A Challenging Case Of Pulmonary Arterial Hypertension: Never Judge By Appearances

Abstract We report the case of a patient with symptomatic pulmonary hypertension (PH) associated with diffuse systemic sclerosis (SSc) whose initial assessment suggested a group 3 (clinical classification) PH. The patient had a history of drugs/toxins consumption, which contributed to the development of intrinsic pulmonary vascular disease. This changed the panorama towards the diagnosis of pulmonary arterial hypertension (PAH), with important therapeutic and prognostic implications. In fact, the excellent clinical, laboratory and hemodynamic response to therapy confirmed the hypothesis of a case of drug-associated PAH (DPAH) in a patient with diffuse SSc and lung disease. Considering the presence of DPAH, it was deemed necessary to assess acute vasoreactivity during right heart catheterization (RHC). If criteria were met, the clinical scenario may change towards a favorable and sustained clinical and hemodynamic response with oral calcium channel blockers. However, the response to inhaled nitric oxide was negative in our patient and the therapeutic strategy with dual oral combination therapy with tadalafil and ambrisentan was continued. After six-months of therapy the patient significantly improved, from a high to a low risk of one-year mortality.

Assessment of calcinosis in Portuguese patients with systemic sclerosis - a multicenter study.

INTRODUCTION/OBJECTIVES: The study aims to define the clinical and subclinical calcinosis prevalence, the sensitivity of radiographed site and clinical method for its diagnosis, and the phenotype of Portuguese systemic sclerosis (SSc) patients with calcinosis. METHOD: A cross-sectional multicenter study was conducted with SSc patients fulfilling Leroy/Medsger 2001 or ACR/EULAR 2013 classification criteria, registered in the Reuma.pt. Calcinosis was assessed through clinical examination and radiographs of hands, elbows, knees, and feet. Independent parametric or non-parametric tests, multivariate logistic regression, and sensitivity calculation of radiographed site and clinical method for calcinosis detection were performed. RESULTS: We included 226 patients. Clinical calcinosis was described in 63 (28.1%) and radiological calcinosis in 91 (40.3%) patients, of which 37 (40.7%) were subclinical. The most sensitive location to detect calcinosis was the hand (74.7%). Sensitivity of the clinical method was 58.2%. Calcinosis patients were more often female (p = 0.008) and older (p < 0.001) and had more frequently longer disease duration (p < 0.001), limited SSc (p = 0.017), telangiectasia (p = 0.039), digital ulcers (p = 0.001), esophageal (p < 0.001) and intestinal (p = 0.003) involvements, osteoporosis (p = 0.028), and late capillaroscopic pattern (p < 0.001). In multivariate analysis, digital ulcers (OR 2.63, 95% CI 1.02-6.78, p = 0.045) predicted overall calcinosis, esophageal involvement (OR 3.52, 95% CI 1.28-9.67, p = 0.015) and osteoporosis (OR 4.1, 95% CI 1.2-14.2, p = 0.027) predicted hand calcinosis, and late capillaroscopic pattern (OR 7.6, 95% CI 1.7-34.9, p = 0.009) predicted knee calcinosis. Anti-nuclear antibody positivity was associated with less knee calcinosis (OR 0.021, 95% CI 0.001-0477, p = 0.015). CONCLUSIONS: Subclinical calcinosis high prevalence suggests that calcinosis is underdiagnosed and radiographic screening might be relevant. Multifactorial pathogenesis may explain calcinosis predictors' variability. Key Points • Prevalence of subclinical calcinosis in SSc patients is substantial. • Hand radiographs are more sensitive to detect calcinosis than other locations or clinical method. • Digital ulcers were associated with overall calcinosis, esophageal involvement and osteoporosis were associated with hand calcinosis, and late sclerodermic pattern in nailfold capillaroscopy was associated with knee calcinosis. • Anti-nuclear antibody positivity may be a protective factor for knee calcinosis.

Epidemiology of bacteremia in a pediatric population - A 10-year study.

INTRODUCTION: With the widespread introduction of conjugate meningococcal and pneumococcal vaccines, the prevalence and etiology of invasive bacterial infections have changed. We aimed to review all cases of bacteremia in a level II pediatric department over a ten-year period in the post-pneumococcal conjugate vaccine era. METHODS: We reviewed all positive blood cultures (BC) obtained in our department between 2007 and 2016. Results were classified as contaminants, potential pathogens or confirmed pathogens, based on species, number of positive BC in the episode and the patients' medical history. Demographic and clinical data were collected for patients with identified pathogens. RESULTS: A total of 638 positive BC were identified (6.6% of total BC); 120 (1.2%) were considered to represent true bacteremia. The most frequently identified microorganism was Streptococcus pneumoniae (29.2%), with a decrease in the number of cases between 2008 and 2015. Staphylococcus aureus was the second most common organism (19.2%) being 21.7% of these methicillin-resistant. Escherichia coli was the most common isolate in children aged less than three months. CONCLUSION: We found a rate of true bacteremia in children similar to recent studies. Although Streptococcus pneumoniae remains the most common microorganism, its prevalence may be declining. Monitoring microbiological data in children has implications in practice, particularly in local antibiotic prescription.

