Inflammatory Myopathy: When Electromyography and Autoantibodies Don't Help the Diagnosis.

Inflammatory myopathies (IM) are the most treatable myopathies. Necrotizing autoimmune myositis is a distinct clinicopathologic entity that starts either acutely or subacutely. Autoimmunity is essencial in the pathogenesis of myositis and autoantibodies may be present in more than 50% of patients. We present the case of a 73-year-old man with elevated levels of CK and aldolase, and proximal symmetric muscle weakness and weight loss. The etiological investigation revealed, via muscle biopsy, a necrotizing autoimmune myositis, even though the majority of usual autoantibodies and electromyography were negative. The case demonstrates the importance of combining the patient's symptoms, neurological examination, and analytical changes to corroborate the suspicion of myopathy.

Real-Life Data on Readmissions of Worsening Heart Failure Outpatients in a Heart Failure Clinic.

Introduction Recurrent hospitalizations for worsening heart failure (WHF) represent a major global public health concern, resulting in significant individual morbimortality and socioeconomic costs. This real-life study aimed to determine the rate and predictors of readmission for WHF in a cohort of outpatients with chronic heart failure (CHF) followed in a heart failure clinic (HFC) at a university hospital. Methods We conducted a longitudinal, observational, and retrospective study of all consecutive CHF patients seen at the HFC of the São Francisco Xavier Hospital, Lisbon, by a multidisciplinary team in 2019. The patients were followed for one year and were on optimized therapy. The inclusion criteria for the study were patients who had been hospitalized and subsequently discharged at least three months prior to their enrollment. Patient demographics, heart failure (HF) characterization, comorbidities, pharmacological treatment, treatments of decompensated HF in the day hospital (DH), hospitalizations for WHF, and death were recorded. We applied logistic regression analysis to assess predictors of hospital readmission for HF. Results A total of 351 patients were included: 90 patients (26%) had WHF requiring treatment with intravenous diuretics in the DH; 45 patients (mean age: 79.1 ± 9.0 years) were readmitted for decompensated HF within one year (12.8%) with no gender difference, while 87.2% of the patients (mean age: 74.9 ± 12.1 years) were never readmitted. Readmitted patients were significantly older than those who were not (p=0.031). Additionally, they had a higher New York Heart Association (NYHA) functional classification (p<.001), were on a higher daily dose of furosemide (p=0.008) at the time of the inclusion visit, were more frequently affected by the chronic obstructive pulmonary disease (COPD) (p=0.004); had been treated more often in the DH for WHF (p<.001) and had a higher mortality rate (p<.001) at one year. Conclusions This study aimed to determine WHF patient readmission rates and predictors. According to our results, a higher NYHA class, the need for treatment in the DH for WHF, a daily dose of furosemide equal to or greater than 80 mg, and COPD were predictors of readmission for WHF. CHF patients continue to experience WHF and recurrent hospitalizations despite therapeutic advances and close follow-up in the HFC with the multidisciplinary team. Besides COPD, the HF readmission risk factors found were mainly related to advanced disease. Furthermore, the structured and multidisciplinary approach of our disease management program likely contributed to our relatively low rate of readmissions.

A single dose of COVID-19 vaccine induces a strong T cell and B cell response in healthcare professionals recovered from SARS-CoV-2 infection.

A broad understanding on how SARS-CoV-2 infection and vaccination mobilize the immune system is necessary to find the best predictors of long-term protection and identify individuals that would benefit from additional vaccine doses. This study aims to understand the effect of a single dose of Pfizer-BioNTech BNT162b2 COVID-19 vaccine, in individuals recovered from SARS-CoV-2 infection, on circulating CD4+ T follicular helper (Tfh)-cells, Spike-specific T-cells and IgG/IgA antibodies. For that, peripheral blood samples from 50 healthcare professionals, recovered from SARS-CoV-2 infection, collected immediately before (T1) and 15 days after (T2) vaccine administration, were used to analyze the frequency and numbers of Tfh-cells and their subsets, serum titers of SARS-CoV-2-specific antibodies, and SARS-CoV-2-specific T-cells. Six months after infection (T1), 96% of recovered participants presented either IgG or T-cells specific for Spike, however, Spike-specific T-cells were missing in 16% of them. These individuals presented lower levels of Spike-specific IgG (T1 and T2), IgA (T1), and Spike-specific T-cells (T2). Vaccination increased the percentage of participants reactive for Spike-specific T-cells (from 64 to 98%), IgG (from 90 to 100%) and IgA (from 48 to 98%). It also mobilized circulating Tfh-cells, increasing their frequency and activation, and promoting Tfh17 polarization, restoring the decreased numbers of Tfh-cells (especially Tfh17) observed in recovered participants. Interestingly, Tfh percentage correlated with Spike-specific IgG levels. Our data showed that a single dose of vaccine efficiently restored Spike-specific T-cells, and IgG and IgA antibodies. Mobilization of Tfh-cells, and their correlation with IgG levels, suggest that vaccination induced a functional Tfh cell response.

