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Background: The factors necessitating the need for referrals for in-person evaluations by a dermatologist are not adequately understood and have not been studied using automated text mining so far. The objective of this study was to compare the prevalence of required in-person dermatologist care in the presence or absence of certain clinical features. Methods: Observational cross-sectional study of 11,661 teledermatology reports made from February 2017 to March 2020. Results: The need for dermoscopy was associated with a 348% increase in the possibility of referral for in-person dermatologist evaluations (prevalence ratio [PR]: 4.48, 95% confidence interval [CI]: 4.17-4.82). Infectious diseases were associated with a 64% lower possibility of referral (PR: 0.36, 95% CI: 0.30-0.43). Discussion: Some lesions and poorly documented cases are challenging to assess remotely. This study presents a different approach to research more detailed data from teledermatology reports, using text mining, and points out the risk magnitude for demanding dermatologic in-person care of which feature analyzed. As limitations, the variables related to lesion location, size, and extension were not analyzed and the dictionaries used were originally in Brazilian Portuguese. Conclusions: Teledermatology seems sufficient for the management of 75% of clinical cases, especially acute in young patients with inflammatory or infectious lesions. Referrals for in-person dermatologist consultations were not only strongly associated with the need for dermoscopy, but also for therapeutic reasons like surgical procedures, phototherapy, and the use of some systemic medications.
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Dermatologia , Dermatopatias , Telemedicina , Humanos , Dermatologia/métodos , Estudos Transversais , Dermatologistas , Telemedicina/métodos , Encaminhamento e Consulta , Dermatopatias/diagnóstico , Dermatopatias/epidemiologia , Dermatopatias/terapiaRESUMO
INTRODUCTION: Known risk factors for multiple sclerosis (MS) include smoking, a low vitamin D status, obesity, and EBV, while the inflammatory feature of the disease strongly suggests the presence of additional infectious agents. The association between use of antibiotics and MS risk that could shed light on these factors is still undetermined. We aimed to evaluate the association between antibiotics and MS risk, in the Emilia-Romagna region (RER), Italy. METHODS: All adult patients with MS seen at any RER MS center (2015-2017) were eligible. For each of the 877 patients included, clinical information was collected and matched to 5 controls (RER residents) (n = 4,205) based on age, sex, place of residence, and index year. Information on antibiotic prescription was obtained through the linkage with the RER drug prescription database. RESULTS: Exposure to any antibiotic 3 years prior to the index year was associated with an increased MS risk (OR = 1.52; 95% CI = 1.29-1.79). Similar results were found for different classes. No dose-response effect was found. DISCUSSION/CONCLUSIONS: Our results suggest an association between the use of antibiotics and MS risk in RER population. However, further epidemiological studies should be done with information on early life and lifestyle factors.
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Antibacterianos , Esclerose Múltipla , Adulto , Antibacterianos/efeitos adversos , Estudos de Casos e Controles , Humanos , Itália/epidemiologia , Esclerose Múltipla/tratamento farmacológico , Esclerose Múltipla/epidemiologia , Obesidade , Fatores de RiscoRESUMO
INTRODUCTION: Patients with early onset dementia (EOD), defined as dementia with symptom onset at age <65, frequently present with atypical syndromes. However, the epidemiology of different EOD presentations, including variants of Alzheimer's disease (AD) and frontotemporal dementia (FTD), has never been investigated all together in a population-based study. Epidemiologic data of all-cause EOD are also scarce. METHODS: We investigated EOD epidemiology by identifying patients with EOD seen in the extended network of dementia services of the Modena province, Northern Italy (≈700,000 inhabitants) from 2006 to 2019. RESULTS: In the population age 30 to 64, incidence was 13.2 per 100,000/year, based on 160 new cases from January 2016 to June 2019, and prevalence 74.3 per 100,000 on June 30, 2019. The most frequent phenotypes were the amnestic variant of AD and behavioral variant of FTD. DISCUSSION: EOD affects a significant number of people. Amnestic AD is the most frequent clinical presentation in this understudied segment of the dementia population.
