The genome sequence of the black-footed limpet, Patella depressa (Pennant, 1777).

We present a genome assembly from an individual Patella depressa (the black-footed limpet; Mollusca; Gastropoda; Patellogastropoda; Patellidae). The genome sequence is 683.7 megabases in span. Most of the assembly is scaffolded into 9 chromosomal pseudomolecules. Gene annotation of this assembly on Ensembl identified 20,502 protein coding genes.

The genome sequence of the common limpet, Patella vulgata (Linnaeus, 1758).

We present a genome assembly from an individual Patella vulgata (the common limpet; Mollusca; Gastropoda; Patellogastropoda; Patellidae). The genome sequence is 695.4 megabases in span. Most of the assembly is scaffolded into 9 chromosomal pseudomolecules. The mitochondrial genome has also been assembled and is 14.93 kilobases in length. Gene annotation of this assembly on Ensembl identified 19,378 protein coding genes.

Spatial distribution of meiofaunal and macrofaunal assemblages in the tidal flats of the southern Korean coast in relation to natural and anthropogenic impacts.

We investigated the spatial variability of macrofaunal and meiofaunal assemblages in intertidal flats on the southern coast of Korea. Abiotic and biotic samples were collected at five stations. The species richness, density, and composition of the assemblages differed significantly among stations. Nematoda and Annelida were the most dominant meiofaunal and macrofaunal taxa, respectively, although taxon dominance differed among stations. Distance-based linear models showed that sediment-related variables and heavy metals were the main environmental factors determining the spatial variability of the assemblages. Macrofauna had only sediment-related variables and heavy metals as major environmental factors, but meiofauna were also influenced by other environmental factors such as sea surface temperature, dissolved oxygen-related variables, and salinity. This study can provide basic ecological data for understanding the spatial distribution of macro-meiofaunal assemblages and aid in the development of marine environmental management strategies on the western south coast of Korea.

Metrics matter: Multiple diversity metrics at different spatial scales are needed to understand species diversity in urban environments.

Worldwide, natural habitats are being replaced by artificial structures due to urbanisation. Planning of such modifications should strive for environmental net gain that benefits biodiversity and ecosystems. Alpha (α) and gamma (γ) diversity are often used to assess 'impact' but are insensitive metrics. We test several diversity measures across two spatial scales to compare species diversity in natural and artificial habitats. We show γ-diversity indicates equivalency in biodiversity between natural and artificial habitats, but natural habitats support greater taxon (α) and functional richness. Within-site ß-diversity was also greater in natural habitats, but among-site ß-diversity was greater in artificial habitats, contradicting the commonly held view that urban ecosystems are more biologically homogenous than natural ecosystems. This study suggests artificial habitats may in fact provide novel habitat for biodiversity, challenges the applicability of the urban homogenisation concept and highlights a significant limitation of using just α-diversity (i.e., multiple metrics are needed and recommended) for assessing environmental net gain and attaining biodiversity conservation goals.

Shoreline sentinels of global change show the consequences of extreme events.

Anthropogenic climate change along with the more frequent extreme weather it prompts, are having direct and indirect effects on distributions and abundance of species with consequence for community structure-especially if habitat providers are lost. Rocky shores have long been recognized as tractable experimental arenas for ecology contributing to theory. They have also emerged as important sentinel systems for tracking climate change responses of marine biodiversity and ecosystems, capitalizing on both historic broadscale surveys and time series. Combining these twin traditions is a powerful approach for better understanding and forecasting climate change impacts. Sustained observing allows extreme events to be detected and explored by in-parallel experimentation.

Applying landscape metrics to species distribution model predictions to characterize internal range structure and associated changes.

