Duchenne muscular dystrophy is characterised by fibrofatty replacement of muscle, resulting in dilated cardiomyopathy. Hypertrophic cardiomyopathy affects 1:200–1:500 people and is characterised by asymmetric ventricular septal hypertrophy. To date, there have been two separately reported cases describing the combined pathology of these disorders. Herein, we expand upon these reports with a case series describing longitudinal findings in three patients with Duchenne muscular dystrophy who developed hypertrophic cardiomyopathy.

As the federal government continues to expand upon and improve its data sharing policies over the past 20 years, complex challenges remain. Our interviews with U.S. academic genetic researchers (n=23) found that the burden, translation, industry limitations, and consent structure of data sharing remain major governance challenges.

Avian schistosomes are snail-borne trematode parasites (Trichobilharzia spp.) that can cause a nasty skin rash in humans when their cercariae mistake us for their normal bird hosts. We sought to investigate drivers of the spatial distribution of Trichobilharzia cercaria abundance throughout Northern Michigan lakes. For 38 sites on 16 lakes, we assessed several dozen potential environmental predictors that we hypothesized might have direct or indirect effects on overall cercaria abundance, based on known relationships between abiotic and biotic factors in wetland ecosystems. We included variables quantifying local densities of intermediate hosts, temperature, periphyton growth rates, human land use and hydrology. We also measured daily abundance of schistosome cercariae in the water over a 5-week period, supported by community scientists who collected and preserved filtered water samples for qPCR. The strongest predictor of cercaria abundance was Lymnaea host snail density. Lymnaea density was higher in deeper lakes and at sites with more deciduous tree cover, consistent with their association with cool temperature habitats. Contrary to past studies of human schistosomes, we also found a significant negative relationship between cercaria abundance and submerged aquatic vegetation, possibly due to vegetation blocking cercaria movement from offshore snail beds. If future work shows that these effects are indeed causal, then these results suggest possible new approaches to managing swimmer's itch risk in northern MI lakes, such as modifying tree cover and shallow-water vegetation at local sites.

This article is a clinical guide which discusses the “state-of-the-art” usage of the classic monoamine oxidase inhibitor (MAOI) antidepressants (phenelzine, tranylcypromine, and isocarboxazid) in modern psychiatric practice. The guide is for all clinicians, including those who may not be experienced MAOI prescribers. It discusses indications, drug-drug interactions, side-effect management, and the safety of various augmentation strategies. There is a clear and broad consensus (more than 70 international expert endorsers), based on 6 decades of experience, for the recommendations herein exposited. They are based on empirical evidence and expert opinion—this guide is presented as a new specialist-consensus standard. The guide provides practical clinical advice, and is the basis for the rational use of these drugs, particularly because it improves and updates knowledge, and corrects the various misconceptions that have hitherto been prominent in the literature, partly due to insufficient knowledge of pharmacology. The guide suggests that MAOIs should always be considered in cases of treatment-resistant depression (including those melancholic in nature), and prior to electroconvulsive therapy—while taking into account of patient preference. In selected cases, they may be considered earlier in the treatment algorithm than has previously been customary, and should not be regarded as drugs of last resort; they may prove decisively effective when many other treatments have failed. The guide clarifies key points on the concomitant use of incorrectly proscribed drugs such as methylphenidate and some tricyclic antidepressants. It also illustrates the straightforward “bridging” methods that may be used to transition simply and safely from other antidepressants to MAOIs.

Patients with Duchenne muscular dystrophy have multiple risk factors for lower extremity oedema. This study sought to define the frequency and predictors of oedema. Patients aged 15 years and older were screened by patient questionnaire, and the presence of oedema was confirmed by subsequent physical exam. Twenty-four of 52 patients (46%) had oedema, 12 of whom had swelling extending above the foot and two with sores/skin breakdown. There was no significant difference in age, frequency, or duration of glucocorticoid use, non-invasive respiratory support use, forced vital capacity, cardiac medication use, or ejection fraction between patients with and without oedema (all p > 0.2). Those with oedema had a greater time since the loss of ambulation (8.4 years versus 3.5 years; p = 0.004), higher body mass index (28.3 versus 24.8; p = 0.014), and lower frequency of deflazacort use (67% versus 89%; p = 0.008). Multivariate analysis revealed a longer duration of loss of ambulation (p = 0.02) and higher body mass index (p = 0.009) as predictors of oedema. Lower extremity oedema is common in Duchenne muscular dystrophy but independent of cardiac function. Interventions focused on minimising body mass index increases over time may be a therapeutic target.

