Primary Central Nervous System Lymphoma Complicated by Cerebral Venous Sinus Thrombosis: A Case Report.

Central nervous system (CNS) lymphoma is a rare and aggressive primary neoplasm that comprises a small proportion of brain tumours and non-Hodgkin lymphomas. We present a case report of a 64-year-old woman with CNS lymphoma, who exhibited cognitive changes, weight loss and neurological symptoms. Imaging scans revealed multiple lesions in the brain and thrombosis in the venous sinuses. A diagnosis of diffuse large B-cell lymphoma of the CNS was confirmed through histological examination. The patient underwent treatment with corticosteroids and chemotherapy, but experienced clinical deterioration with thrombocytopenia and disease progression. Despite efforts to manage complications and provide targeted therapy, the patient passed away. Primary CNS lymphoma typically responds well to chemotherapy, and prognostic factors such as age and functional status play a significant role in patient outcomes. However, complications such as thromboembolism pose challenges during treatment due to the hypercoagulable state induced by chemotherapy agents. The pathophysiology of thromboembolic events in the context of malignancy remains uncertain but may involve direct tumour compression, vascular invasion and alterations in coagulation factors. The diagnostic process for CNS lymphoma can be complex, and the information obtained from cerebrospinal fluid analysis, including flow cytometry, may be limited in cases with low cell counts. Ongoing research exploring genetic tests and biomarkers shows promise for improving diagnostic accuracy in such cases. This case underscores the need for comprehensive management strategies that address both the neoplasm and its associated complications, to optimise patient outcomes. LEARNING POINTS: Primary CNS lymphoma is a rare primary neoplasm, being even rarer in immunocompetent patients.In 25% of all cases of CNS lymphoma, it is complicated by cerebral venous thromboembolism.Leptomeningeal spread can occur with or without MRI evidence and is diagnosed with cerebrospinal fluid (CSF) flow cytometry. Age and functional status are important prognostic factors.

Levodopa-Induced Pseudopheochromocytoma.

Pseudopheochromocytoma is a pathological condition presenting with paroxysmal hypertension with normal or moderate elevation in catecholamines and metanephrine levels, but no evidence of a tumoural cause. Imaging studies and I-123 metaiodobenzylguanidine scintigraphy are essential for exclusion of pheocromocytoma. We describe a case of pseudopheochromocytoma related to levodopa in a patient with paroxysmal hypertension, headache, sweating, palpitations and increased plasmatic and urinary metanephrine levels, without adrenal or extra-adrenal tumour. The beginning of the patient's clinical symptoms coincided with the initiation of the levodopa treatment and the complete resolution of the symptoms occurred after the discontinuation of levodopa. LEARNING POINTS: Pseudopheochromocytoma and pheochromocytoma may have the same clinical and laboratorial presentation but different aetiologies.The diagnosis of pseudopheochromocytoma is based on paroxysmal hypertension with normal or increased plasma and urine levels of catecholamines or metanephrines after exclusion of a tumoural process.The pseudopheochromocytoma may be associated with levodopa, alone or in combination with other drugs that are likely to interfere with dopamine or catecholamine metabolism.

Impact of delaying botulinum toxin treatment in patients with migraine during the COVID-19 pandemic.

BACKGROUND: Due to coronavirus disease 2019 (COVID-19) pandemic response measures, the administration of botulinum toxin (BTX) was delayed for many patients during the first lockdown period in Portugal. OBJECTIVES: To review the impact of postponing BTX treatment on migraine control. METHODS: This was a retrospective, single-center study. Patients with chronic migraine who had done at least three previous BTX cycles and were considered responders were included. The patients were divided into two groups, one that has had their treatment delayed (group P), and one that has not (controls). The Phase III Research Evaluating Migraine Prophylaxis Therapy (PREEMPT) protocol was used. Migraine-related data were obtained at baseline and at three subsequent visits. RESULTS: The present study included two groups, group P (n = 30; 47.0 ± 14.5 years; 27 females, interval baseline -1st visit: 5.5 [4.1-5.8] months) and the control group (n = 6; 57.7 ± 13.2 years; 6 females; interval baseline-1st visit 3.0 [3.0-3.2] months). No difference between the groups was present at baseline. When compared to baseline, the number of days/month with migraine (5 [3-6.2] vs. 8 [6-15] p < 0.001), days using triptans/month (2.5 [0-6] vs. 3 [0-8], p = 0.027) and intensity of pain (7 [5.8-10] vs. 9 [7-10], p = 0.012) were greater in the first visit for group P, while controls did not present a significant variation. The worsening of migraine-related indicators decreased in the following visits; however, even in the third visit, it had not returned to baseline. Correlations were significant between the delayed time to treatment and the increase in days/month with migraines at the first visit after lockdown (r = 0.507; p = 0.004). CONCLUSIONS: There was a deterioration of migraine control after postponed treatments, with a direct correlation between the worsening of symptoms and the number of months that the treatment was delayed.