Pneumorrhachis Secondary to an Infected Sacral Decubitus Ulcer.

Pneumorrhachis (PR) is a rare phenomenon, which consists in the presence of air in the spinal canal. There are various aetiologies, being the most common traumatic, non-traumatic and iatrogenic. The diagnosis is primarily done through radiographic findings and it is necessary to understand the mechanism behind its origin. PR secondary to decubitus ulcer (DU) infection is rare. PR is associated with great morbidity and mortality. In selected cases, surgical intervention may be necessary. A 67-year-old woman, dependent, was admitted to the emergency room (ER) and diagnosed with an infected sacral DU, later discharged with antibiotics. She was readmitted to the ER two weeks later, with prostration and fever. On examination, she scored five points on the Glasgow coma scale, had bilateral Babinsky sign and a deep sacral ulcer with bone exposure. A cranial computerized tomography (CT) demonstrated "high cervical and endochannel emphysema in the upper slope of the cervical segment" and the CT scan of the spine showed "endochannel air along the cervical-dorsal and lumbar rachis in an epidural location and inside the dural sac (evoking laceration of the dura mater) (…) and densification of the sacrococcygeal soft tissues (diagnosis of PR secondary to DU infection)". Broad-spectrum antibiotics were started and the patient was evaluated by General Surgery, which described a large sacral ulcer with signs of the previous debridement and bone exposure, with no indication for surgical debridement, only for chemical debridement. Despite all the measures instituted, the patient died in the ER.

Hyponatremia: A Tip of the Iceberg in the Late Diagnosis of Tuberous Sclerosis Associated With Lymphangioleiomyomatosis.

Tuberous sclerosis (TS) is a rare, autosomal dominant, multisystem genetic disease that causes multiple benign tumors in the brain and other vital organs. Rarely, it can be associated with lymphangioleiomyomatosis (LMA) that is characterized by the proliferation of immature smooth muscle cells in the walls of the airways, venules, and lymphatic vessels in the lung. Here, we present the case of a 44-year-old intellectually disabled woman with a history of marked polydipsia who presented to the emergency department with persistent vomiting. She was hemodynamically stable and did not have any fever. The analytical study showed severe and symptomatic hyponatremia. On physical examination, multiple skin lesions compatible with angiofibromas were noted and the diagnosis of TS was made (confirmed with the genetic study). The multiorgan study documented the presence of multiple cystic images in the lung parenchyma associated with LMA. The aim of this case report is to highlight the importance of targeting cutaneous lesions for a rapid diagnosis of this pathology and to identify the etiology of a severe (symptomatic) ionic disorder and referral to a multidisciplinary team.

Quantification and phenotypic characterization of peripheral blood Vδ1 + T cells in chronic lymphocytic leukemia and monoclonal B cell lymphocytosis.

BACKGROUND: Vδ1+ T cells, a subset of γδ T cells, are responsible for innate-like immune responses. Recently, an anti-tumor function mediated by MHC-unrestricted recognition of lipid and stress molecules, has also been described in these cells. This study aimed to quantify and phenotypically characterize circulating Vδ1+ T cells in B cell Chronic Lymphocytic Leukemia (CLL) and Monoclonal B cell lymphocytosis (MBL). METHODS: This study enrolled 58 individuals distributed in five groups: Binet B and C CLL (n = 9), Binet A CLL (n = 26), High count-MBL (n = 10), Low count-MBL (n = 5), and a control group (n = 8). The phenotypic characterization of Vδ1+ T cells, as well as the other T cell subpopulations (CD4+ , CD8+ , CD4+ /CD8+ , and Vδ1- γδT cells), were assessed by flow cytometry, evaluating the frequency of each subset expressing CD27, CD69, and cytoplasmic granzyme B. RESULTS: We observed an increasing percentage of Vδ1+ T cells belonging to CD27- compartment from controls to advanced stages of the disease, which was accompanied by an increasing percentage of these cells expressing granzyme B, a phenotypic pattern that was also observed in the other T cell subpopulations under study since earlier stages of the disease. Moreover, a striking expansion of Vδ1+ T cells in Binet B and C CLL was observed. CONCLUSIONS: These experiment findings point to an expansion of CD27- Vδ1+ T cells with a cytotoxic profile, from controls to advanced stages of the disease, which points to a role of Vδ1+ T cells in the host's anti-tumor responses against clonal B-cells in MBL and CLL. © 2018 Clinical Cytometry Society.