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Demência/epidemiologia , Adulto , Fatores Etários , Idade de Início , Idoso , Doença de Alzheimer/epidemiologia , Amnésia/epidemiologia , Feminino , Demência Frontotemporal/epidemiologia , Humanos , Incidência , Itália/epidemiologia , Masculino , Pessoa de Meia-Idade , Prevalência , Estudos Retrospectivos , Fatores SexuaisRESUMO
BACKGROUND: Interpersonal violence has increased as a health concern, especially in psychiatry practice, over the last decades. Nevertheless, most patients with stable mental disorders do not present an increased risk of violence, and mental disorder is not a necessary or sufficient cause of violent behaviours. People with mental disorders endorse more often a number of risk factors for violence that could confound this association, such as young age and male gender. The aim of this study was to investigate the effect of age, gender, and diagnosis on reported levels of interpersonal violence in a sample of people with severe mental illness. METHODS: The sample was composed of 160 inpatients: 73 with a psychosis within the schizophrenia spectrum, 53 with a mood disorder and 34 with a personality disorder. All patients enrolled in the study were assessed for experiences of victimization and perpetration of interpersonal violence using the Karolinska Interpersonal Violence Scale interview. Demographic variables were also collected. RESULTS: Both violence perpetration and victimization negatively correlated with age. Compared to males, females were exposed to higher degree of victimization in childhood and adulthood, whereas males were more involved in the perpetration of violence in childhood. Personality disorders were associated with higher levels of interpersonal violence, both perpetration and victimization; an interaction effect of gender and diagnosis was also observed for violence perpetration in adulthood. Distinct patterns of interpersonal violence did emerge for the diagnostic groups with mood disorder showing a victimization pattern, personality disorders a perpetration pattern and psychoses less defined patterns. CONCLUSIONS: The main finding is that psychotic disorders, mood disorders and personality disorders have different patterns of violent experiences interacting with age and gender. This study offers a better understanding of how gender and age could affect violent behaviours. Moreover, study findings may increase the comprehension of the reason why some mental disorders, compared to others, are more associated with the risk of victimization or perpetration of violence. These patterns could have pathophysiological or pathoplastic meaning addressing clinical and diagnostic trajectories and they could interact with other intervening risk factors.
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Vítimas de Crime , Sinais (Psicologia) , Transtornos Psicóticos , Violência/estatística & dados numéricos , Adulto , Distribuição por Idade , Criança , Feminino , Humanos , Relações Interpessoais , Masculino , Fatores de Risco , Distribuição por SexoRESUMO
Pregnancy in sickle cell disease is a problem due to the adverse outcomes related to the disease. Research into the role of chemokines in sickle cell disease is available, but studies investigating the disease in pregnancy are scarce. Our data show the chemokine profiles of pregnant women with sickle cell disease compared with control groups. There were no differences in MCP-1 level among the groups, but IL-8 and MIG were likely related with disease activity. In addition, levels of IP-10 were higher in pregnant women with sickle cell disease and, interestingly, RANTES levels were higher in normal pregnancy when compared to pregnancy in sickle cell disease. More studies should be encouraged to fully elucidate chemokine activity during pregnancy in sickle cell disease.
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Anemia Falciforme/sangue , Quimiocinas/sangue , Complicações Hematológicas na Gravidez/sangue , Adolescente , Adulto , Feminino , Humanos , Gravidez , Estudos ProspectivosRESUMO
Social functioning (SF) has mainly been studied in major psychoses in relation to symptom severity, but other factors may interfere with the achievement of a functional remission. The aim of this study is to explore interpersonal violence (IV), service engagement (SE), and social network (SN), together with demographics, as predictors of SF in a sample of subjects with severe mental illness (SMI). Consecutive adult inpatients were evaluated using self-report and clinician-rated questionnaires. Findings suggest that IV, SE, SN, male sex, and illness duration explained 39.1% of SF variance in people affected by SMI. IV was the strongest predictor, followed by sex and duration of illness. Lifetime expression of violence is a stronger predictor than lifetime exposure to violence. Positive SE and SN were found to predict SF, whereas age was not associated. This study underlines the need of other non-symptom-related variables for the comprehension of SF in mental disorders.