Distributional shifts in species ranges provide critical evidence of ecological responses to climate change. Assessments of climate-driven changes typically focus on broad-scale range shifts (e.g. poleward or upward), with ecological consequences at regional and local scales commonly overlooked. While these changes are informative for species presenting continuous geographic ranges, many species have discontinuous distributions-both natural (e.g. mountain or coastal species) or human-induced (e.g. species inhabiting fragmented landscapes)-where within-range changes can be significant. Here, we use an ecosystem engineer species (Sabellaria alveolata) with a naturally fragmented distribution as a case study to assess climate-driven changes in within-range occupancy across its entire global distribution. To this end, we applied landscape ecology metrics to outputs from species distribution modelling (SDM) in a novel unified framework. SDM predicted a 27.5% overall increase in the area of potentially suitable habitat under RCP 4.5 by 2050, which taken in isolation would have led to the classification of the species as a climate change winner. SDM further revealed that the latitudinal range is predicted to shrink because of decreased habitat suitability in the equatorward part of the range, not compensated by a poleward expansion. The use of landscape ecology metrics provided additional insights by identifying regions that are predicted to become increasingly fragmented in the future, potentially increasing extirpation risk by jeopardising metapopulation dynamics. This increased range fragmentation could have dramatic consequences for ecosystem structure and functioning. Importantly, the proposed framework-which brings together SDM and landscape metrics-can be widely used to study currently overlooked climate-driven changes in species internal range structure, without requiring detailed empirical knowledge of the modelled species. This approach represents an important advancement beyond predictive envelope approaches and could reveal itself as paramount for managers whose spatial scale of action usually ranges from local to regional.

Implications of attention and executive functioning weaknesses in youth with Fontan circulation.

Youth with Fontan circulation (Fontan) are at-risk for impairments in attention and executive functioning (EF) due to a confluence of genetic, prenatal, surgical, and medical risk factors. We sought to describe attention and EF in this population, measured via standardized performance-based tests and caregiver rating scales. We then examined how weaknesses in attention and EF were related to outcomes in other neurobehavioral domains, including adaptive behavior and academic achievement. Our sample included 93 youth with Fontan who were referred for neuropsychological evaluations as part of standard clinical care. The cohort as a whole measured between 0.18 to 0.99 standard deviations below normative means across domains of attention, EF, academic achievement, and intellectual ability. In addition, caregiver-reported concerns for attention, EF, anxiety, and depression were elevated, and approximately 0.35 to 0.85 standard deviations above normative means. Lastly, caregiver-reported adaptive behavior measured 0.93 to 1.24 standard deviations below normative values. Academic outcomes were differentially affected by demographic and attention/EF variables, while depression and caregiver-reported EF predicted adaptive behavior. Findings from this study underscore the importance of routine neuropsychological evaluation as part of comprehensive, multidisciplinary care for individuals with Fontan, with the goal of enhancing neurobehavioral and functional outcomes across the lifespan.

A UK consensus algorithm for early treatment modification in newly diagnosed systemic light-chain amyloidosis.

Depth of response is the critical determinant of prognosis in amyloid light-chain (AL) amyloidosis. Here, we aim to identify patients who are unlikely to improve response based on analysis of baseline characteristics and 1-month response. In a multivariate model, difference in involved amyloidogenic and uninvolved serum free light chains (dFLC) at diagnosis (dFLC >400 mg/l, odds ratio [OR] 4.051, p < 0.005) and no response at 1 month (OR 4.787, p < 0.005) were significant predictors of no improvement in response. Only 5% of patients with a dFLC of >400 mg/l and no response at 1 month improved their response (p < 0.005). We suggest that these patients should switch treatment early, subject to their functional status.

The changing dynamics of community care and support in rural Malawi: The impact on Women's health and wellbeing at end of life.

In this critical ethnographic study, we examined women's end of life experiences in Malawi, one of the few countries in the world with a national palliative care policy. Specifically, we explored how women's and their caregivers' experiences were shaped by family and community care, and material needs. Interviews and observations with female clients of a non-governmental organization in rural Central Malawi, and with their caregivers, revealed that community-level support was both precarious and critical. We found three main themes: (1) I stay with them well, (2) we eat together, and (3) everyone is for themselves. The analysis illustrates the centrality of community care, social in/exclusion, and availability of stable food, shelter, medical, and caregiving resources on health and wellbeing at end of life. We provide recommendations to strengthen community care opportunities and women's resource bases.

Diagnosis and management of sleep disorders in Prader-Willi syndrome.

