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Physical activity and diet have complementary benefits for attenuating adverse health effects that childhood cancer survivors experience after the end of treatment. This review aimed to explore the impact of combined physical activity and diet interventions on the health status of childhood cancer survivors. A thorough review of the literature was carried out using the Cochrane Library, PubMed, Scielo, LILACS and Web of Science databases for studies published until June 2024. Studies concerning survivors of childhood cancer of any type engaged exclusively in combined diet/physical activity interventions were included in this review. Three manuscripts met the inclusion criteria. The results indicated that combined physical activity plus diet interventions showed beneficial impacts for the intestinal microbiome, percentage of calories from sweet foods, daily consumption of sugary beverages, reduction in sedentary activity, and mood (1 out of 1 study for each of the presented outcomes). Although more rigorous studies are needed, these findings showed promising results regarding the impact of interventions involving both physical activity and diet on childhood cancer survivors

IntroductionSystemic lupus erythematosus (SLE) is a clinically heterogeneous disease that presents a challenge for clinicians. To identify potential biomarkers for diagnosis and disease activity in SLE, we investigated a selected yet broad panel of cytokines and autoantibodies in patients with SLE, healthy controls (HC), and patients with other autoimmune diseases (AIDs).MethodsSerum samples from 422 SLE patients, 546 HC, and 1223 other AIDs were analysed within the frame of the European PRECISESADS project (NTC02890121). Cytokine levels were determined using Luminex panels, and autoantibodies using different immunoassays.ResultsOf the 83 cytokines analysed, 29 differed significantly between patients with SLE and HC. Specifically, CCL8, CXCL13, and IL-1RA levels were elevated in patients with active, but not inactive, SLE versus HC, as well as in patients with SLE versus other AIDs. The levels of these cytokines also correlated with SLE Disease Activity Index 2000 (SLEDAI-2K) scores, among five other cytokines. Overall, the occurrence of autoantibodies was similar across SLEDAI-2K organ domains, and the correlations between autoantibodies and activity in different organ domains were weak.DiscussionOur findings suggest that, upon validation, CCL8, CXCL13, and IL-1RA could serve as promising serum biomarkers of activity in SLE.

Abstract Background This study aimed to investigate the trajectory of damage accrual, associated factors, and impact on health-related quality of life (HR-QoL) in a multicenter cohort of patients with Behçet’s syndrome (BS) over 2 years of follow-up. Methods Patients recruited in the BS Overall Damage Index (BODI) validation study were prospectively monitored for 2 years and assessed for damage accrual, defined as an increase ≥1 in the BODI score, and HR-QoL was evaluated by the SF-36 questionnaire. Logistic and multiple linear regression models were built to determine factors associated with damage accrual and impairment in the different SF-36 domains. Results During follow-up, 36 out of 189 (19.0%) patients had an increase ≥1 in the BODI score with a mean (SD) difference of 1.7 (0.8) (p

Summary: Idiopathic inflammatory myopathies (IIM) are a group of chronic autoimmune diseases mainly affecting proximal muscles. Absence of meaningful prognostic factors in IIM has hindered new therapies development. Glycans are essential molecules that regulate immunological tolerance and consequently the onset of autoreactive immune response.We showed that muscle biopsies from patients with IIM revealed a deficiency in the glycosylation pathway resulting in loss of branched N-glycans. At diagnosis, this glycosignature predicted disease relapse and treatment refractoriness. Peripheral CD4+ T cells from active-disease patients shown a deficiency in branched N-glycans, linked to increased IL-6 production. Glycan supplementation, restoring homeostatic glycosylation profile, led to a decrease in IL-6 levels.This study highlights the biological and clinical importance of glycosylation in IIM immunopathogenesis, providing a potential mechanism for IL-6 production. This pinpoints muscle glycome as promising biomarker for personalized follow-up and a potential target for new therapies in a patients’ subgroup with an ominous evolution.