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Serviços Comunitários de Saúde Mental , Relações Interpessoais , Transtornos Mentais/psicologia , Ajustamento Social , Rede Social , Violência/psicologia , Adulto , Serviços Comunitários de Saúde Mental/tendências , Estudos Transversais , Feminino , Humanos , Masculino , Transtornos Mentais/diagnóstico , Pessoa de Meia-Idade , Aceitação pelo Paciente de Cuidados de Saúde/psicologia , Autorrelato , Violência/tendênciasRESUMO
Osteoarthritis (OA) is the most prevalent rheumatic disease and is a leading cause of decreased quality of life (QoL). The OA Quality of Life questionnaire (OAQoL) is an OA-specific patient-reported outcome measures. The aim of this study was to translate and validate the original UK English version of the Osteoarthritis Quality of Life (OAQoL) questionnaire into European Portuguese. The translation of the questionnaire was carried out according to a dual panel methodology (bilingual panel followed by lay panel). This was followed by cognitive debriefing interviews (CDIs) with OA patients to assess comprehension and relevance of the translated questionnaire. Finally, a validation survey was conducted to assess its psychometric properties. The Portuguese OAQoL, a comparator scale (the Nottingham Health Profile-NHP) as well as questions relating to demographic and disease information were administered to OA patients. A sub-sample of patients also completed the Portuguese OAQoL two weeks later, to assess test-retest reliability. The internal consistency, construct validity and known group validity (according to perceived OA severity) of the scale was also assessed. Both the bilingual and lay panels consisted of five individuals and no major difficulties relating to the translation process were identified. A total of ten patients with OA participated in the CDIs. The mean time to complete the questionnaire was 5 min. These interviews revealed that the Portuguese version of the OAQoL was clear, relevant and easy to complete. Finally, 53 OA patients (44 females; mean age of 67.6 years) completed the validation survey. Cronbach's alpha coefficient was 0.87, demonstrating high internal consistency. Test-retest reliability, assessed by Spearman's rank correlation coefficient, was 0.86. Moderate correlations were found with the majority of the NHP sections, providing evidence of construct validity. Significant differences in OAQoL scores were found between patients who differed according to their perceived OA severity, providing evidence of known group validity. The Portuguese version of the OAQoL is a valid and reliable questionnaire that can be used to assess QoL in OA, both in clinical practice and for research purposes.
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Osteoartrite/fisiopatologia , Qualidade de Vida , Idoso , Assistência à Saúde Culturalmente Competente , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Osteoartrite/psicologia , Medidas de Resultados Relatados pelo Paciente , Portugal , Índice de Gravidade de Doença , Inquéritos e Questionários , TraduçõesRESUMO
BACKGROUND: Cardiorespiratory fitness (CRF) and physical activity (PA) seem to have positive effects on academic achievement (AA), although in most studies, objective methods of assessment have not been used. AIM: To examine the associations of CRF and PA with AA. SUBJECTS AND METHODS: A sample of 640 youngsters (10-18 years) participated in this cross-sectional study. CRF was estimated with the 20-metre shuttle run test and PA was measured using accelerometers. School grades were used to describe AA using mathematics and native language final grades. The relationship between CRF and PA with AA was analysed using ANCOVA. RESULTS: Results show a significant effect of CRF levels on native language grades, after adjustment for confounders [F(3,414) = 3,99 p < 0.05], but not for mathematics [F(3,360) = 0.95 p > 0.05]. No significant effects of PA levels on AA were found. CONCLUSIONS: CRF is associated with moderate-to-vigorous PA (MVPA) and higher levels of CRF may influence AA. CRF is positively correlated with MVPA, suggesting that higher amounts of MVPA might improve CRF and indirectly influence AA. Since CRF is dependent on higher intensities of PA, we can suggest a focus on implementing improvement strategies to increase the intensity of physical education lessons.