Influence of Aloe vera on water absorption and enzymatic in vitro degradation of alginate hydrogel films.

This study investigates the influence of Aloe vera on water absorption and the in vitro degradation rate of Aloe vera-Ca-alginate hydrogel films, for wound healing and drug delivery applications. The influence of A. vera content (5%, 15% and 25%, v/v) on water absorption was evaluated by the incubation of the films into a 0.1 M HCl solution (pH 1.0), acetate buffer (pH 5.5) and simulated body fluid solution (pH 7.4) during 24h. Results show that the water absorption is significantly higher for films containing high A. vera contents (15% and 25%), while no significant differences are observed between the alginate neat film and the film with 5% of A. vera. The in vitro enzymatic degradation tests indicate that an increase in the A. vera content significantly enhances the degradation rate of the films. Control films, incubated in a simulated body fluid solution without enzymes, are resistant to the hydrolytic degradation, exhibiting reduced weight loss and maintaining its structural integrity. Results also show that the water absorption and the in vitro degradation rate of the films can be tailored by changing the A. vera content.

Development of novel alginate based hydrogel films for wound healing applications.

Alginate and Aloe vera are natural materials widely investigated and used in the biomedical field. In this research work, thin hydrogel films composed by alginate and Aloe vera gel in different proportions (95:5, 85:15 and 75:25, v/v) were prepared and characterized. The films were evaluated regarding the light transmission behavior, contact angle measurements, and chemical, thermal and mechanical properties. These thin hydrogel films, prepared by crosslinking reaction using 5% calcium chloride solution, were also investigated relatively to their water solubility and swelling behavior. Results showed that Aloe vera improved the transparency of the films, as well their thermal stability. The developed films present adequate mechanical properties for skin applications, while the solubility studies demonstrated the insolubility of the films after 24h of immersion in distilled water. The water absorption and swelling behavior of these films were greatly improved by the increase in Aloe vera proportion.

Particulate matter and health risk under a changing climate: assessment for Portugal.

The potential impacts of climate-induced changes in air pollution levels and its impacts on population health were investigated. The IPCC scenario (SRES A2) was used to analyse the effects of climate on future PM10 concentrations over Portugal and their impact on short-term population exposure and mortality. The air quality modelling system has been applied with high spatial resolution looking on climate changes at regional scale. To quantify health impacts related to air pollution changes, the WHO methodology for health impact assessment was implemented. The results point to 8% increase of premature mortality attributed to future PM10 levels in Portugal. The pollution episodes with daily average PM10 concentration above the current legislated value (50 µg·m⁻³) would be responsible for 81% of attributable cases. The absolute number of deaths attributable to PM10 under future climate emphasizes the importance of indirect effects of climate change on human health.

Cultural circuits of climate change in U.K. broadsheet newspapers, 1985-2003.

This article argues for a cultural perspective to be brought to bear on studies of climate change risk perception. Developing the "circuit of culture" model, the article maintains that the producers and consumers of media texts are jointly engaged in dynamic, meaning-making activities that are context-specific and that change over time. A critical discourse analysis of climate change based on a database of newspaper reports from three U.K. broadsheet papers over the period 1985-2003 is presented. This empirical study identifies three distinct circuits of climate change-1985-1990, 1991-1996, 1997-2003-which are characterized by different framings of risks associated with climate change. The article concludes that there is evidence of social learning as actors build on their experiences in relation to climate change science and policy making. Two important factors in shaping the U.K.'s broadsheet newspapers' discourse on "dangerous" climate change emerge as the agency of top political figures and the dominant ideological standpoints in different newspapers.