Systemic lupus erythematosus (SLE), antiphospholipid syndrome (APS), and Sjögren’s syndrome (SS) are heterogeneous autoimmune diseases. Severe manifestations and refractory/intolerance to conventional immunosuppressants demand other options, namely biological drugs, and small molecules. We aimed to define evidence and practice-based guidance for the off-label use of biologics in SLE, APS, and SS. Recommendations were made by an independent expert panel, following a comprehensive literature review and two consensus rounds. The panel included 17 internal medicine experts with recognized practice in autoimmune disease management. The literature review was systematic from 2014 until 2019 and later updated by cross-reference checking and experts’ input until 2021. Preliminary recommendations were drafted by working groups for each disease. A revision meeting with all experts anticipated the consensus meeting held in June 2021. All experts voted (agree, disagree, neither agree nor disagree) during two rounds, and recommendations with at least 75% agreement were approved. A total of 32 final recommendations (20 for SLE treatment, 5 for APS, and 7 for SS) were approved by the experts. These recommendations consider organ involvement, manifestations, severity, and response to previous treatments. In these three autoimmune diseases, most recommendations refer to rituximab, which aligns with the higher number of studies and clinical experience with this biological agent. Belimumab sequential treatment after rituximab may also be used in severe cases of SLE and SS. Second-line therapy with baricitinib, bortezomib, eculizumab, secukinumab, or tocilizumab can be considered in SLE-specific manifestations. These evidence and practice-based recommendations may support treatment decision and, ultimately, improve the outcome of patients living with SLE, APS, or SS.

Resumo Este trabalho tece reflexões sobre a geografia do voto das eleições de 2006 para deputados estaduais, em Minas Gerais, considerando o perfil da representação dos municípios da Região Metropolitana de Belo Horizonte. A hipótese considerada é que as regiões metropolitanas seriam sub-representadas nos Legislativos, dado o seu alto grau de competição eleitoral, em relação ao restante dos municípios. As estratégias eleitorais dos candidatos privilegiariam a atuação nos municípios não metropolitanos, que ofereceriam maiores chances de maximização de votos. São utilizadas informações georreferenciadas aos locais de votação. Definidos os padrões de votação, eles são relacionados com as características da trajetória de carreira e a produção legislativa dos deputados, visando a testar algumas hipóteses, especialmente a da conexão eleitoral.

Macrophagic myofasciitis (MMF) is a rare immune-mediated myopathy that seems to be triggered by aluminium hydroxide adjuvant used in vaccines. Its presentation is relatively heterogeneous and treatment with steroids leads to improvement, although there is little evidence regarding the role of other immunosuppressants. The histological findings in MMF seem to be the result of an abnormal presence in the inoculation site of aluminium, which can induce an immune-mediated muscular disease in susceptible persons. The authors describe the case of a patient with an atypical presentation of macrophagic myofasciitis, with histological confirmation in a muscle biopsy distant from the inoculation site, and a good therapeutic response to tacrolimus and mycophenolate mofetil, as well as a discussion on the pathologic basis, controversies and emerging treatments for this condition.

Objective To develop and validate the evidence-based and consensus-based Behçet’s Syndrome Overall Damage Index (BODI).Methods Starting from 120 literature-retrieved preliminary items, the BODI underwent multiple Delphi rounds with an international multidisciplinary panel consisting of rheumatologists, internists, ophthalmologists, neurologists, and patient delegates until consensus was reached on the final content. The BODI was validated in a cross-sectional multicentre cohort of 228 patients with Behçet’s syndrome (BS) through the study of (a) correlation between BODI and Vasculitis Damage Index (VDI) and (b) correlation between BODI and disease activity measures (ie, Behçet’s Disease Current Activity Form (BDCAF), Physician Global Assessment (PGA), Patient Global Assessment (PtGA)), c) content and face validity and (d) feasibility.Results The final BODI consists of 4 overarching principles and 46 unweighted-items grouped into 9 organ domains. It showed good to excellent reliability, with a mean Cohen’s k of 0.84 (95% CI 0.78 to 0.90) and a mean intra-class correlation coefficient of 0.88 (95% CI 0.80 to 0.95). Overall, 128 (56.1%) patients had a BODI score ≥1, with a median score of 1.0 (range 0–14). The BODI significantly correlated with the VDI (r=0.693, p

Aproximadamente 70 a 90% dos doentes com Esclerose Sistémica apresentam envolvimento gastrointestinal e, em 10% dos casos, este envolvimento constitui a forma de apresentação de Esclerose Sistémica. Todo o trato gastrointestinal pode estar afectado sendo o esófago o órgão mais frequentemente descrito.