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Sucesso Acadêmico , Aptidão Cardiorrespiratória , Exercício Físico , Adolescente , Criança , Estudos Transversais , Feminino , Humanos , Masculino , PortugalAssuntos
Adenosina Desaminase/genética , Antirreumáticos/uso terapêutico , Infliximab/uso terapêutico , Peptídeos e Proteínas de Sinalização Intercelular/genética , Mutação , Poliarterite Nodosa/tratamento farmacológico , Poliarterite Nodosa/genética , Adulto , Aspirina/análogos & derivados , Aspirina/uso terapêutico , Feminino , Humanos , Lisina/análogos & derivados , Lisina/uso terapêutico , Poliarterite Nodosa/diagnóstico , Prednisolona/uso terapêuticoRESUMO
Reported alterations in T(reg) cells from type 1 diabetes (T1D) patients led us to a revision of their phenotypical features compared with controls. A fine cytometric analysis was designed for their characterization, using a panel of markers including FOXP3, CTLA4, glucocorticoid-induced TNFR family related (GITR) and CD127. The frequency of peripheral CD4(+)CD25(hi) T(reg) cells was similar between samples. However, the yield of sorted T(reg) cells was significantly lower in patients than in controls. When comparing the T(reg)-cell phenotype between samples, the only difference concerned the expression of GITR. A significant decrease of GITR(+) cells and GITR mean fluorescence intensity within the T(reg)-cell population, and to a lesser extent in the effector population, was observed in T1D compared with controls. Moreover, GITR expression was analyzed in several conditions of T-cell activation and differences were only observed in T1D T(reg) cells versus controls when responding to sub-optimal stimulation, that is, soluble anti-CD3 or medium alone but not in the presence of anti-CD3-/anti-CD28-coated beads. However, expanded T1D T(reg)-cell-mediated suppression was as efficient as that mediated by their control counterparts, showing no association between their regulatory capacity and the reduced GITR. Our results show a higher susceptibility to apoptosis in patients' versus controls' T(reg) cells, suggesting that GITR is a T(reg)-cell marker that would be primarily involved in T(reg)-cell survival rather than in their suppressor function.
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Diabetes Mellitus Tipo 1/imunologia , Proteína Relacionada a TNFR Induzida por Glucocorticoide/metabolismo , Linfócitos T Reguladores/imunologia , Adulto , Antígenos CD4/metabolismo , Separação Celular , Sobrevivência Celular , Feminino , Citometria de Fluxo , Proteína Relacionada a TNFR Induzida por Glucocorticoide/genética , Humanos , Imunofenotipagem , Subunidade alfa de Receptor de Interleucina-2/metabolismo , Masculino , Pessoa de Meia-Idade , Adulto JovemRESUMO
Understanding how emotional processing modulates learning and memory is crucial for the treatment of neuropsychiatric disorders characterized by emotional memory dysfunction. We investigate how human medial temporal lobe (MTL) neurons support emotional memory by recording spiking activity from the hippocampus, amygdala, and entorhinal cortex during encoding and recognition sessions of an emotional memory task in patients with pharmaco-resistant epilepsy. Our findings reveal distinct representations for both remembered compared to forgotten and emotional compared to neutral scenes in single units and MTL population spiking activity. Additionally, we demonstrate that a distributed network of human MTL neurons exhibiting mixed selectivity on a single-unit level collectively processes emotion and memory as a network, with a small percentage of neurons responding conjointly to emotion and memory. Analyzing spiking activity enables a detailed understanding of the neurophysiological mechanisms underlying emotional memory and could provide insights into how emotion alters memory during healthy and maladaptive learning.
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Emoções , Memória , Neurônios , Humanos , Emoções/fisiologia , Neurônios/fisiologia , Memória/fisiologia , Masculino , Adulto , Feminino , Lobo Temporal/fisiologia , Tonsila do Cerebelo/fisiologia , Córtex Entorrinal/fisiologia , Hipocampo/fisiologia , Adulto JovemRESUMO
Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disorder which may affect the gastrointestinal system. Half of the patients with SLE experience gastrointestinal symptoms, with the most common being nausea, vomiting, anorexia, and abdominal pain. Mesenteric vasculitis is a severe and rare complication of SLE and one of the most frequent causes of severe acute abdominal pain. The authors present a case of a 57-year-old woman with SLE who was diagnosed with necrotizing mesenteric vasculitis following a urinary septic shock. The patient was treated with high-dose corticosteroid therapy and cyclophosphamide, with resolution of the clinical picture.