Clinical experience and a growing body of evidence suggest that sleep disturbances are common in people with Prader-Willi syndrome (PWS). PWS is a rare neuroendocrine disorder characterized by early hypotonia and feeding difficulties; developmental delays; endocrinopathies; and behavioral concerns, especially rigidity, anxiety, and behavioral outbursts. PWS is also characterized by decreased resting energy expenditure and transition to hyperphagia and obesity. We propose that, for many people with PWS, clinical diagnosis and management of sleep disorders is an unmet need. We present current information to suggest disordered sleep is a significant burden for individuals with PWS and often overlooked. While central and obstructive sleep apnea are more widely recognized in PWS, other sleep disorders have increasingly gained recognition, including hypersomnia, narcolepsy-like phenotypes, and insomnia. Sleep disorders can impact behavior, cognition, and quality of life and health for individuals with PWS. Our goal is to bring sleep disorders to the forefront of therapeutic intervention for patients with PWS. This paper presents a review of the literature and recommendations for clinical practice based on published research and our clinical experience as sleep specialists, geneticists, psychiatrists, pediatricians, otolaryngologists, and pulmonologists with extensive experience with this patient population. We recommend that management of sleep be considered an integral part of successful medical management of PWS. Further research concerning sleep problems in PWS is urgently needed to develop best practices and work toward a consensus statement for medical management to meet the needs of people with PWS. CITATION: Duis J, Pullen LC, Picone M, et al. Diagnosis and management of sleep disorders in Prader-Willi syndrome. J Clin Sleep Med. 2022;18(6):1687-1696.

On the diversity and distribution of a data deficient habitat in a poorly mapped region: The case of Sabellaria alveolata L. in Ireland.

Data that can be used to monitor biodiversity through time are essential for conservation and management. The reef-forming worm, Sabellaria alveolata (L. 1767) is currently classed as 'Data Deficient' due to an imbalance in the spread of data on its distribution. Little is known about the distribution of this species around Ireland. Using data archaeology, we collated past and present distribution records and discovered that S. alveolata has a discontinuous distribution with large gaps between populations. Many regions lack data and should be targeted for sampling. Biodiversity surveys revealed that S. alveolata supported diverse epibiotic algal communities. Retrograding (declining) reefs supported greater infaunal diversity than prograding (growing) reefs or sand, suggesting that S. alveolata is a dynamic ecosystem engineer that has a lasting legacy effect. Similar research should be carried out for other Data Deficient species, habitats and regions. Such data are invaluable resources for management and conservation.

Carfilzomib or bortezomib in combination with cyclophosphamide and dexamethasone followed by carfilzomib maintenance for patients with multiple myeloma after one prior therapy: results from a multicenter, phase II, randomized, controlled trial (MUKfive).

The proteasome inhibitors, carfilzomib and bortezomib, are widely used to treat myeloma but head-to-head comparisons have produced conflicting results. We compared the activity of these proteasome inhibitors in combination with cyclophosphamide and dexamethasone (KCd vs. VCd) in second-line treatment using fixed duration therapy and evaluated the efficacy of carfilzomib maintenance. MUKfive was a phase II controlled, parallel group trial that randomized patients (2:1) to KCd (n=201) or VCd (n=99); responding patients on carfilzomib were randomized to maintenance carfilzomib (n=69) or no further treatment (n=72). Primary endpoints were: (i) very good partial response (non-inferiority, odds ratio [OR] 0.8) at 24 weeks, and (ii) progression-free survival. More participants achieved a very good partial response or better with carfilzomib than with bortezomib (40.2% vs. 31.9%, OR=1.48, 90% confidence interval [CI]: 0.95, 2.31; non-inferior), with a trend for particular benefit in patients with adverse-risk disease. KCd was associated with higher overall response (partial response or better, 84.0% vs. 68.1%, OR=2.72, 90% CI: 1.62, 4.55, P=0.001). Neuropathy (grade ≥3 or ≥2 with pain) was more common with bortezomib (19.8% vs. 1.5%, P<0.0001), while grade ≥3 cardiac events and hypertension were only reported in the KCd arm (3.6% each). The median progression-free survival in the KCd arm was 11.7 months vs. 10.2 months in the VCd arm (hazard ratio [HR]=0.95, 80% CI: 0.77, 1.18). Carfilzomib maintenance was associated with longer progression-free survival, median 11.9 months vs. 5.6 months for no maintenance (HR 0.59, 80% CI: 0.46-0.77, P=0.0086). When used as fixed duration therapy in first relapase, KCd is at least as effective as VCd, and carfilzomib is an effective maintenance agent. This trial was registered with International Standard Randomised Controlled Trial Number (ISRCTN) identifier: ISRCTN17354232.

Obstructive sleep apnea and early weight loss among adolescents undergoing bariatric surgery.

