Managing climate refugia for freshwater fishes under an expanding human footprint.

Within the context of climate adaptation, the concept of climate refugia has emerged as a framework for addressing future threats to freshwater fish populations. We evaluated recent climate-refugia management associated with water use and landscape modification by comparing efforts in the US states of Oregon and Massachusetts, for which there are contrasting resource use patterns. Using these examples, we discuss tools and principles that can be applied more broadly. Although many early efforts to identify climate refugia have focused on water temperature, substantial gains in evaluating other factors and processes regulating climate refugia (eg stream flow, groundwater availability) are facilitating refined mapping of refugia and assessment of their ecological value. Major challenges remain for incorporating climate refugia into water-quality standards, evaluating trade-offs among policy options, addressing multiple species' needs, and planning for uncertainty. However, with a procedurally transparent and conceptually sound framework to build upon, recent efforts have revealed a promising path forward.

Climate-change refugia: biodiversity in the slow lane.

Climate-change adaptation focuses on conducting and translating research to minimize the dire impacts of anthropogenic climate change, including threats to biodiversity and human welfare. One adaptation strategy is to focus conservation on climate-change refugia (that is, areas relatively buffered from contemporary climate change over time that enable persistence of valued physical, ecological, and sociocultural resources). In this Special Issue, recent methodological and conceptual advances in refugia science will be highlighted. Advances in this emerging subdiscipline are improving scientific understanding and conservation in the face of climate change by considering scale and ecosystem dynamics, and looking beyond climate exposure to sensitivity and adaptive capacity. We propose considering refugia in the context of a multifaceted, long-term, network-based approach, as temporal and spatial gradients of ecological persistence that can act as "slow lanes" rather than areas of stasis. After years of discussion confined primarily to the scientific literature, researchers and resource managers are now working together to put refugia conservation into practice.

Insight into the phenotype of infants with Pompe disease identified by newborn screening with the common c.-32-13T>G "late-onset" GAA variant.

OBJECTIVE: Newborn screening (NBS) has led to early diagnosis and early initiation of treatment for infantile onset Pompe Disease (IOPD). However, guidelines for management of late onset Pompe disease (LOPD) via NBS, especially with the IVS c.-32-13T>G are not clear. This IVS variant is noted in 68-90% cases with LOPD and has been presumed to result in "adult" disease in compound heterozygosity, with a few cases with earlier onset and a mild to no phenotype in homozygosity. Our study evaluates newborns with LOPD having IVS variant with a diligent multidisciplinary approach to determine if they have an early presentation. METHODS: Seven children with LOPD identified by NBS with IVS variant (3 compound heterozygous, and 4 homozygous) were evaluated with clinical, biochemical (CK, AST, ALT, and urinary Glc4), cardiac evaluation, physical therapy (PT), occupational, and speech/language therapy. RESULTS: All seven patients demonstrated motor involvement by age 6months; the three patients with c.-32-13 T>G variant in compound heterozygosity had symptoms as neonates. Patients with c.-32-13 T>G variant in compound heterozygosity had more involvement with persistent hyperCKemia, elevated AST and ALT, swallowing difficulties, limb-girdle weakness, delayed motor milestones, and were initiated on ERT. The patients with c.-32-13T>G variant in homozygosity had normal laboratory parameters, and presented with very subtle yet LOPD specific signs, identified only by meticulous assessments. CONCLUSION: This patient cohort represents the first carefully phenotyped cohort of infants with LOPD with the "late-onset" GAA variant c.-32-13T>G detected by NBS in the USA. It emphasizes not only the opportunity for early detection of skeletal and other muscle involvement in infants with c.-32-13T>G variant but also a high probability of overlooking or underestimating the significance of clinically present and detectable features. It can thus serve as a valuable contribution in the development of evaluation and treatment algorithms for infants with LOPD.

Motor Function Test Reliability During the NeuroNEXT Spinal Muscular Atrophy Infant Biomarker Study.