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Gastroenteropatias , Lúpus Eritematoso Sistêmico , Lesões do Sistema Vascular , Vasculite , Feminino , Humanos , Pessoa de Meia-Idade , Lúpus Eritematoso Sistêmico/complicações , Vasculite/complicações , Gastroenteropatias/complicações , Ciclofosfamida/uso terapêutico , Dor Abdominal/complicações , Lesões do Sistema Vascular/complicaçõesRESUMO
Dementia is a major neurologic syndrome characterized by severe cognitive decline, and it has a detrimental impact on overall physical health, leading to conditions such as frailty, changes in gait, and fall risk. Depending on whether symptoms occur before or after the age of 65, it can be classified as early-onset (EOD) or late-onset (LOD) dementia. The present study is aimed at investigating the role of cardiovascular factors on EOD and LOD risk in an Italian population. Using a case-control study design, EOD and LOD cases were recruited at the Modena Cognitive Neurology Centers in 2016-2019. Controls were recruited among caregivers of all the dementia cases. Information about their demographics, lifestyles, and medical history were collected through a tailored questionnaire. We used the odds ratio (OR) and 95% confidence interval (CI) to estimate the EOD and LOD risk associated with the investigated factors after adjusting for potential confounders. Of the final 146 participants, 58 were diagnosed with EOD, 34 with LOD, and 54 were controls. According to their medical history, atrial fibrillation was associated with increased disease risk (ORs 1.90; 95% CI 0.32-11.28, and 3.64; 95% CI 0.32-41.39 for EOD and LOD, respectively). Dyslipidemia and diabetes showed a positive association with EOD, while the association was negative for LOD. We could not evaluate the association between myocardial infarction and EOD, while increased risk was observed for LOD. No clear association emerged for carotid artery stenosis or valvular heart disease. In this study, despite the limited number of exposed subjects and the high imprecision of the estimates, we found positive associations between cardiovascular disease, particularly dyslipidemia, diabetes, and atrial fibrillation, and EOD.
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Fibrilação Atrial , Demência , Humanos , Estudos de Casos e Controles , Itália/epidemiologia , Masculino , Feminino , Fibrilação Atrial/epidemiologia , Fibrilação Atrial/etiologia , Demência/epidemiologia , Demência/etiologia , Idoso , Fatores de Risco , Pessoa de Meia-Idade , Idoso de 80 Anos ou mais , Doenças Cardiovasculares/epidemiologia , Doenças Cardiovasculares/etiologiaRESUMO
Autoreactive T cells, responsible for the destruction of pancreatic ß cells in type 1 diabetes, are known to have a skewed TCR repertoire in the NOD mouse. To define the autoreactive T cell repertoire in human diabetes, we searched for intraislet monoclonal expansions from a recent onset in human pancreas to then trace them down to the patient's peripheral blood and spleen. Islet infiltration was diverse, but five monoclonal TCR ß-chain variable expansions were detected for Vß1, Vß7, Vß11, Vß17, and Vß22 families. To identify any sequence bias in the TCRs from intrapancreatic T cells, we analyzed 139 different CDR3 sequences. We observed amino acid preferences in the NDN region that suggested a skewed TCR repertoire within infiltrating T cells. The monoclonal expanded TCR sequences contained amino acid combinations that fit the observed bias. Using these CDR3 sequences as a marker, we traced some of these expansions in the spleen. There, we identified a Vß22 monoclonal expansion with identical CDR3 sequence to that found in the islets within a polyclonal TCR ß-chain variable repertoire. The same Vß22 TCR was detected in the patient's PBMCs, making a cross talk between the pancreas and spleen that was reflected in peripheral blood evident. No other pancreatic monoclonal expansions were found in peripheral blood or the spleen, suggesting that the Vß22 clone may have expanded or accumulated in situ by an autoantigen present in both the spleen and pancreas. Thus, the patient's spleen might be contributing to disease perpetuation by expanding or retaining some autoreactive T cells.