ANTECEDENTES: Devido às medidas de resposta à pandemia de coronavirus disease 2019 (covid-19), a administração de toxina botulínica (TXB) foi adiada para muitos pacientes durante o primeiro confinamento em Portugal. OBJETIVOS: Avaliar o impacto do adiamento do tratamento com TXB no controle da enxaqueca. MéTODOS: Estudo retrospectivo unicêntrico. Foram incluídos pacientes com enxaqueca crônica com pelo menos três ciclos prévios de TXB e que tenham sido considerados respondedores. Os pacientes foram divididos em dois grupos, sendo um com atraso do tratamento (grupo P) e outro sem atraso (controles). O protocolo Phase III Research Evaluating Migraine Prophylaxis Therapy (PREEMPT) foi utilizado. Dados clínicos relacionados com a enxaqueca foram obtidos na consulta inicial (T0) e nas três consultas subsequentes (T1­3). RESULTADOS: O presente estudo incluiu dois grupos, o grupo P (n = 30; 47,0 ± 14,5 anos; 27 mulheres, intervalo T0-1ª visita: 5,5 [4,1­5,8] meses) e o grupo controle (n = 6; 57,7 ± 13,2 anos; 6 mulheres; intervalo T0­1ª visita 3,0 [3,0­3,2] meses). Os grupos não apresentavam nenhuma diferença no início do estudo. Quando comparado à T0, o número de dias/mês com enxaqueca (5 [3­6,2] vs. 8 [6­15], p < 0,001), dias usando triptanos/mês (2,5 [0­6] vs. 3 [0­8], p = 0,027) e intensidade da dor (7 [5,8­10] vs. 9 [7­10], p = 0,012) foram maiores na primeira visita no grupo P, não apresentando os controles variação significativa. A piora dos indicadores relacionados com a enxaqueca diminuiu nas visitas seguintes; porém, mesmo na terceira visita, ainda não haviam retornado ao basal. As correlações foram significativas entre o atraso do tratamento e o aumento de dias/mês com enxaqueca na primeira consulta após o confinamento (r = 0,507; p = 0,004). CONCLUSãO: Houve piora clínica da enxaqueca após o adiamento do tratamento em correlação direta com a duração do atraso.

Pd(II) and Pt(II) Trinuclear Chelates with Spermidine: Selective Anticancer Activity towards TNBC-Sensitive and -Resistant to Cisplatin.

Triple-negative breast cancer (TNBC) is one of the most aggressive forms of breast cancer and constitutes 10-20% of all breast cancer cases. Even though platinum-based drugs such as cisplatin and carboplatin are effective in TNBC patients, their toxicity and development of cancer drug resistance often hamper their clinical use. Hence, novel drug entities with improved tolerability and selectivity profiles, as well as the ability to surpass resistance, are needed. The current study focuses on Pd(II) and Pt(II) trinuclear chelates with spermidine (Pd3Spd2 and Pt3Spd2) for evaluating their antineoplastic activity having been assessed towards (i) cisplatin-resistant TNBC cells (MDA-MB-231/R), (ii) cisplatin-sensitive TNBC cells (MDA-MB-231) and (iii) non-cancerous human breast cells (MCF-12A, to assess the cancer selectivity/selectivity index). Additionally, the complexes' ability to overcome acquired resistance (resistance index) was determined. This study revealed that Pd3Spd2 activity greatly exceeds that displayed by its Pt analog. In addition, Pd3Spd2 evidenced a similar antiproliferative activity in both sensitive and resistant TNBC cells (IC50 values 4.65-8.99 µM and 9.24-13.34 µM, respectively), with a resistance index lower than 2.3. Moreover, this Pd compound showed a promising selectivity index ratio: >6.28 for MDA-MB-231 cells and >4.59 for MDA-MB-231/R cells. Altogether, the data presently gathered reveal Pd3Spd2 as a new, promising metal-based anticancer agent, which should be further explored for the treatment of TNBC and its cisplatin-resistant forms.