We sought to define the prevalence of echocardiographic abnormalities in long-term survivors of paediatric hematopoietic stem cell transplantation and determine the utility of screening in asymptomatic patients. We analysed echocardiograms performed on survivors who underwent hematopoietic stem cell transplantation from 1982 to 2006. A total of 389 patients were alive in 2017, with 114 having an echocardiogram obtained ⩾5 years post-infusion. A total of 95 patients had echocardiogram performed for routine surveillance. The mean time post-hematopoietic stem cell transplantation was 13 years. Of 95 patients, 77 (82.1%) had ejection fraction measured, and 10/77 (13.0%) had ejection fraction z-scores ⩽−2.0, which is abnormally low. Those patients with abnormal ejection fraction were significantly more likely to have been exposed to anthracyclines or total body irradiation. Among individuals who received neither anthracyclines nor total body irradiation, only 1/31 (3.2%) was found to have an abnormal ejection fraction of 51.4%, z-score −2.73. In the cohort of 77 patients, the negative predictive value of having a normal ejection fraction given no exposure to total body irradiation or anthracyclines was 96.7% at 95% confidence interval (83.3–99.8%). Systolic dysfunction is relatively common in long-term survivors of paediatric hematopoietic stem cell transplantation who have received anthracyclines or total body irradiation. Survivors who are asymptomatic and did not receive radiation or anthracyclines likely do not require surveillance echocardiograms, unless otherwise indicated.

Hyperbolic polariton modes are highly appealing for a broad range of applications in nanophotonics, including surfaced enhanced sensing, sub-diffractional imaging, and reconfigurable metasurfaces. Here we show that attenuated total reflectance (ATR) micro-spectroscopy using standard spectroscopic tools can launch hyperbolic polaritons in a Kretschmann–Raether configuration. We measure multiple hyperbolic and dielectric modes within the naturally hyperbolic material hexagonal boron nitride as a function of different isotopic enrichments and flake thickness. This overcomes the technical challenges of measurement approaches based on nanostructuring, or scattering scanning near-field optical microscopy. Ultimately, our ATR approach allows us to compare the optical properties of small-scale materials prepared by different techniques systematically.

Studies were conducted to determine whether glufosinate treatments to glufosinate-resistant cotton caused changes in floral morphology, pollen viability, and seed set. Four glufosinate treatments were included: (1) glufosinate applied postemergence over the top (POST) at the four-leaf stage, (2) glufosinate applied POST at the eight-leaf stage, (3) the first two treatments sequentially, and (4) a POST application at the four-leaf stage followed by (fb) a postemergence-directed stem application (PDS) at the eight-leaf stage. Glufosinate was consistently applied at 0.49 kg ai/ha. A nontreated control was included. Glufosinate treatments did not affect stigma height, length of the staminal column, or pollen viability. However, the distance from the top anther to the tip of the stigma was less in plants treated with an eight-leaf POST treatment than in nontreated plants, although this difference is not likely to influence pollen deposition because in both cases anthers reached above the stigma tip. Plants receiving four-leaf POST fb eight-leaf PDS treatment with glufosinate had eight seeds per boll less than nontreated plants; however, the more rigorous four-leaf POST fb eight-leaf POST treatment did not differ from the nontreated in seeds per boll.

Lake Pend Oreille is the largest (36,000 ha or 91,000 ac) freshwater lake in Idaho. Approximately 27% or 10,000 ha of the lake is littoral zone habitat supporting aquatic macrophyte growth. Eurasian watermilfoil has invaded large areas of this littoral zone habitat, with early estimates suggesting approximately 2,000 ha by the mid 2000s. Idaho State Department of Agriculture developed a state-wide eradication program in response to the threats posed by Eurasian watermilfoil, which attempts to quantify Eurasian watermilfoil infestations and its effects on the native plant community. Littoral zone point intercept surveys were conducted in 2007 and 2008 to monitor the trends in aquatic macrophyte community structure and assess management strategies against Eurasian watermilfoil. Lake Pend Oreille has a species-rich aquatic macrophyte community of more than 50 species. Lake-wide, the presence of Eurasian watermilfoil significantly decreased from 2007 (12.5%) to 2008 (7.9%). The native plant community has remained stable from 2007 to 2008 despite lake-wide management activities. In managed areas, the frequency of Eurasian watermilfoil during the 2008 assessment was 23.6% after herbicide applications. This represents a 63% reduction in Eurasian watermilfoil presence from the 2007 (64.5%) survey. When 2,4-D was combined with endothall, the presence of Eurasian watermilfoil declined from 63% (2007) to 36.5% in 2008. Eurasian watermilfoil treated with triclopyr also declined significantly, 64% to 18.2%. When all treatment methods were pooled and compared with areas that were not treated, the presence of Eurasian watermilfoil was significantly greater (52.5%) in untreated areas as opposed to treated areas (23%). The removal of Eurasian watermilfoil resulted in an increase in native species in most areas. Currently, there is as little as 200 ha of Eurasian watermilfoil remaining, which represents an overall reduction of 90% in approximately 7 yr of management.

