covered seedbeds with overhead irrigation and use the mulch cover for only one crop. LDPE mulches are costly to remove from the field and dispose. Biodegradable mulches that eliminate removal and disposal costs would be of significant benefit, provided that weeds are adequately suppressed. Cotton gin trash is a biodegradable waste product, composed of fiber fragments and seed pieces. Using a proprietary process, cotton gin trash can be chopped, pressed, and heated into a loose mat and stored on a continuous roll. Preliminary studies were conducted in 2010 and 2011 to determine if rolled cotton fiber mulch made from gin trash could be applied as a seedbed cover using conventional application equipment and adequately suppress weeds. Mulching materials (rolls 91 cm wide) were applied with a mulch layer that produced a finished seedbed 40 cm wide. ‘Athena' cantaloupe and ‘Crimson Sweet' watermelon were transplanted using a waterwheel transplanter. Mulching materials included rolled cotton fiber mulch sprayed with boiled linseed oil after mulch application, rolled cotton fiber mulch sprayed with black latex paint, black LDPE, and bare ground. Herbicide treatments included ethalfluralin, halosulfuron, and glyphosate applied as directed spray and nontreated with herbicides. Rolled cotton fiber mulch was easily applied with a conventional mulch layer with no modification and minimal adjustment, producing no tears or holes. Biodegradable cotton fiber mulch treated with boiled linseed oil or black latex paint suppressed weeds equally well as LDPE and all were better than bare-ground plots. Herbicides improved control of all weeds over the nontreated control and this effect was independent of weed suppression provided by seedbed mulches. Cantaloupe and watermelon yields were not affected by seedbed mulches, but were increased by weed control provided by herbicides.

Five hundred five blood cultures collected through a peripheral intravenous catheter (PIV) in an emergency department were matched to cultures obtained by dedicated venipuncture from the same patient within 10 minutes. The relative risk of contamination for cultures collected through PIVs compared with dedicated venipuncture was 1.83 (95% confidence interval, 1.08–3.11).

The financial impact of an antimicrobial stewardship program in operation for more than 11 years was determined by calculating the reduction in antimicrobial expenditures minus program labor costs. Depending on the method of inflation adjustment used, the program was associated with average cost savings of $920,070 to $2,064,441 per year.

Background: The purpose of this study was to examine the mediating impact of executive functioning on the link between other neuropsychological domain scores and informant-based rating of functional status.

Methods: Data on 181 participants were analyzed from an ongoing epidemiological study of rural health, Project FRONTIER (mean age = 64.6 ± 13.8 years, 69% women, 42% Mexican American). Executive functioning was assessed by the EXIT25 and other neuropsychological domains were assessed via the Repeatable Battery for the Assessment of Neuropsychological Status (RBANS). Informant-based rating of functional status was assessed via the Clinical Dementia Rating Scale sum of boxes scores (CDR SB).

Results: RBANS Index scores were each significantly (p < 0.05) related to CDR SB scores and EXIT25 scores. EXIT25 score was a significant partial mediator of the link between four RBANS indices (Immediate Memory, Attention, Visuospatial/Construction, Delayed Memory) and CDR SB scores, and a complete mediator of the fifth index (Language).

Conclusion: Executive functioning is a mediator of the link between other neuropsychological domains and daily functioning. Neuropsychological assessments that do not measure executive functioning will provide only a partial clinical picture with adults and elders.

We explain how higher homotopy operations, defined topologically, may be identified under mild assumptions with (the last of) the Dwyer-Kan-Smith cohomological obstructions to rectifying homotopy-commutative diagrams.