Impact of delaying botulinum toxin treatment in patients with migraine during the COVID-19 pandemic / O impacto do atraso no tratamento com toxina botulínica em pacientes com enxaqueca no contexto da pandemia da COVID-19

Abstract Background Due to coronavirus disease 2019 (COVID-19) pandemic response measures, the administration of botulinum toxin (BTX) was delayed for many patients during the first lockdown period in Portugal. Objectives To review the impact of postponing BTX treatment on migraine control. Methods This was a retrospective, single-center study. Patients with chronic migraine who had done at least three previous BTX cycles and were considered responders were included. The patients were divided into two groups, one that has had their treatment delayed (group P), and one that has not (controls). The Phase III Research Evaluating Migraine Prophylaxis Therapy (PREEMPT) protocol was used. Migraine-related data were obtained at baseline and at three subsequent visits. Results The present study included two groups, group P (n = 30; 47.0 ± 14.5 years; 27 females, interval baseline -1st visit: 5.5 [4.1-5.8] months) and the control group (n = 6; 57.7 ± 13.2 years; 6 females; interval baseline-1st visit 3.0 [3.0-3.2] months). No difference between the groups was present at baseline. When compared to baseline, the number of days/month with migraine (5 [3-6.2] vs. 8 [6-15] p < 0.001), days using triptans/month (2.5 [0-6] vs. 3 [0-8], p = 0.027) and intensity of pain (7 [5.8-10] vs. 9 [7-10], p = 0.012) were greater in the first visit for group P, while controls did not present a significant variation. The worsening of migraine-related indicators decreased in the following visits; however, even in the third visit, it had not returned to baseline. Correlations were significant between the delayed time to treatment and the increase in days/month with migraines at the first visit after lockdown (r = 0.507; p = 0.004). Conclusions There was a deterioration of migraine control after postponed treatments, with a direct correlation between the worsening of symptoms and the number of months that the treatment was delayed.

Resumo Antecedentes Devido às medidas de resposta à pandemia de coronavirus disease 2019 (covid-19), a administração de toxina botulínica (TXB) foi adiada para muitos pacientes durante o primeiro confinamento em Portugal. Objetivos Avaliar o impacto do adiamento do tratamento com TXB no controle da enxaqueca. Métodos Estudo retrospectivo unicêntrico. Foram incluídos pacientes com enxaqueca crônica com pelo menos três ciclos prévios de TXB e que tenham sido considerados respondedores. Os pacientes foram divididos em dois grupos, sendo um com atraso do tratamento (grupo P) e outro sem atraso (controles). O protocolo Phase III Research Evaluating Migraine Prophylaxis Therapy (PREEMPT) foi utilizado. Dados clínicos relacionados com a enxaqueca foram obtidos na consulta inicial (T0) e nas três consultas subsequentes (T1-3). Resultados O presente estudo incluiu dois grupos, o grupo P (n = 30; 47,0 ± 14,5 anos; 27 mulheres, intervalo T0-1ª visita: 5,5 [4,1-5,8] meses) e o grupo controle (n = 6; 57,7 ± 13,2 anos; 6 mulheres; intervalo T0-1ª visita 3,0 [3,0-3,2] meses). Os grupos não apresentavam nenhuma diferença no início do estudo. Quando comparado à T0, o número de dias/mês com enxaqueca (5 [3-6,2] vs. 8 [6-15], p < 0,001), dias usando triptanos/mês (2,5 [0-6] vs. 3 [0-8], p = 0,027) e intensidade da dor (7 [5,8-10] vs. 9 [7-10], p = 0,012) foram maiores na primeira visita no grupo P, não apresentando os controles variação significativa. A piora dos indicadores relacionados com a enxaqueca diminuiu nas visitas seguintes; porém, mesmo na terceira visita, ainda não haviam retornado ao basal. As correlações foram significativas entre o atraso do tratamento e o aumento de dias/mês com enxaqueca na primeira consulta após o confinamento (r = 0,507; p = 0,004). Conclusão Houve piora clínica da enxaqueca após o adiamento do tratamento em correlação direta com a duração do atraso.