Introduction: Type 2 diabetes mellitus is a common disease, affecting up to 13.1% of the Portuguese population. In addition to the known micro and macrovascular complications, drug side effects constitute a major concern, leading to changes in the treatment guidelines, which favor safety over efficacy. Metformin is the first-line pharmacological treatment for most patients with type 2 diabetes mellitus; however, it has been associated with vitamin B12 deficiency in up to 30% of treated patients. The authors describe the prevalence of vitamin B12 deficiency in a diabetic population and explore the possible underlying factors. Material and Methods: Retrospective, observational study. Clinical and laboratory data of type 2 diabetes mellitus patients whose vitamin B12 status was evaluated in the last decade (2005 - 2016) were analyzed. Patients with known malabsorptive syndromes or having undergone bariatric surgery were excluded from the study. Statistical analysis of the data was done and the results were considered statistically significant at p values < 0.05. Results: The study included a total of 1007 patients (58% women) with a mean age of 66.4 ± 12.2 years and 11 ± 10.4 years of type 2 diabetes mellitus duration. These patients had a high prevalence of complications: diabetic renal disease 47.7%, neuropathy 9.2%, retinopathy 14.9%, coronary artery disease 8.4%, cerebrovascular disease 10.9%, and peripheral arterial disease 5.5%. Vitamin B12 deficiency (< 174 ng / dL) was present in 21.4% of the population and this subgroup was older (68.4 vs 65.8 years, p = 0.006), had a longer type 2 diabetes mellitus duration (13.35 vs 10.36 years; p = 0.001), higher prevalence of retinopathy (20.9% vs 13.3%; p = 0.005) and thyroid dysfunction (34% vs 23.7%; p = 0.002). Vitamin B12 deficiency was also more frequent in patients treated with metformin (24.7% vs 15.8%; p = 0.017), antiplatelet agents (25.4% vs 16.2%, p < 0.001), and calcium channel blockers (26.8% vs 18.2%; p = 0.001). After adjustment for possible confounders, the variables associated with B12 deficiency were: metformin, hypothyroidism, age and type 2 diabetes mellitus duration. Discussion: Despite the retrospective design, the results report a high prevalence of vitamin B12 deficiency in the type 2 diabetic population. This study also demonstrates that the B12 deficiency risk is higher in older people, with longer diabetes mellitus duration, hypothyroidism and treated with metformin. Conclusion: Further studies are needed to identify the risk factors for the B12 deficit. The recognition of these variables will contribute to optimize the screening and prevention of the B12 deficiency in type 2 diabetes mellitus.

Systemic lupus erythematosus (SLE) is an autoimmune disease with a strong ethnic and gender bias. In a transancestral genetic association study, Langefeldet al. identify 24 novel regions associated with risk to lupus and propose a cumulative hits hypothesis for loci conferring risk to SLE.

Primary adrenal lymphoma is a rare malignancy. It frequently presents bilaterally and with symptoms of adrenal insufficiency. Amiodarone may induce secondary organ dysfunction, and thyrotoxicosis develops in 15% of cases. The symptomatology of both conditions is nonspecific, especially in the elderly, and a high suspicion index is necessary for appropriate diagnosis. A 78-year-old female presented to the emergency department with confusion, nausea and vomiting. She had recently been to the emergency department with urinary tract infection, vomiting and acute hypochloremic hyponatremia. Upon re-evaluation, the leukocyturia persisted and because of TSH 0.01 μU/mL and free-T4 68 (10–18) pmol/L, she was admitted to the Endocrinology ward. Further evaluation supported amiodarone-induced thyroiditis type 2. Sepsis ensued, in the setting of nosocomial pneumonia. Hemodynamic instability, hyponatremia, hypoglycemia and vomiting raised the suspicion of adrenocortical insufficiency. Fluid resuscitation and hydrocortisone led to clinical improvement, and adrenal insufficiency was admitted. The thoracoabdominal tomography suggested an endobronchic primary lesion with hepatic and adrenal secondary deposits (6.6 and 7 cm), but this was confirmed neither on pleural effusion nor on bronchofibroscopic fluid analyses. The adrenals were not accessible for biopsy. Despite high-dose hydrocortisone maintenance, the patient died before definite diagnosis. The autopsy confirmed primary non-Hodgkin lymphoma.

