Effectiveness of population-based recovery actions for threatened southern mountain caribou.

Habitat loss is affecting many species, including the southern mountain caribou (Rangifer tarandus caribou) population in western North America. Over the last half century, this threatened caribou population's range and abundance have dramatically contracted. An integrated population model was used to analyze 51 years (1973-2023) of demographic data from 40 southern mountain caribou subpopulations to assess the effectiveness of population-based recovery actions at increasing population growth. Reducing potential limiting factors on threatened caribou populations offered a rare opportunity to identify the causes of decline and assess methods of recovery. Southern mountain caribou abundance declined by 51% between 1991 and 2023, and 37% of subpopulations were functionally extirpated. Wolf reduction was the only recovery action that consistently increased population growth when applied in isolation, and combinations of wolf reductions with maternal penning or supplemental feeding provided rapid growth but were applied to only four subpopulations. As of 2023, recovery actions have increased the abundance of southern mountain caribou by 52%, compared to a simulation with no interventions. When predation pressure was reduced, rapid population growth was observed, even under contemporary climate change and high levels of habitat loss. Unless predation is reduced, caribou subpopulations will continue to be extirpated well before habitat conservation and restoration can become effective.

A case of mevalonate kinase deficiency, neonatal Sweet syndrome, and inflammatory bowel disease.

Mevalonate kinase deficiency is a group of rare metabolic autoinflammatory disorders that present with recurrent fevers, abdominal pain, arthralgias, adenopathy, and a variety of cutaneous manifestations. The skin findings may mimic cellulitis, erythema elevatum diutinum, IgA vasculitis, and Sweet syndrome, and there is often a morbilliform or urticarial rash and aphthous stomatitis. Mevalonate kinase deficiency is one of the identified monogenic variants that can cause very early onset inflammatory bowel disease (IBD). We present a rare case of a patient with mevalonate kinase deficiency, neonatal Sweet syndrome, and infantile-onset IBD, who has been successfully treated with canakinumab therapy.

LOGAN-CV: A Prospective Study of a Multifaceted Intervention Targeting United States Clinicians to Improve Guideline-Based Management of Lipid-Lowering Therapy.

INTRODUCTION: The 2018 American Heart Association (AHA)/American College of Cardiology (ACC)/Multisociety blood cholesterol guidelines recommend clinicians consider adding non-statin therapy for patients with very high-risk (VHR) atherosclerotic cardiovascular disease (ASCVD) and low-density lipoprotein cholesterol (LDL-C) ≥ 70 mg/dl while receiving maximally tolerated statins. However, according to a recent study, only 17.1% of patients with established ASCVD received appropriate lipid-lowering therapy (LLT) intensification. Here, we describe the design of a prospective, 12-month study (LOGAN-CV) evaluating a multifaceted site-level intervention to enhance clinicians' adherence to guidelines to improve LDL-C levels for patients with VHR ASCVD. METHODS: Clinicians from up to ten research sites are eligible if they care for adult patients with ASCVD. Interventions include educational modules, a cloud-based performance platform providing clinicians a tailored summary of their LDL-C management performance, newsletters, periodic peer-to-peer calls, and pre- and post-intervention surveys evaluating knowledge, attitudes, and beliefs around LDL-C management, with additional interventions for clinicians demonstrating a lower readiness to make treatment decisions based on guideline recommendations. Patients with VHR ASCVD, defined as having recent myocardial infarction and LDL-C ≥ 70 mg/dl despite statin treatment, will be included in the study. Patient data will be collected from electronic medical records from baseline (clinician enrollment) through the 12-month intervention. The study started in October 2022, with anticipated completion in March 2024. PLANNED OUTCOMES: The change in proportion of patients with LDL-C < 70 mg/dl achieved at any time during the 12-month intervention (primary); LLT intensification, changes in guideline-aligned LDL-C testing and LLT titration over 12 months, and change in overall clinicians' knowledge, attitudes, and beliefs are key outcomes of interest. The LOGAN-CV study addresses a critical unmet need in LDL-C control in patients with VHR ASCVD and evaluates the effect of a multifaceted intervention targeting clinicians to improve their adherence to guidelines and consequently improve clinical outcomes for patients.