BACKGROUND: The NeuroNEXT SMA Infant Biomarker Study, a two year, longitudinal, multi-center study of infants with SMA type 1 and healthy infants, presented a unique opportunity to assess multi-site rater reliability on three infant motor function tests (MFTs) commonly used to assess infants with SMA type 1. OBJECTIVE: To determine the effect of prospective MFT rater training and the effect of rater experience on inter-rater and intra-rater reliability for the Test of Infant Motor Performance Screening Items (TIMPSI), the Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP-INTEND) and the Alberta Infant Motor Scale (AIMS). METHODS: Training was conducted utilizing a novel set of motor function test (MFT) videos to optimize accurate MFT administration and reliability for the study duration. Inter- and intra-rater reliability of scoring for the TIMPSI and inter-rater reliability of scoring for the CHOP INTEND and the AIMS was assessed using intraclass correlation coefficients (ICC). Effect of rater experience on reliability was examined using ICC. Agreement with 'expert' consensus scores was examined using Pearson's correlation coefficients. RESULTS: Inter-rater reliability on all MFTs was good to excellent. Intra-rater reliability for the primary MFT, the TIMPSI, was excellent for the study duration. Agreement with 'expert' consensus was within predetermined limits (≥85%) after training. Evaluator experience with SMA and MFTs did not affect reliability. CONCLUSIONS: Reliability of scores across evaluators was demonstrated for all three study MFTs and scores were reproducible on repeated administration. Evaluator experience had no effect on reliability.

Seeking and Accepting: U.S. Clergy Theological and Moral Perspectives Informing Decision Making at the End of Life.

BACKGROUND: People with serious illness frequently rely on religion/spirituality to cope with their diagnosis, with potentially positive and negative consequences. Clergy are uniquely positioned to help patients consider medical decisions at or near the end of life within a religious/spiritual framework. OBJECTIVE: We aimed to examine clergy knowledge of end-of-life (EOL) care and beliefs about the role of faith in EOL decision making for patients with serious illness. DESIGN: Key informant interviews, focus groups, and survey. SETTING/SUBJECTS: A purposive sample of 35 active clergy in five U.S. states as part of the National Clergy End-of-Life Project. MEASUREMENT: We assessed participant knowledge of and desire for further education about EOL care. We transcribed interviews and focus groups for the purpose of qualitative analysis. RESULTS: Clergy had poor knowledge of EOL care; 75% desired more EOL training. Qualitative analysis revealed a theological framework for decision making in serious illness that balances seeking life and accepting death. Clergy viewed comfort-focused treatments as consistent with their faith traditions' views of a good death. They employed a moral framework to determine the appropriateness of EOL decisions, which weighs the impact of multiple factors and upholds the importance of God-given free will. They viewed EOL care choices to be the primary prerogative of patients and families. Clergy described ambivalence about and a passive approach to counseling congregants about decision making despite having defined beliefs regarding EOL care. CONCLUSIONS: Poor knowledge of EOL care may lead clergy to passively enable congregants with serious illness to pursue potentially nonbeneficial treatments that are associated with increased suffering.

How Community Clergy Provide Spiritual Care: Toward a Conceptual Framework for Clergy End-of-Life Education.

CONTEXT: Community-based clergy are highly engaged in helping terminally ill patients address spiritual concerns at the end of life (EOL). Despite playing a central role in EOL care, clergy report feeling ill-equipped to spiritually support patients in this context. Significant gaps exist in understanding how clergy beliefs and practices influence EOL care. OBJECTIVES: The objective of this study was to propose a conceptual framework to guide EOL educational programming for community-based clergy. METHODS: This was a qualitative, descriptive study. Clergy from varying spiritual backgrounds, geographical locations in the U.S., and race/ethnicities were recruited and asked about optimal spiritual care provided to patients at the EOL. Interviews were audio taped, transcribed, and analyzed following principles of grounded theory. A final set of themes and subthemes were identified through an iterative process of constant comparison. Participants also completed a survey regarding experiences ministering to the terminally ill. RESULTS: A total of 35 clergy participated in 14 individual interviews and two focus groups. Primary themes included Patient Struggles at EOL and Clergy Professional Identity in Ministering to the Terminally Ill. Patient Struggles at EOL focused on existential questions, practical concerns, and difficult emotions. Clergy Professional Identity in Ministering to the Terminally Ill was characterized by descriptions of Who Clergy Are ("Being"), What Clergy Do ("Doing"), and What Clergy Believe ("Believing"). "Being" was reflected primarily by manifestations of presence; "Doing" by subthemes of religious activities, spiritual support, meeting practical needs, and mistakes to avoid; "Believing" by subthemes of having a relationship with God, nurturing virtues, and eternal life. Survey results were congruent with interview and focus group findings. CONCLUSION: A conceptual framework informed by clergy perspectives of optimal spiritual care can guide EOL educational programming for clergy.