Central diabetes insipidus (DI) is a rare clinical entity characterized by low circulating levels of antidiuretic hormone (ADH) presenting with polyuria and volume depletion. Pituitary surgery is the most common cause of central DI in adults. Pituitary and hypothalamic disease, particularly invasive neoplasms, rarely cause DI, being idiopathic cases responsible for the majority of non-surgical cases. HIV patients, especially those with poor virulogical control, are prone to the development of CNS neoplasms, particularly lymphomas. These neoplasms usually become manifest with mass effects and seizures. Central DI and hypopituitarism are uncommon initial manifestations of primary CNS lymphomas. The authors describe the case of 29-year-old female, HIV-positive patient whose CNS lymphoma presented with DI.

Introduction: Several studies have demonstrated the benefits of having a regular care provider on the control of chronic diseases. Our study intends to clarify the effects of the transition to a new diabetologist on metabolic control in type 2 diabetes patients followed-up in a tertiary care setting. Material and Methods: Retrospective study performed in an endocrinology outpatient clinic. We randomly selected 50 type 2 diabetes patients for a control group and 50 for a study group. In the study group, we registered the last evaluation before the physician change (year 0) and at the end of each year (year 1, 2 and 3) with the new doctor. Evaluated variables — body mass index, blood pressure, HbA1c and lipid profile — were compared yearly between groups. Results: There was a decrease in mean HbA1c levels (0.4% – 0.5%, p < 0.05) in year 1 and 2 when compared to year 0 in the study group, but not in the control group. This reduction was superior (0.5% – 1.4%, p < 0.05) in patients whose baseline HbA1c was greater than 7%. The other studied variables did not vary significantly throughout follow-up in either group. Discussion: In our study the transition to a different type 2 diabetes physician was associated with a decrease in mean HbA1c and this difference was greater in less well controlled patients. Conclusion: Switching to a new physician may not be harmful and may actually have benefits for the glycemic control of some type 2 diabetes patients.

O vitiligo, uma leucodermia adquirida particularmente prevalente, caracteriza-se pelo impacto psicossocial, cronicidade e resistência aos tratamentos. Na sua génese intervêm de forma intrincada mecanismos genéticos, ambientais, stress oxidativo e autoimunidade. As comorbilidades imunologicamente mediadas ocorrem com frequência variável mas significativa e devem justificar uma atenção redobrada por parte dos Dermatologistas. A síndrome de Schmidt – síndrome poliglandular autoimune do tipo II é definida pela ocorrência de doença de Addison e patologia autoimune da tiroideia e/ou diabetes mellitus tipo 1. Relatamos caso de uma doente com 64 anos de idade, com antecedentes de doenças imunologicamente mediadas, incluindo história de tiroidite autoimune e de vitiligo acrofacial estável o qual, nos últimos 3 meses, sofrera uma modificação impressiva nas suas extensão e morfologia, no contexto de uma melanodermia difusa recém-instalada. A avaliação laboratorial efectuada documentou hipocortisolismo, ulteriormente confirmado por provas funcionais, bem como marcadores imunológicos e imagiologia compatíveis: doença de Addison autoimune enquadrável numa síndrome de Schmidt. A correcção da insuficiência endócrina permitiu equilibrar a doente nos planos hidroelectrolítico e metabólico, tendo sido observados uma reversão da hipermelanose e estabilização do vitiligo. Salientamse a progressão importante de um vitiligo até então estável; as novas particularidades morfológicas da dermatose – lesões em confetti, vitiligo tricrómico e fenómeno de Koebner – e a hipermelanose difusa numa doente com história de múltiplas manifestações de autoimunidade. Conclui-se pela necessidade de reconhecer e, logo, suspeitar de comorbilidades imunologicamente mediadas do vitiligo, em particular quando na presença de atipias semiológicas ou evolutivas e no contexto de história clínica sugestiva.