BACKGROUND: Little is known regarding obstructive sleep apnea's (OSA's) prevalence or the factors related to OSA remission post-metabolic bariatric surgery (MBS) in adolescents. OBJECTIVES: To identify the baseline OSA prevalence in adolescents with severe obesity and examine factors associated with post-MBS OSA remission. SETTING: Tertiary-care children's hospital. METHODS: We conducted a retrospective chart review of 81 patients pre-MBS with OSA assessments done between June 2017 to September 2020 to collect demographic characteristics; co-morbidities; polysomnography (PSG) results, if indicated; and weight data. Chi-square or Mann-Whitney tests compared baseline characteristics and surgical outcomes by pre-MBS OSA status. McNemar's test or t tests assessed differences in baseline characteristics, stratified by remission versus no remission of OSA. RESULTS: The patients were 71% female, had an average age of 16.9 ± 2.0 years, and had a mean body mass index (BMI) of 47.9 ± 7.3 kg/m2. Half (50%) of the patients were Hispanic and 20% had type 2 diabetes. The OSA prevalence, defined as an Obstructive Apnea Hypopnea Index (OAHI) score ≥5, was 54% pre-MBS (n = 44), with 43% having severe OSA (OAHI > 30). Those with OSA were older (17.3 versus 16.4 yr, respectively; P = .05), more likely to be male (79% versus 42%, respectively; P = .022), and had higher baseline weights (142.0 versus 126.4 kg, respectively; P = .001) than those without OSA. Of the 23 patients with a post-MBS PSG result (average 5 mo post MBS), 15 (66%) had remission of OSA. Patients with OSA remission had a lower average pre-MBS BMI (46.0 versus 57.7 kg/m2, respectively; P < .001) and weight (132.9 versus 172.6 kg, respectively; P = .002) but no significant differences in percentage weight loss through 12 months post MBS versus those with continued OSA. CONCLUSION: The OSA prevalence in an adolescent MBS population was higher than that in the general adolescent population with severe obesity. Remission of OSA was correlated with lower pre-MBS BMI and weight, but not weight loss within the first year post-MBS.

A long-term ecological monitoring of subtidal macrozoobenthos around Dokdo waters, East Sea, Korea.

Dokdo's high marine biodiversity has received worldwide attention recently. A total of 578 macrozoobenthos are recorded since the 1960s, but ecology of Dokdo's fauna is unknown. We monitored Dokdo's subtidal macrozoobenthos for 5 years in 2013-17, in the present study. Five stations representing three subtidal habitats were monitored; 1) oceanic plateau, 2) coastal terrace, and 3) island wharf. In total, 13,664 individuals belonging to 141 taxa were recorded during the survey. The number of species and density varied greatly among stations without distinct year-round variation. Faunal compositions significantly differed in time and space, reflecting varied faunal adaptations in a harsh environment. Whilst, temporal stability in faunal assemblages was evidenced for some dominant or cosmopolitan taxa. High spatial heterogeneity reflects site-specific oceanographic conditions. Meantime, sea-surface temperature and wave action were associated with year-round faunal compositions. Overall, the Dokdo's macrozoobenthos significantly contribute to marine biodiversity of the East Sea.

Occurrence and assemblage composition of intertidal non-native species may be influenced by shipping patterns and artificial structures.

Habitat modification coupled with the spread of non-native species (NNS) are among the top threats to marine biodiversity globally. Species are known to be transported to new locations via international shipping and secondarily spread via regional vessels and artificial structures. Rapid Assessment Surveys (RAS) combining quantitative and semi-quantitative methods compared NNS richness and assemblage composition on intertidal natural rocky shores and artificial structures in harbours in different regions along the south coast of England. Quantitative data showed that artificial habitats supported higher richness than natural habitats, while semi-quantitative data found no difference in richness among habitat types. This result was attributed to additional species found in rock pools during searches of complex microhabitats in natural habitats. Assemblages on artificial structures differed among regions, with regions and harbours with greater numbers of vessels supporting greater richness. Results highlight the importance of shipping and artificial structures for NNS introduction and spread.

Transmissible spongiform encephalopathy in goats: is PrP rapid test sensitivity affected by genotype?