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Diabetes Mellitus Tipo 1/imunologia , Ilhotas Pancreáticas/imunologia , Ilhotas Pancreáticas/metabolismo , Receptores de Antígenos de Linfócitos T/biossíntese , Baço/imunologia , Baço/metabolismo , Subpopulações de Linfócitos T/imunologia , Subpopulações de Linfócitos T/metabolismo , Sequência de Aminoácidos , Doenças Autoimunes/imunologia , Doenças Autoimunes/metabolismo , Doenças Autoimunes/patologia , Movimento Celular/imunologia , Regiões Determinantes de Complementaridade/biossíntese , Regiões Determinantes de Complementaridade/sangue , Diabetes Mellitus Tipo 1/metabolismo , Diabetes Mellitus Tipo 1/patologia , Feminino , Humanos , Região Variável de Imunoglobulina/biossíntese , Região Variável de Imunoglobulina/sangue , Ilhotas Pancreáticas/patologia , Ativação Linfocitária/imunologia , Dados de Sequência Molecular , Receptores de Antígenos de Linfócitos T/sangue , Baço/patologia , Subpopulações de Linfócitos T/patologia , Adulto JovemRESUMO
AIM: The aim of this study is to investigate the relation between perpetration and victimization of violence with gender and age at onset of mental disorders in patients with severe mental disorders. METHODS: 216 patients were recruited and evaluated with the Italian version of the Karolinska Interpersonal Violence Scale. RESULTS: We found higher levels of victimization of violence in women than men and more violence in patients with lower age at onset. DISCUSSION: Gender and age at onset impact on violence in the adult life in a sample of patients with severe mental disorders. CONCLUSIONS: Our findings confirmed the need of an early diagnosis and gender-tailored management of interpersonal violence.
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Vítimas de Crime , Transtornos Mentais , Masculino , Adulto , Humanos , Feminino , Idade de Início , Violência , Transtornos Mentais/complicações , Transtornos Mentais/epidemiologiaRESUMO
Arthritis in the paediatric population is the hallmark of many rheumatic inflammatory diseases, as well as other cutaneous, infectious, or neoplastic conditions. It can be quite devastating, whereby prompt recognition and treatment of these disorders are essential. However, arthritis can sometimes be mistaken for other cutaneous or genetic conditions leading to misdiagnosis and overtreatment. Pachydermodactyly is a rare and benign form of digital fibromatosis, usually manifested by swelling of the proximal interphalangeal joints of both hands, mimicking arthritis. The authors report a case of a 12-year-old boy with a one-year history of painless swelling of the proximal interphalangeal joints of both hands that was referred to the Paediatric Rheumatology department due to the suspicion of juvenile idiopathic arthritis. The diagnostic work-up was unremarkable, and the patient remained asymptomatic over an 18-month follow-up period. A diagnosis of pachydermodactyly was assumed and no treatment was introduced, given the benign nature of the disorder and absence of symptoms. Therefore, it was possible to safely discharge the patient from the Paediatric Rheumatology clinic.
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Artrite Juvenil , Dermatite , Fibroma , Dermatopatias , Masculino , Humanos , Criança , Artrite Juvenil/diagnóstico , Pele , Fibroma/diagnóstico , Articulações dos Dedos/diagnóstico por imagemRESUMO
Industrial metal-rich sludge can improve soil properties, but it is potentially toxic to soils and adjacent aquatic systems. The soil-sludge-water interactions influence metals bioavailability over time, a phenomenon mostly regulated by the still debatable "sludge physical protection" or "sludge delayed release" hypotheses. The present study aimed to investigate: (1) whether sludge increases soil aggregate stability against slaking, (2) which hypothesis mostly regulates metal release from soils to water and (3) the ecotoxicity of the metals released during soil slaking for aquatic organisms. Under a realistic field scale, soils amended with an industrial sludge or spiked with equivalent metal solutions (of Cr, Cu, Ni, Zn) were collected over three months to test soil aggregate stability, the ecotoxicity of the slaking water and metal contents in soil and water. The "sludge physical protection" was verified for all metals, though for Cu the "sludge delayed release" hypothesis appears plausible after three months. Soil amendment with sludge did not lead to effects on the growth of the microalga Raphidocelis subcapitata, contrarily to the observed for the metal-spiked soil. Criteria regulating soils sludge-amendment management should thus include doses not hazardous to biota, and not only metal threshold levels.