Clergy Views on a Good Versus a Poor Death: Ministry to the Terminally Ill.

BACKGROUND: Clergy are often important sources of guidance for patients and family members making medical decisions at the end-of-life (EOL). Previous research revealed spiritual support by religious communities led to more aggressive care at the EOL, particularly among minority patients. Understanding this phenomenon is important to help address disparities in EOL care. OBJECTIVE: The study objective was to explore and describe clergy perspectives regarding "good" versus "poor" death within the participant's spiritual tradition. METHODS: This was a qualitative, descriptive study. Community clergy from various spiritual backgrounds, geographical locations within the United States, and races/ethnicities were recruited. Participants included 35 clergy who participated in one-on-one interviews (N = 14) and two focus groups (N = 21). Semistructured interviews explored clergy viewpoints on factors related to a "good death." Principles of grounded theory were used to identify a final set of themes and subthemes. RESULTS: A good death was characterized by wholeness and certainty and emphasized being in relationship with God. Conversely, a "poor death" was characterized by separation, doubt, and isolation. Clergy identified four primary determinants of good versus poor death: dignity, preparedness, physical suffering, and community. Participants expressed appreciation for contextual factors that affect the death experience; some described a "middle death," or one that integrates both positive and negative elements. Location of death was not viewed as a significant contributing factor. CONCLUSIONS: Understanding clergy perspectives regarding quality of death can provide important insights to help improve EOL care, particularly for patients highly engaged with faith communities. These findings can inform initiatives to foster productive relationships between clergy, clinicians, and congregants and reduce health disparities.

Patterns of Freshwater Species Richness, Endemism, and Vulnerability in California.

The ranges and abundances of species that depend on freshwater habitats are declining worldwide. Efforts to counteract those trends are often hampered by a lack of information about species distribution and conservation status and are often strongly biased toward a few well-studied groups. We identified the 3,906 vascular plants, macroinvertebrates, and vertebrates native to California, USA, that depend on fresh water for at least one stage of their life history. We evaluated the conservation status for these taxa using existing government and non-governmental organization assessments (e.g., endangered species act, NatureServe), created a spatial database of locality observations or distribution information from ~400 data sources, and mapped patterns of richness, endemism, and vulnerability. Although nearly half of all taxa with conservation status (n = 1,939) are vulnerable to extinction, only 114 (6%) of those vulnerable taxa have a legal mandate for protection in the form of formal inclusion on a state or federal endangered species list. Endemic taxa are at greater risk than non-endemics, with 90% of the 927 endemic taxa vulnerable to extinction. Records with spatial data were available for a total of 2,276 species (61%). The patterns of species richness differ depending on the taxonomic group analyzed, but are similar across taxonomic level. No particular taxonomic group represents an umbrella for all species, but hotspots of high richness for listed species cover 40% of the hotspots for all other species and 58% of the hotspots for vulnerable freshwater species. By mapping freshwater species hotspots we show locations that represent the top priority for conservation action in the state. This study identifies opportunities to fill gaps in the evaluation of conservation status for freshwater taxa in California, to address the lack of occurrence information for nearly 40% of freshwater taxa and nearly 40% of watersheds in the state, and to implement adequate protections for freshwater taxa where they are currently lacking.

Potential factors affecting survival differ by run-timing and location: linear mixed-effects models of Pacific salmonids (Oncorhynchus spp.) in the Klamath River, California.