Characterization of a late-onset multiple sclerosis Portuguese cohort.

BACKGROUND: Late-onset multiple sclerosis (LOMS) is defined as the onset of symptoms above 50 years, corresponding to an increasingly recognized subset of MS. This study aimed at comparing demographic and clinical data of patients with LOMS to those of early-onset MS (EOMS) from a Portuguese cohort. METHODS: Retrospective chart review of an MS cohort from a Portuguese tertiary center. RESULTS: From 746 patients with MS (61.7% female), we identified 39 cases with presentation after 50 years of age (22 males and 17 females), corresponding to 5.3%. The mean age at onset was 55.4 (±5.0) for LOMS and 29.5 (±8.9) for EOMS. There was no significant difference in disease duration. The most common type was relapsing-remitting MS, accounting for 51.5% and 83.9% of LOMS and EOMS patients, respectively. Primary-progressive MS (PPMS) was significantly more represented in the LOMS group (41.0%) (p < 0.01). The median EDSS was significantly higher in the LOMS group (4.75, 0.0-7.5) when compared to the EOMS group (2.0, 0.0-9,0). The most frequent presenting feature was myelitis in both LOMS (48.7%) and EOMS patients (47.4%), resulting in significantly higher EDSS (p = 0.003). CONCLUSIONS: LOMS is associated with higher EDSS when considering the same disease duration, translating into increased disability.

Interim FDG18-PET SUVmax Variation Adds Prognostic Value to Deauville 5-Point Scale in the Identification of Patients with Ultra-High-Risk Diffuse Large B Cell Lymphoma.

INTRODUCTION: Interim response evaluation by 18F-fluorodeoxyglucose positron emission tomography/computed tomography (iPET) in diffuse large B cell lymphoma (DLBCL) could be important to rule out disease progression and has been suggested to be predictive of survival. However, treatment guidance by iPET is not yet recommended for DLBCL in clinical practice. We aimed to compare the predictive value of iPET when utilizing the visual Deauville 5-point scale (DS) and the semiquantitative variation of maximum standardized uptake value (ΔSUVmax). MATERIALS AND METHODS: We included 85 patients diagnosed with DLBCL and uniformly treated with standard protocols. iPET with DS of 1-3 and/or ΔSUVmax ≥66% was defined as negative. Univariable and multivariable Cox regression analyses were performed to determine the independent factors affecting progression free survival (PFS) or overall survival (OS) and to estimate PFS and OS. RESULTS: iPET positivity, measured by DS or ΔSUVmax, showed predictive value of disease refractoriness, improved by combining DS and ΔSUVmax. After a median follow-up of 50.1 months, iPET was an independent predictor for both PFS and OS when interpreted by DS, but only for PFS by ΔSUVmax. Combined visual and semiquantitative analysis (D4-5 & ΔSUVmax<66%) was an independent predictor of PFS and OS, and allowed to identify an ultra-high-risk subgroup of patients with very dismal outcome, increasing the discriminating capacity for iPET. CONCLUSION: Our study suggests that combined DS and ΔSUVmax in iPET assessment predicts refractory disease and distinguishes ultra-high-risk DLBCL patients with a very dismal prognosis, who may benefit from PET-guided therapy adjustment.

Anti-NMDAr Encephalitis and COVID-19 in a Patient With Systemic pANCA-Vasculitis and Recurrent Varicella Zoster Infection.