An Update on Patient-Reported Outcomes in Interventional Radiology: The Future Measure of Our Success.

The field of interventional radiology continues to expand rapidly, offering an increasing range of alternatives to open surgical procedures. This minimally invasive imaging-guided approach promises faster recovery times and a theoretically improved patient experience; however, there is limited evidence documenting that these promises are actually delivered. Patient-reported outcomes are a way of collecting data on the patient experience increasingly used in clinical trials and the provision of surgical services and informing clinical practice across a range of elective procedures. Currently underutilised in interventional radiology, patient-reported outcomes have the potential to significantly impact how we deliver care by allowing evaluation of the perceived benefit derived by a patient after undergoing a procedure and to permit comparison with more invasive open procedures from the patient perspective.

Persistent, Pruritic, Reddish-Brown Papules and Plaques: An Atypical Presentation of Adult-Onset Still's Disease.

Adult-onset Still's disease (AOSD) is a systemic inflammatory condition characterized by recurrent fevers and a dermatologic eruption. The eruption is classically described as migratory and evanescent, composed of salmon-pink to erythematous macules, patches, and papules. However, a much rarer skin rash can also be seen in the setting of AOSD. This eruption has a different morphology, appearing as fixed, extremely pruritic papules and plaques. The histology of this atypical form of AOSD is distinct from that of the more common evanescent eruption. Management of AOSD is multi-faceted, aimed at controlling both the acute and chronic phases. Increased awareness of this more uncommon cutaneous presentation of AOSD is vital so that the appropriate diagnosis can be rendered. Herein, the authors describe an atypical presentation of AOSD in a 44-year-old male patient who presented with persistent, pruritic, brownish papules and plaques on the trunk and extremities.

Congenital cutaneous candidiasis in a full-term neonate.

We present a case of a full-term neonate born with respiratory distress and a widespread erythematous rash, who was found to have congenital cutaneous candidiasis (CCC). The significance of this report is to contribute to the pre-existing literature on the rarity of CCC, but also to share a case of a patient who was successfully treated conservatively with topical antifungal agents only.

Xanthogranulomatous Pyelonephritis: A Narrative Review with Current Perspectives on Diagnostic Imaging and Management, Including Interventional Radiology Techniques.

Xanthogranulomatous Pyelonephritis (XGP) is a rare, chronic granulomatous inflammatory condition thought to arise secondary to a combination of obstruction, recurrent bacterial infection and an incomplete immune response resulting in focal or diffuse renal destruction. This destruction may be profound with the potential to infiltrate surrounding tissues and viscera. The imaging features of XGP can be ambiguous, mimicking malignancy, tuberculosis (TB) and malakoplakia earning the title of "the great imitator". Computed tomography (CT) is the mainstay of XGP diagnosis and staging, accurately quantifying the stone burden and staging the renal destruction, including the extent of extra-renal spread. Although some cases in children have been successfully treated with antibiotics alone, nephrectomy remains the most common treatment for XGP in adults. The specific management strategy needs to be tailored to individual patients given the potential constellation of renal and extrarenal abnormalities. Although XGP has classically required open nephrectomy, laparoscopic nephrectomy has an increasing role to play arising from the advancement in laparoscopic skills, technique and instruments. Nephron-sparing partial nephrectomy may be considered in the focal form. Interventional radiology techniques most often play a supportive role, eg, in the initial drainage of associated abscesses, but have rarely achieved renal salvage. This narrative review seeks to synthesise the existing literature and summarise the radiological approach and interventional radiology management situated in a clinical context.

Evaluation of a Clinical Platform to Promote Chronic Disease Management.