Understanding factors influencing survival of Pacific salmonids (Oncorhynchus spp.) is essential to species conservation, because drivers of mortality can vary over multiple spatial and temporal scales. Although recent studies have evaluated the effects of climate, habitat quality, or resource management (e.g., hatchery operations) on salmonid recruitment and survival, a failure to look at multiple factors simultaneously leaves open questions about the relative importance of different factors. We analyzed the relationship between ten factors and survival (1980-2007) of four populations of salmonids with distinct life histories from two adjacent watersheds (Salmon and Scott rivers) in the Klamath River basin, California. The factors were ocean abundance, ocean harvest, hatchery releases, hatchery returns, Pacific Decadal Oscillation, North Pacific Gyre Oscillation, El Niño Southern Oscillation, snow depth, flow, and watershed disturbance. Permutation tests and linear mixed-effects models tested effects of factors on survival of each taxon. Potential factors affecting survival differed among taxa and between locations. Fall Chinook salmon O. tshawytscha survival trends appeared to be driven partially or entirely by hatchery practices. Trends in three taxa (Salmon River spring Chinook salmon, Scott River fall Chinook salmon; Salmon River summer steelhead trout O. mykiss) were also likely driven by factors subject to climatic forcing (ocean abundance, summer flow). Our findings underscore the importance of multiple factors in simultaneously driving population trends in widespread species such as anadromous salmonids. They also show that the suite of factors may differ among different taxa in the same location as well as among populations of the same taxa in different watersheds. In the Klamath basin, hatchery practices need to be reevaluated to protect wild salmonids.

Climate change vulnerability of native and alien freshwater fishes of California: a systematic assessment approach.

Freshwater fishes are highly vulnerable to human-caused climate change. Because quantitative data on status and trends are unavailable for most fish species, a systematic assessment approach that incorporates expert knowledge was developed to determine status and future vulnerability to climate change of freshwater fishes in California, USA. The method uses expert knowledge, supported by literature reviews of status and biology of the fishes, to score ten metrics for both (1) current status of each species (baseline vulnerability to extinction) and (2) likely future impacts of climate change (vulnerability to extinction). Baseline and climate change vulnerability scores were derived for 121 native and 43 alien fish species. The two scores were highly correlated and were concordant among different scorers. Native species had both greater baseline and greater climate change vulnerability than did alien species. Fifty percent of California's native fish fauna was assessed as having critical or high baseline vulnerability to extinction whereas all alien species were classified as being less or least vulnerable. For vulnerability to climate change, 82% of native species were classified as highly vulnerable, compared with only 19% for aliens. Predicted climate change effects on freshwater environments will dramatically change the fish fauna of California. Most native fishes will suffer population declines and become more restricted in their distributions; some will likely be driven to extinction. Fishes requiring cold water (<22°C) are particularly likely to go extinct. In contrast, most alien fishes will thrive, with some species increasing in abundance and range. However, a few alien species will likewise be negatively affected through loss of aquatic habitats during severe droughts and physiologically stressful conditions present in most waterways during summer. Our method has high utility for predicting vulnerability to climate change of diverse fish species. It should be useful for setting conservation priorities in many different regions.

Lack of achievement of a full score on the childhood myositis assessment scale by healthy four-year-olds and those recovering from juvenile dermatomyositis.

OBJECTIVE: To test 4-year-olds, using 14 maneuvers of the Childhood Myositis Assessment Scale (CMAS), comparing healthy children with those with juvenile dermatomyositis (DM). METHODS: Healthy 4-year-olds (n = 28) completed the CMAS. Their scores were compared with children with juvenile DM (n = 18) who had a muscle Disease Activity Score (DAS-M) of 0. RESULTS: The healthy children achieved a mean ± SD CMAS score of 46.6 ± 2.3 (interquartile range 46-47). There were no significant differences between boys and girls, and the scores were not significantly associated with height or weight. The greatest variation involved items that assessed endurance. Item 1, neck raise, yielded a mean ± SD score of 28.2 ± 19.3 seconds, with a mean ± SD CMAS score of 2.5 ± 0.9 (maximum score 5). Item 3, leg lift, yielded a mean ± SD score of 55.5 ± 37.3 seconds, with a mean ± SD CMAS score of 3.1 ± 1.1 (maximum score 5). Item 5, sit-ups maneuver, yielded a mean ± SD score of 5.3 ± 1.1 sit-ups. Almost identical data were obtained for the 18 treated children with juvenile DM who had normal strength on the DAS-M. CONCLUSION: Healthy children ages 4 years do not achieve the total CMAS score of 52 attained by older children. Both boys and girls were remarkably consistent, with a mean CMAS score of 46.6. Children ages 4 years with juvenile DM with a DAS-M of 0 also achieved a CMAS score of 46.6. We conclude that half of 4-year-old children achieve a mean CMAS score of 46 or 47, not a total CMAS score of 52, suggesting that weakness may be overdiagnosed in 4-year-olds with an inflammatory myopathy.