Introduction: There is a complex interplay between systemic autoimmunity, immunosuppression, and infections. Any or all of these can result in neurologic manifestations, requiring diligence on the part of neurologists. Case report: We herein report a case of a patient on immunosuppressive treatment for a vasculitis that resulted in zoster meningoencephalitis. This was further complicated by the development of anti-NMDAr encephalitis, the etiology of which is undetermined and further discussed in this paper. The patient eventually developed COVID-19 during hospitalization, succumbing to the respiratory infection. Conclusion: This case emphasizes that post-infectious autoimmune disorders are becoming increasingly recognized and that they should still be considered in patients who are on immunosuppression. Practitioners should be aware of the complex relationship between autoimmunity and immunosuppression and consider both throughout the disease course.

Preneoplastic somatic mutations including MYD88L265P in lymphoplasmacytic lymphoma.

Normal cell counterparts of solid and myeloid tumors accumulate mutations years before disease onset; whether this occurs in B lymphocytes before lymphoma remains uncertain. We sequenced multiple stages of the B lineage in elderly individuals and patients with lymphoplasmacytic lymphoma, a singular disease for studying lymphomagenesis because of the high prevalence of mutated MYD88. We observed similar accumulation of random mutations in B lineages from both cohorts and unexpectedly found MYD88L265P in normal precursor and mature B lymphocytes from patients with lymphoma. We uncovered genetic and transcriptional pathways driving malignant transformation and leveraged these to model lymphoplasmacytic lymphoma in mice, based on mutated MYD88 in B cell precursors and BCL2 overexpression. Thus, MYD88L265P is a preneoplastic event, which challenges the current understanding of lymphomagenesis and may have implications for early detection of B cell lymphomas.

Association Between Serum Myostatin Levels, Hospital Mortality, and Muscle Mass and Strength Following ST-Elevation Myocardial Infarction.

AIM: This study aimed to evaluate the association between serum myostatin levels, hospital mortality, and muscle mass and strength following ST-segment elevation myocardial infarction (STEMI). METHODS: This was a prospective observational study. Within 48 hours of admission, bioelectrical impedance and handgrip strength were assessed and blood samples collected for myostatin evaluation. Hospital mortality was recorded. A multiple logistic regression model was also constructed, adjusted by parameters that exhibited significant differences in the univariate analysis, to evaluate the association between myostatin levels and hospital mortality. RESULTS: One hundred and two (102) patients were included: mean age was 60.5±10.6 years, 67.6% were male, and 6.9% died during hospital stay. Univariate analysis showed that patients with lower myostatin levels had higher mortality rates. Serum myostatin levels positively correlated with handgrip strength (r=0.355; p<0.001) and appendicular skeletal muscle mass index (r=0.268; p=0.007). Receiver operating characteristic (ROC) curve analysis revealed that lower myostatin levels were associated with hospital mortality at the <2.20 ng/mL cut-off. Multiple logistic regression showed that higher serum myostatin levels were associated with reduced hospital mortality when adjusted by ß blocker use (OR, 0.228; 95% CI, 0.054-0.974; p=0.046). CONCLUSIONS: Serum myostatin concentrations positively correlated with muscle mass and strength in STEMI patients. Further assessment of serum myostatin association with mortality should be conducted using a larger sample and assessing the additive value to the Global Registry of Acute Coronary Events (GRACE) or thrombolysis in myocardial infarction (TIMI) risk scores.

Kriging Versus Cokriging and Collokated Cokriging for Soil Physical-Hydraulic Attributes and their Influence on Soybean Growth

Abstract in Brazil, management agricultural practices not currently consider the soil spatial variability as a result, crop growth can be non-uniform and yields often is low. This research aims to compare Kriging, Cokriging and Collocated cokriging using soil physical and hydraulic properties and their influences on soybean development. We hypothesized that spatial variability of physical and hydraulic properties has influence on soybean development and this variability can be better represented by Collocated Cokriging method. To test these hypotheses, we accessed the soil physical and hydraulic attributes in a field experiment under no-till system, cultivated with soybean. Geostatistical interpolators were applied to generate maps from which spatial dependence of the variables was evaluated. The experiment was conducted on a sandy clay loam Oxisol, on an experimental station located in Ponta Grossa, Paraná, Brazil. Evaluation of the soil attributes was performed: bulk density (BD), particle size distribution, saturated soil hydraulic conductivity (K fs ), total porosity (TP), macroporosity and microporosity. The plant was plant height and stand. Data analysis were performed by geostatistical methods; the spatial dependence was established using experimental univariate and cross semivariograms with datasets. Modeling semivariograms led to the generation of attribute maps by Kriging, Cokriging and Collocated cokriging. The estimation by Cokriging and Collocated cokriging was similar from Kriging. From the semivariogram, it was possible to identify that soil and plant attributes were spatially related with each other. The soya growth was mainly changed by slope of the area and little changed by saturated hydraulic conductivity.