Transmissible spongiform encephalopathy (TSE) surveillance in goats relies on tests initially approved for cattle, subsequently assessed for sheep, and approval extrapolated for use in "small ruminants." The current EU-approved immunodetection tests employ antibodies against various epitopes of the prion protein PrPSc, which is encoded by the host PRNP gene. The caprine PRNP gene is polymorphic, mostly at codons different from the ovine PRNP. The EU goat population is much more heterogeneous than the sheep population, with more PRNP-related polymorphisms, and with marked breed-related differences. The ability of the current tests to detect disease-specific PrPSc generated against these different genetic backgrounds is currently assumed, rather than proven. We examined whether common polymorphisms within the goat PRNP gene might have any adverse effect on the relative performance of EU-approved rapid tests. The sample panel comprised goats from the UK, Cyprus, France, and Italy, with either experimental or naturally acquired scrapie at both the preclinical and/or unknown and clinical stages of disease. Test sensitivity was significantly lower and more variable when compared using samples from animals that were preclinical or of unknown status. However, all of the rapid tests included in our study were able to correctly identify all samples from animals in the clinical stages of disease, apart from samples from animals polymorphic for serine or aspartic acid at codon 146, in which the performance of the Bio-Rad tests was profoundly affected. Our data show that some polymorphisms may adversely affect one test and not another, as well as underline the dangers of extrapolating from other species.

ATS Core Curriculum 2020. Pediatric Pulmonary Medicine.

The American Thoracic Society Core Curriculum updates clinicians annually in adult and pediatric pulmonary disease, medical critical care, and sleep medicine, in a 3- to 4-year recurring cycle of topics. These topics will be presented at the 2020 International Conference. Below is the pediatric pulmonary medicine core, including pediatric hypoxemic respiratory failure; modalities in noninvasive management of chronic respiratory failure in childhood; surgical and nonsurgical management of congenital lung malformations; an update on smoke inhalation lung injury; an update on vaporizers, e-cigarettes, and other electronic delivery systems; pulmonary complications of sarcoidosis; pulmonary complications of congenital heart disease; and updates on the management of congenital diaphragmatic hernia.

Global-scale species distributions predict temperature-related changes in species composition of rocky shore communities in Britain.

Changes in rocky shore community composition as responses to climatic fluctuations and anthropogenic warming can be shown by changes in average species thermal affinities. In this study, we derived thermal affinities for European Atlantic rocky intertidal species by matching their known distributions to patterns in average annual sea surface temperature. Average thermal affinities (the Community Temperature Index, CTI) tracked patterns in sea surface temperature from Portugal to Norway, but CTI for communities of macroalgae and plant species changed less than those composed of animal species. This reduced response was in line with the expectation that communities with a smaller range of thermal affinities among species would change less in composition along thermal gradients and over time. Local-scale patterns in CTI over wave exposure gradients suggested that canopy macroalgae allow species with ranges centred in cooler than local temperatures ('cold-affinity') to persist in otherwise too-warm conditions. In annual surveys of rocky shores, communities of animal species in Shetland showed a shift in dominance towards warm-affinity species ('thermophilization') with local warming from 1980 to 2018 but the community of plant and macroalgal species did not. From 2002 to 2018, communities in southwest Britain showed the reverse trend in CTI: declining average thermal affinities over a period of modest temperature decline. Despite the cooling, trends in species abundance were in line with the general mechanism of direction and magnitude of long-term trends depending on the difference between species thermal affinities and local temperatures. Cold-affinity species increased during cooling and warm-affinity ones decreased. The consistency of responses across different communities and with general expectations based on species thermal characteristics suggests strong predictive accuracy of responses of community composition to anthropogenic warming.

Identifying niche and fitness dissimilarities in invaded marine macroalgal canopies within the context of contemporary coexistence theory.

Contemporary coexistence theory provides a framework for predicting invasiveness and impact of Invasive Non-Native Species (INNS) by incorporating differences in niche and fitness between INNS and co-occurring native species. The widespread invasive kelp Undaria pinnatifida is considered a high-risk INNS, although a robust evidence base regarding its invasiveness and impact is lacking in many regions. Invaded macroalgal canopies at nine coastal sites in the southwest UK were studied over three years to discern whether Undaria is coexisting or competing with native canopy-forming species across different habitat types. Spatial, temporal and depth-related trends in species distributions and abundance were recorded within intertidal and subtidal rocky reef as well as on marina pontoons. A primary succession experiment also examined competitive interactions between species. In rocky reef habitats, Undaria had lower fitness compared to long-lived native perennials, but was able to coexist due to niche dissimilarity between species. In contrast, Undaria was likely to be competing with short-lived native annuals on rocky reef due to large niche overlap and similar fitness. In marina habitats, Undaria dominated over all other canopy formers due to low niche diversification and higher fitness. Generalisations on INNS impact cannot be made across habitats or species, without considering many abiotic factors and biotic interactions.