Health related quality of life (HRQL) and survival are two important outcome measures in chronic diseases. This study aimed to compared HRQL in patients with different chronic diseases of immune system and normative data from the general Portuguese Population. It was selected 103 out-patients, by convenience, to complete SF-36v2. The lowest scores were found among measures for general health (41.0), vitality (47.5), bodily pain (51.0), mental health (56.4); women, except for role-physical, and patients with auto-immune diseases have had the worse scores on all assessed dimension of subjective health, when compared with normative data. Highest scores were obtained in the following scales: physical functioning (69.1), social functioning (66.9), role-emotional (64.9). Living with chronic immune disease have impact on HRQL and it can be expected that the Portuguese version of SF-36v2 provide valid and reliable HRQL data.

Este trabalho busca demonstrar como a relação entre ideias, atores, instituições e interesses explicam a formação da agenda da reforma do sistema público de ensino de Minas Gerais das últimas décadas do século passado. Atores diversos em termos de ideologia e interesses compartilham um mesmo paradigma de reforma educacional, segundo os valores de descentralização e participação. Apesar disso, desenvolvem argumentos diferenciados.Este trabajo tiene por objeto demostrar como la relación entre ideas, actores, instituciones e intereses explican la formación de la agenda de la reforma del sistema público de enseñanza de Minas Gerais (Provincia de Brasil) de las últimas décadas del siglo pasado. Distintos actores, en términos de ideología e intereses, comparten un mismo paradigma de reforma educacional, según los valores de descentralización y participación. A pesar de ello, desarrollan argumentos diferenciados.This article aims to demonstrate how the relationship among different ideas, actors, institutions and interests explain the policy making processes of the reform of the State public school system in Minas Gerais, Brazil in the last decades of the last century. The different actors, presenting distinct political perspectives, interests and ideologies shared one common policy paradigm: decentralization and participation. However, they developed different arguments.

O Fenómeno de Raynaud (FR) é uma manifestação clínica comum, traduzida por vasoespasmos recorrentes dos dedos, na maioria das vezes provocados pela exposição ao frio ou estímulos emocionais. Pode ser primário (idiopática) ou secundária a diversas doenças ou condições, incluindo doenças do tecido conjuntivo, como esclerose sistémica. A patogênese da FR é mal compreendido. Actualmente pensa-se que a lesão endotelial e a disfunção vascular associada podem ser as primeiras alteração patogénicas e responsável pelas complicações vasculares nos doentes com RP. As alterações funcionais (vasoconstrição) e as mudanças estruturais (proliferação intimal e obstrução dos capilares e artérias), traduzem-se clinicamente como RP e úlceras digitais. O FR é em muitos casos a primeira manifestação da doença secundária associado e precede as outras manifestações clínicas em anos.Raynaud´s Phenomenon (RP) is a common clinical disorder, manifested by recurrent vasospasm of fingers and toes, often triggered by exposure to cold temperatures or emotional stress. It can be primary (idiopathic) or secondary to several diseases or conditions, including connective tissue diseases such as systemic sclerosis. The pathogenesis of RP is poorly understood. It is generally accepted that endothelial damage and vascular dysfunction may be the earliest pathogenetic alteration and ethological factor responsible for vascular complications in RP patients. Functional abnormalities (vasoconstriction) and structural changes (intimal proliferation, obstruction) are expressed clinically as RP and digital ulcers. RP is the first manifestation of the secondary associated disease and precedes other clinical manifestations by years.

O objetivo do trabalho é apresentar os dilemas envolvidos no esforço de se conceituar federalismo. Aborda desde as referências mais básicas do significado de federalismo até algumas tentativas que buscam detalhar mais detidamente o conceito. Estabelecido um quadro geral de aspectos que definiriam o federalismo, buscaremos demonstrar que, ainda assim, ao abordar realidades diversas a partir desse quadro, permanece apresentando um problema de definição. Para exemplificar a persistência dessa indefinição conceitual, faremos referência a casos concretos cujas características específicas podem ajudar a evidenciar os dilemas envolvidos nesse esforço de sugerir um conceito de federalismo. Busca-se argumentar que as abordagens formalistas, que focam nas características das instituições, correm o risco de apresentar conclusões equivocadas. O tipo específico de federalismo existente depende de como suas instituições imbricam com contextos políticos particulares. Assim, os tipos particulares de instituições federativas combinam com outras variáveis políticas, redundando em resultados diversificados. Além disso, as instituições são ativadas por processos políticos específicos. A forma como o poder se distribui em um dado sistema político depende, em parte, das características de suas instituições, mas depende também de como o jogo político se processa