Frailty and mortality in long-term care facilities for older people in Brazil: a survival analysis / Fragilidade e mortalidade em instituições de longa permanência para idosos no Brasil: estudo de sobrevivência

OBJECTIVES: To investigate the effect of frailty on 1-year mortality in long term-care facility (LTCF) residents. METHODS: This was a prospective cohort study with survival analysis of 209 participants living in 15 Brazilian LTCFs. Data on chronic diseases, age, sex, medication use, dependence in activities of daily living (ADLs; Katz index), and frailty (FRAIL scale) were collected at baseline, and death after 1 year was the outcome measure. Kaplan-Meier estimate and log-rank test were used to analyze the survival of residents. RESULTS: In the initial assessment, 65.07 of the residents were women, and the median age was 82 (interquartile range, 71­88) years, with 55% being over 80 years old. Overall, 88% had 2 or more diseases, 59.81% were using 5 or more medications, 42.11% were considered frail, 34.92% pre-frail, and 22.97% robust, and 69.94% were dependent in 3 or more ADLs. During the 12-month follow-up, 19.61% of the residents (n=41) died. In the survival analysis for death, there was a statistically significant association with frailty (p=0.03) and dependence in ADLs (p=0.04). CONCLUSIONS: In this population of LTCF residents, frailty and functional dependence were associated with death.

OBJETIVOS: Investigar o efeito da fragilidade na mortalidade em 1 ano em residentes de instituições de longa permanência para idosos (ILPIs). METODOLOGIA: Estudo de coorte prospectivo com análise de sobrevivência de 209 participantes residentes em 15 ILPIs brasileiras. Dados sobre doenças crônicas, idade, sexo, uso de medicamentos, dependência nas atividades da vida diária (AVDs; índice de Katz) e fragilidade (escala FRAIL) foram coletados no início do estudo, e morte após 1 ano foi a medida de desfecho. A estimativa de Kaplan-Meier e o teste de log-rank foram usados para analisar a sobrevida dos residentes.. RESULTADOS: Na avaliação inicial, 65,07% dos residentes eram mulheres e a mediana da idade era de 82 (intervalo interquartil, 71­88) anos, 55% com mais de 80 anos. Em geral, 88% tinham 2 ou mais doenças, 59,81% usavam 5 ou mais medicamentos, 42,11% foram considerados frágeis, 34,92% pré-frágeis e 22,97% robustos e 69,91% eram dependentes em 3 ou mais AVDs. No decorrer do seguimento de 12 meses, 19,61% dos residentes (n =41) evoluíram para óbito. Na análise de sobrevivência para evento morte, houve associação estatisticamente significativa com fragilidade (p=0,03) e dependência para AVDs (p=0,04). CONCLUSÕES: Nesta população de residentes de ILPIs, fragilidade e dependência funcional estiveram associadas ao óbito.

[Asthma Management and Control in Portuguese Speaking Countries]. / Gestão e Controlo da Asma em Países de Expressão Portuguesa.

INTRODUCTION: Asthma affects more than 339 million people worldwide. In the Community of Portuguese Speaking Countries, in 2016, its prevalence ranged from 9.5% (Portugal) to 3.91% (Brazil). Chronic disease management programs aim to improve the health status of patients with chronic disease and reduce associated costs. The objective of this study is to identify models of asthma asthma 'management and control' that are that are implemented in the Community of Portuguese Speaking Countries (CPLP), and analyse them through the integrated disease management model. MATERIAL AND METHODS: A rapid review of the PubMed indexed scientific literature and grey literature on 'management and control of asthma' in the countries of the Community of Portuguese-Speaking Countries was carried out. RESULTS: Portugal, Brazil and Mozambique presented publications on 'management and control of asthma', at different stages of implementation. Clinical management and organization and service delivery are the dimensions of integrated disease management most addressed in publications. DISCUSSION: The implementation of asthma management and control programs is influenced by health systems, care delivery structures, and the surrounding political and social environment. The dimensions of funding and information systems are the most difficult to implement given the degree of economic, social and technological development of most countries under study. CONCLUSION: Only Portugal, Brazil and Mozambique adopted asthma integrated disease management as the main form of asthma management and control. The programs developed by these countries can constitute a model for asthma integrated disease management in the other countries under study.