Miscanthus sinensis is a perennial grass native to Asia, but since its introduction to the United States in the late 19th century, it has become both a major ornamental crop and invasive species. Previous studies of the ecology of M. sinensis in both its introduced and native ranges have suggested that it may be occupying a novel ecological niche in the introduced range. Miscanthus sinensis and its daughter species, Miscanthus × giganteus, are under evaluation as bioenergy crops; therefore, characterization of the ecology and environmental niche of M. sinensis is essential to mitigate the risk of fostering future invasion in the United States. In July 2011, we surveyed 18 naturalized M. sinensis populations spanning the U.S. distribution, covering a 6° latitudinal gradient from North Carolina to Massachusetts. Miscanthus sinensis populations ranged in size from 3 to 181,763 m2 with densities between 0.0012 and 2.2 individuals m−2, and strongly favored highly disturbed and unmanaged habitats such as roadsides and forest edges. Population size and individual plant morphology (i.e., tiller height, basal diameter, and tiller number) were not affected by soil characteristics and nutrient availability, though increased tree canopy cover was associated with reduced population size (P < 0.0001). Plant size and vigor were not significantly affected by low light availability, which supports previous suggestions of shade tolerance of M. sinensis. In summary, M. sinensis can tolerate a broad range of climatic conditions, light availability, and nutrient availability in the eastern United States, suggesting risk of further invasion beyond its current distribution in the United States.

Field studies were conducted in 2000 and 2001 to evaluate corn yield-loss predictions generated by WeedSOFT, a computerized weed management decision aid. Conventional tillage practices were used to produce corn in 76-cm rows in Illinois, Indiana, Kansas, Michigan, Missouri, Nebraska, and Wisconsin. A total of 21 site-years from these seven states were evaluated in this study. At 4 wk after planting, weed densities and size, crop-growth stage, estimated weed-free yield, and environmental conditions at the time of application were entered into WeedSOFT to generate POST treatments ranked by percent maximum yield (PMY). POST treatments were chosen with yield losses ranging from 0 to 20%. Data were subjected to linear regression analysis by state and pooled over all states to determine the relationship between actual and predicted yield loss. A slope value equal to one implies perfect agreement between actual and predicted yield loss. Slope value estimates for Illinois and Missouri were equal to one. Actual yield losses were higher than the software predicted in Kansas and lower than predicted in Michigan, Nebraska, and Wisconsin. Slope value estimate from a data set containing all site years was equal to one. This research demonstrated that variability in yield-loss predictions occurred at sites that contained a high density of a single weed specie (>100/m2) regardless of its competitive index (CI); at sites with a predominant broadleaf weed with a CI greater than five, such as Palmer amaranth, giant ragweed, common sunflower, and common cocklebur; and at sites that experience moderate to severe drought stress.

Knowledge on the ecology of humpback whales in the eastern North Atlantic is lacking by comparison with most other ocean basins. Humpback whales were historically over-exploited in the region and are still found in low relative abundances. This, coupled with their large range makes them difficult to study. With the aim of informing more effective conservation measures in Ireland, the Irish Whale and Dolphin Group began recording sightings and images suitable for photo-identification of humpback whales from Irish waters in 1999. Validated records submitted by members of the public and data from dedicated surveys were analysed to form a longitudinal study of individually recognizable humpback whales. The distribution, relative abundance and seasonality of humpback whale sighting records are presented, revealing discrete important areas for humpback whales in Irish coastal waters. An annual easterly movement of humpback whales along the southern coast of Ireland is documented, mirroring that of their preferred prey: herring and sprat. Photo-identification images were compared with others collected throughout the North Atlantic (N = 8016), resulting in matches of two individuals between Ireland and Iceland, Norway and the Netherlands but no matches to known breeding grounds (Cape Verde and West Indies). This study demonstrates that combining public records with dedicated survey data is an effective approach to studying low-density, threatened migratory species over temporal and spatial scales that are relevant to conservation and management.

Thin native oxide layers can dominate the mechanical properties of metallic thin films. However, to date there has been little quantification of how such overlayers affect yield and fracture during indentation in constrained film systems. To gain insight into such processes, electrical contact resistance was measured in situ during nanoindentation on constrained thin films of epitaxial Cr and polycrystalline Al, both possessing a native oxide overlayer. Measurements during loading of the films show both increases and decreases in current, which can then be used to distinguish between various sources of plasticity. Ex situ measurements of the oxide thickness are used to provide a starting point for elasticity simulations of stress in both systems. The results show that dislocation nucleation in the metal film can be differentiated from oxide fracture during indentation.