O autor analisa a relação entre a literatura que trata da configuração das instituições democráticas, em décadas recentes, e aqueles trabalhos que abordam as características da cultura política dos espaços urbanos. Argumenta sobre a necessidade de se viabilizar o diálogo entre as perspectivas teóricas culturalista e institucionalista, que, até agora, se desenvolvem de forma paralela e estanque.The author analyses how scholars perceive democracy today. The writers who seek to explain the democratization process use cultural or institutional frameworks. However, the conclusion is that democracy should be studied by cultural and institutional factors. It is argued that this dialogue between both is needed.

Autoimmune diseases (AIDs) are characterized by a multifactorial aetiology and a complex genetic background, with the MHC region playing a major role. We genotyped for HLA-DRB1 locus 1228 patients with AIDs-213 with Systemic Lupus Erythematosus (SLE), 166 with Psoriasis or Psoriatic Arthritis (Ps + PsA), 153 with Rheumatoid Arthritis (RA), 67 with Systemic Sclerosis (SSc), 536 with Multiple Sclerosis (MS), and 93 with Myasthenia Gravis (MG) and 282 unrelated controls. We confirmed previously established associations of HLA-DRB115 (OR = 2.17) and HLA-DRB103 (OR = 1.81) alleles with MS, HLA-DRB103 with SLE (OR = 2.49), HLA-DRB101 (OR = 1.79) and HLA-DRB104 (OR = 2.81) with RA, HLA-DRB107 with Ps + PsA (OR = 1.79), HLA-DRB101 (OR = 2.28) and HLA-DRB108 (OR = 3.01) with SSc, and HLA-DRB103 with MG (OR = 2.98). We further observed a consistent negative association of HLA-DRB113 allele with SLE, Ps + PsA, RA, and SSc (18.3%, 19.3%, 16.3%, and 11.9%, resp., versus 29.8% in controls). HLA-DRB113 frequency in the AIDs group was 20.0% (OR = 0.58). Although different alleles were associated with particular AIDs, the same allele, HLA-DRB113, was underrepresented in all of the six diseases analysed. This observation suggests that this allele may confer protection for AIDs, particularly for systemic and rheumatic disease. The protective effect of HLA-DRB113 could be explained by a more proficient antigen presentation by these molecules, favouring efficient clonal deletion during thymic selection.

O trabalho tem como objetivo desenvolver os pressupostos básicos da perspectiva neoinstitucionalista para a análise de políticas públicas. Inicia-se buscando situar o neoinstitucionalismo no debate mais amplo, especificamente abordando o esforço de sua consolidação como contraposição aos modelos pluralista e marxista. Buscando registrar o desenvolvimento de suas proposições iniciais, no sentido da superação de problemas contidos em suas proposições iniciais, o trabalho ressalta dois aspectos relevantes relacionadodos com modelo discutido: examina o papel das idéias na formulação de políticas públicas e a questao da mudança institucional. Como conclusão, busca apontar alguns pontos fortes e algumas fragilidades de modelo enfocado, dependendo, entre outras coisas, que o neoinstitucionalismo fornece elementos valiosos para estudos empíricos, não dando conta, contudo, de desenvolver teorias de maior alcance sobre a relação entre poder e políticas públicas

This study deals with the reform of the State public school system in Minas Gerais, Brazil. The central aim of the reform is to establish decentralized spaces for participation by the population and professional educators in school management as a way of neutralizing the use of schools for political cronyism. The reform involved a process that spanned the terms of three different Governors, with participation by various actors displaying distinct political perspectives, interests, and ideologies. The study attempts to explain the causal factors affecting the decision-making process. As explanatory factors, the case study suggests the actors' capacity to establish common objectives and utilize available power instruments, the characteristics of the institutional context in which they acted, and the learning process developed with the legacy of previous policies.