Introdução: A asma atinge mais de 339 milhões de pessoas mundialmente. Na Comunidade dos Países de Língua Portuguesa, em 2016, a sua prevalência variou entre 9,15% (Portugal) e 3,91% (Brasil). Os programas de gestão da doença crónica pretendem melhorar o estado de saúde de doentes com doença crónica e reduzir os custos associados. O objetivo deste estudo é identificar modelos de 'gestão e controlo da asma' implementados na Comunidade dos Países de Língua Portuguesa, analisando-os através do modelo de gestão integrada de doença.Material e Métodos: Realizou-se uma revisão rápida da literatura científica indexada na PubMed, e de literatura cinzenta sobre 'gestão e controlo da asma' nos países da Comunidade dos Países de Língua Portuguesa.Resultados: Portugal, Brasil e Moçambique apresentaram publicações sobre 'gestão e controlo da asma', em diferentes fases de implementação dos programas. A gestão clínica e organização e prestação de cuidados são as dimensões mais abordados nas publicações.Discussão: A implementação de programas de gestão e controlo da asma é influenciada pelos sistemas de saúde, estruturas de prestação de cuidados em que se inserem, meio político e social envolventes. As dimensões do financiamento e dos sistemas de informação são as mais difíceis de implementar, dado o desenvolvimento económico, social e tecnológico da maioria dos países em estudo.Conclusão: Apenas Portugal, Brasil e Moçambique adotaram a gestão integrada de doença da asma como principal forma de gestão e controlo da asma. Os programas desenvolvidos por estes países podem servir de modelo nos restantes países em estudo.

Refractory myasthenia gravis: Characteristics of a portuguese cohort.

INTRODUCTION: Some myasthenia gravis (MG) patients are refractory to conventional treatments. METHODS: To describe the clinical features of refractory MG (RMG) and explore the association with human leukocyte antigen HLA-DRB1 alleles, a cohort study of 114 consecutive MG patients was performed. Patients were classified as RMG based on predefined criteria. RESULTS: Twenty-two patients were found to have RMG (19.3%). There were no differences between non-RMG and RMG patients with respect to sex, age of onset, abnormal 3-Hz repetitive nerve stimulation, anti-acetylcholine receptor antibody positivity, thymectomy, thymoma or thymic hyperplasia, and polyautoimmunity. HLA-DRB1*03 was more frequent in the non-RMG vs. control population (P = 3 × 10-6 ). The HLA-DRB1*13 allele was less frequent in non-RMG patients compared with controls (P = 0.002), and less frequent in the non-RMG group compared with the RMG group (P = 0.003). DISCUSSION: HLA-DRB1*03 was more common in non-RMG, and the HLA-DRB1*13 allele appeared to have a protective role, as reported previously in other autoimmune disorders. Muscle Nerve 60: 188-191, 2019.

Case report of recalcitrant allergic contact eczema successfully treated with teriflunomide.

Allergic contact dermatitis (ACD) is a type IV, delayed-type reaction caused by skin contact with low-molecular-weight organic chemicals and metal ions that activate antigen-specific T cells, primarily T-helper 1 (Th1), in a sensitized individual, leading to skin eczema.First-line treatments are based on avoidance of causal agents and topical corticosteroids/immunomodulators. In recalcitrant cases, chronic oral immunosuppressive agents may be used, but they may have serious adverse effects and do not address the immunological disfunction. We report a case of severe ACD, unresponsive to topical or oral immunosuppressive therapy, which resolved itself after treatment with teriflunomide (TF) 14 mg/daily used for multiple sclerosis. TF is a once-daily, oral selective and reversible dihydroorotate dehydrogenase inhibitor, revealing a new treatment option for ACD.