Self-reported data suggest American adults with multiple chronic conditions account for 25.7% to 60% of the population. Despite emphasis on information technology to improve quality in health care, data addressing outcomes of clinically focused, provider-oriented dashboards are limited. To explore integrating performance platforms into clinical care, the authors designed a platform-based intervention to address 2 prevalent chronic conditions with significant long-term burden. This study used a performance platform to enhance clinicians' management of patients with diabetes and osteoporosis. Descriptive statistics were used to summarize patients' surveys and quality metrics, and to analyze clinicians' knowledge, attitudes, and beliefs in the pre and post time frames. The frequency of screening for osteoporosis in women improved post intervention (40% vs. 44%, P < 0.0001), whereas other quality metrics did not. Clinician respondents were primarily physicians (82%), white (73%), internal medicine specialists (58%), with an average of 18 years' experience, and nearly equally male and female. Their percent of correctly answered knowledge questions increased slightly in the postintervention phase for osteoporosis and hypoglycemia (0.53 and 1.74, respectively); however, results were not statistically significant (P > 0.4). Post intervention, clinicians reported that their attitudes and beliefs regarding disease management had changed in the past 6 months in a positive direction. Although few outcomes studied changed over time, results suggest that performance platforms may have a role to play in managing chronic conditions. However, their efficacy must continue to be evaluated in order to improve understanding of optimal approaches to integrating technology into patient care.

Multidisciplinary Tumor Boards in Dermatologic Surgery.

BACKGROUND: Recent advances in diagnostic and therapeutic modalities have made oncologic care ever more challenging, and multidisciplinary tumor boards (MTBs) are increasingly being used as a forum to discuss and coordinate care for complex oncology patients. Literature on the use of MTBs specific to cutaneous oncology and dermatologic surgery remains limited. OBJECTIVE: To share our experiences with cutaneous oncology MTBs at the University of Vermont Medical Center (UVMMC). METHODS: We describe the formation, timing, participation, clinical discussion, case follow-up, and coordination of care of our MTBs. RESULTS: A log of all cases discussed at cutaneous oncology MTBs from August 2018 to August 2019 is presented as an example. Five specific cases are described in further detail to demonstrate critical components of multidisciplinary care. CONCLUSION: The MTBs at UVMMC has created a collaborative environment for providers in multiple specialties to jointly formulate and coordinate optimal treatment plans for difficult cases, particularly when treatment guidelines do not exist or are insufficient. Furthermore, MTBs can serve as an educational forum for all participants.

Atypical blastomycosis in a pregnant woman.

Blastomycosis is a rare fungal infection that frequently involves the skin. Atypical presentations are important to identify, especially in pregnant patients, to initiate appropriate therapy and prevent complications. Uniquely, we describe a case of atypical blastomycosis that presented with painful cutaneous abscesses in a pregnant patient, with dissemination to the central nervous system. The case was successfully treated with liposomal amphotericin B transitioned to voriconazole after delivery without complications for the patient or fetus.

Ipilimumab-associated halo-like inflammatory reactions around nevi during therapy for metastatic melanoma.

Ipilimumab is an immune-modulating drug that is being used today for various cancers including metastatic malignant melanoma. Owing to its mechanism of action, several adverse events have been reported, including some affecting skin. In this work, we report a novel display of multiple ipilimumab-associated halo lichenoid reactions surrounding benign nevi during treatment of metastatic melanoma. A patient underwent treatment with ipilimumab for treatment of stage IIIC melanoma at our center and was monitored for progress and adverse events throughout treatment. During treatment with ipilimumab, the patient clinically developed multiple halo lichenoid reactions surrounding previously present nevi, which histopathologically showed a lichenoid interface dermatitis associated with the mildly atypical nevi and ill-formed granulomata within the infiltrate. Therefore, ipilimumab may be associated with halo lichenoid reactions surrounding benign nevi and this adverse effect should be added to the various dermatologic reactions that patients can develop while being treated with this agent.

Recent progress of probing correlated electron states by point contact spectroscopy.