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Metais Pesados , Poluentes do Solo , Solo , Metais Pesados/toxicidade , Metais Pesados/análise , Esgotos , Água , Poluentes do Solo/toxicidade , Poluentes do Solo/análiseRESUMO
Hypereosinophilia is unusual in rheumatoid arthritis (RA), but can occur in severe long-lasting disease, especially in patients with extra-articular manifestations and high titers of rheumatoid factor (RF). The association of RA and hypereosinophilic syndrome (HES) remains yet poorly known. We present a case of a 46 years old woman with long-standing untreated RA, that presented to emergency department with severe symptoms of constrictive pericarditis with cardiac tamponade and bilateral pleural effusion, that progressed to cardiac arrest, associated to symmetrical polyarthritis and pruritic erythematous skin papules. She was submitted to urgent pericardial drainage and partial pericardiotomy. Laboratory analyses revealed hypereosinophilia, and elevated inflammatory parameters and immunoglobulin E. The histological study of the pericardium showed results consistent with inflammatory fibrinous pericarditis. Taking into account the presence of some characteristics that are usually present in cases of reactive HES instead of idiopathic HES, and after an intensive diagnostic study, that could rule out other potential causes of secondary HES, the diagnosis of HES associated with RA was made. She started glucocorticoids during hospitalization and methotrexate 15mg per week at the first outpatient rheumatology visit. After 12 weeks of treatment, we considered that she was in clinical and analytical remission, consistently maintaining that after a complete tapering of glucocorticoids. This case illustrates that clinicians should be aware that HES (including severe life-threatening cases) can occur in patients with RA, especially in cases of long-lasting disease with high titters of RF and without treatment, even in the absence of extra-articular features.
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Artrite Reumatoide , Tamponamento Cardíaco , Síndrome Hipereosinofílica , Feminino , Humanos , Pessoa de Meia-Idade , Artrite Reumatoide/complicações , Metotrexato/uso terapêutico , Tamponamento Cardíaco/complicações , Pericárdio/patologia , Fator Reumatoide , Glucocorticoides/uso terapêutico , Síndrome Hipereosinofílica/complicaçõesRESUMO
BACKGROUND: Williams syndrome (WS) and Autism Spectrum Disorders (ASD) are neurodevelopmental conditions associated with atypical but opposite face-to-face interactions patterns: WS patients overly stare at others, ASD individuals escape eye contact. Whether these behaviors result from dissociable visual processes within the occipito-temporal pathways is unknown. Using high-density electroencephalography, multivariate signal processing algorithms and a protocol designed to identify and extract evoked activities sensitive to facial cues, we investigated how WS (N = 14), ASD (N = 14) and neurotypical subjects (N = 14) decode the information content of a face stimulus. RESULTS: We found two neural components in neurotypical participants, both strongest when the eye region was projected onto the subject's fovea, simulating a direct eye contact situation, and weakest over more distant regions, reaching a minimum when the focused region was outside the stimulus face. The first component peaks at 170 ms, an early signal known to be implicated in low-level face features. The second is identified later, 260 ms post-stimulus onset and is implicated in decoding salient face social cues. Remarkably, both components were found distinctly impaired and preserved in WS and ASD. In WS, we could weakly decode the 170 ms signal based on our regressor relative to facial features, probably due to their relatively poor ability to process faces' morphology, while the late 260 ms component was highly significant. The reverse pattern was observed in ASD participants who showed neurotypical like early 170 ms evoked activity but impaired late evoked 260 ms signal. CONCLUSIONS: Our study reveals a dissociation between WS and ASD patients and points at different neural origins for their social impairments.
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Transtorno do Espectro Autista , Transtornos do Neurodesenvolvimento , Síndrome de Williams , Eletroencefalografia , HumanosRESUMO
Clinically amyopathic dermatomyositis (CADM) is a rare condition characterized by dermatomyositis skin lesions without clinically apparent muscle involvement. Respiratory involvement is common, occurring in about half of the cases. Spontaneous pneumomediastinum (PnM) is a rare, and often fatal, complication of CADM. We report a case of a 61-year-old female patient who was diagnosed with anti-melanoma differentiation- associated gene 5 antibody-associated CADM and interstitial lung disease. She developed an extensive spontaneous PnM with subcutaneous emphysema. The patient was treated with a conservative approach which was, initially, successful in reducing the size of the PnM. However, the patient died from an eventual nosocomial pneumonia requiring mechanical ventilation. This case illustrates that improving the management of CADM associated PnM, remains a major unmet need.