We review recent progress in point contact spectroscopy (PCS) to extract spectroscopic information out of correlated electron materials, with the emphasis on non-superconducting states. PCS has been used to detect bosonic excitations in normal metals, where signatures (e.g. phonons) are usually less than 1% of the measured conductance. In the superconducting state, point contact Andreev reflection (PCAR) has been widely used to study properties of the superconducting gap in various superconductors. It has been well-recognized that the corresponding conductance can be accurately fitted by the Blonder-Tinkham-Klapwijk (BTK) theory in which the AR occurring near the point contact junction is modeled by three parameters; the superconducting gap, the quasiparticle scattering rate, and a dimensionless parameter, Z, describing the strength of the potential barrier at the junction. AR can be as large as 100% of the background conductance, and only arises in the case of superconductors. In the last decade, there have been more and more experimental results suggesting that the point contact conductance could reveal new features associated with the unusual single electron dynamics in non-superconducting states, shedding a new light on exploring the nature of the competing phases in correlated materials. To correctly interpret these new features, it is crucial to re-examine the modeling of the point contact junctions, the formalism used to describe the single electron dynamics particularly in point contact spectroscopy, and the physical quantity that should be computed to understand the conductance. We will summarize the theories for point contact spectroscopy developed from different approaches and highlight these conceptual differences distinguishing point contact spectroscopy from tunneling-based probes. Moreover, we will show how the Schwinger-Kadanoff-Baym-Keldysh (SKBK) formalism together with the appropriate modeling of the nano-scale point contacts randomly distributed across the junction leads to the conclusion that the point contact conductance is proportional to the effective density of states, a physical quantity that can be computed if the electron self energy is known. The experimental data on iron based superconductors and heavy fermion compounds will be analyzed in this framework. These recent developments have extended the applicability of point contact spectroscopy to correlated materials, which will help us achieve a deeper understanding of the single electron dynamics in strongly correlated systems.

Topological surface states interacting with bulk excitations in the Kondo insulator SmB6 revealed via planar tunneling spectroscopy.

Samarium hexaboride (SmB6), a well-known Kondo insulator in which the insulating bulk arises from strong electron correlations, has recently attracted great attention owing to increasing evidence for its topological nature, thereby harboring protected surface states. However, corroborative spectroscopic evidence is still lacking, unlike in the weakly correlated counterparts, including Bi2Se3 Here, we report results from planar tunneling that unveil the detailed spectroscopic properties of SmB6 The tunneling conductance obtained on the (001) and (011) single crystal surfaces reveals linear density of states as expected for two and one Dirac cone(s), respectively. Quite remarkably, it is found that these topological states are not protected completely within the bulk hybridization gap. A phenomenological model of the tunneling process invoking interaction of the surface states with bulk excitations (spin excitons), as predicted by a recent theory, provides a consistent explanation for all of the observed features. Our spectroscopic study supports and explains the proposed picture of the incompletely protected surface states in this topological Kondo insulator SmB6.

Disseminated cutaneous Curvularia infection in an immunocompromised host; diagnostic challenges and experience with voriconazole.

An increasing spectrum and number of opportunistic fungal pathogens have been reported to cause disease in humans over the past decade. Disseminated phaeohyphomycoses caused by rare dematiaceous molds in immunocompromised patients have a high mortality rate and are increasingly reported in the literature. Early diagnosis of disseminated phaehyphomycosis is critical especially in neutropenic patients but can be hindered by the low sensitivity of fungal blood cultures and low clinical suspicion. Cutaneous manifestations are often the earliest sign of disease and conducting a thorough skin exam in febrile neutropenic patients can lead to more rapid diagnosis and initiation of treatment. PCR amplification and sequencing of mold RNA extracted from paraffin-embedded tissue can be useful for diagnosing rare fungal infections when negative fungal cultures preclude morphologic diagnosis. Effective treatment for disseminated phaehyphomycosis is lacking and there is a need to report experiences with the use of newer antifungals.

Theory of point contact spectroscopy in correlated materials.

We developed a microscopic theory for the point-contact conductance between a metallic electrode and a strongly correlated material using the nonequilibrium Schwinger-Kadanoff-Baym-Keldysh formalism. We explicitly show that, in the classical limit, contact size shorter than the scattering length of the system, the microscopic model can be reduced to an effective model with transfer matrix elements that conserve in-plane momentum. We found that the conductance dI/dV is proportional to the effective density of states, that is, the integrated single-particle spectral function A(ω = eV) over the whole Brillouin zone. From this conclusion, we are able to establish the conditions under which a non-Fermi liquid metal exhibits a zero-bias peak in the conductance. This finding is discussed in the context of recent point-contact spectroscopy on the iron pnictides and chalcogenides, which has exhibited a zero-bias conductance peak.