Epidemiology of bacteremia in a pediatric population – A 10-year study / Epidemiología de la bacteriemia en una población pediátrica: un estudio de 10 años

Introduction: With the widespread introduction of conjugate meningococcal and pneumococcal vaccines, the prevalence and etiology of invasive bacterial infections have changed. We aimed to review all cases of bacteremia in a level II pediatric department over a ten-year period in the post-pneumococcal conjugate vaccine era. Methods: We reviewed all positive blood cultures (BC) obtained in our department between 2007 and 2016. Results were classified as contaminants, potential pathogens or confirmed pathogens, based on species, number of positive BC in the episode and the patients’ medical history. Demographic and clinical data were collected for patients with identified pathogens. Results: A total of 638 positive BC were identified (6.6% of total BC); 120 (1.2%) were considered to represent true bacteremia. The most frequently identified microorganism was Streptococcus pneumoniae (29.2%), with a decrease in the number of cases between 2008 and 2015. Staphylococcus aureus was the second most common organism (19.2%) being 21.7% of these methicillin-resistant. Escherichia coli was the most common isolate in children aged less than three months. Conclusion: We found a rate of true bacteremia in children similar to recent studies. Although Streptococcus pneumoniae remains the most common microorganism, its prevalence may be declining. Monitoring microbiological data in children has implications in practice, particularly in local antibiotic prescription.(AU)

Introducción: Con la introducción generalizada de las vacunas conjugadas meningocócicas y neumocócicas, la prevalencia y la etiología de las infecciones bacterianas invasivas han cambiado. Nuestro objetivo fue revisar todos los casos de bacteriemia en un departamento de pediatría de nivel II durante un período de 10 años en la era de la vacuna conjugada posneumocócica. Métodos: Revisamos todos los hemocultivos (HC) positivos obtenidos en nuestro departamento entre 2007 y 2016. Los resultados se clasificaron como contaminantes, patógenos potenciales o patógenos confirmados, según la especie, el número de HC positivos en el episodio y la historia clínica de los pacientes. Se recopilaron datos demográficos y clínicos de pacientes con patógenos identificados. Resultados: Se identificaron un total de 638 HC positivos (6,6% del total de HC); se consideró que 120 (1,2%) representaban una bacteriemia verdadera. El microorganismo identificado con mayor frecuencia fue Streptococcus pneumoniae (29,2%), con una disminución en el número de casos entre 2008 y 2015. Staphylococcus aureus fue el segundo aislado más común (19,2%); El 21,7% eran resistentes a la meticilina. Escherichia coli fue el aislado más común en niños menores de 3 meses. Conclusión: Encontramos una tasa de bacteriemia verdadera similar a la de estudios recientes. Aunque el Streptococcus pneumoniae sigue siendo el microorganismo más común, su prevalencia puede estar disminuyendo. El seguimiento de datos microbiológicos como este tiene implicaciones en la práctica, especialmente en la prescripción local de antibióticos.(AU)

The safety and persistence of intravenous iloprost in systemic sclerosis.

INTRODUCTION: Vasculopathy is a crucial feature of systemic sclerosis (SSc). It occurs in almost every patient with SSc, with Raynaud's phenomenon (RP) and digital ulcers (DU) having a great impact on the quality of patients' lives. Intravenous (IV) iloprost, a synthetic analogue of prostacyclin, is broadly used to treat RP and DU secondary to SSc. Currently, there is no standard protocol defined for the iloprost treatment of SSc-associated RP and DU, and, consequently, the management of this treatment is largely based on each centre's experience. OBJECTIVE: The objective of this study is to evaluate the safety profile of a particular scheme of IV iloprost used in our centre as the standard treatment of SSc-related vascular complications. METHODS: We retrospectively evaluated the clinical records of SSc patients, classified according to the 2013 European Alliance of Associations for Rheumatology (EULAR) criteria (31) with SSc-related DU and/or severe RP not responsive to CCB, receiving or who have received IV iloprost infusions from January 1st 2011 to March 31st 2021 Results: Within this time frame, 60 patients (n=44 for DU; n=16 for severe RP) were treated with a monthly 10-hour IV iloprost perfusion with a dosing regimen adapted to individual tolerance. Forty-nine of these 60 patients (81.7%) were on iloprost for more than one year. Within 12 months of therapy, 40 patients have healed the DUs (90.9%), with only 4 patients maintaining active DUs. A significant clinical improvement in RP at 12 months was observed in 87.5% (n=14/16) of SSc patients with severe RP. Eleven AE implying treatment dose/frequency adjustments or suspension were recorded (18.3% of patients): severe headache (n=5), hypotension (n=3), tachycardia (n=1), flushing (n=1) and generalised erythroderma (n=1). In all patients, the perfusion rate was reduced in the following treatment sessions with good tolerance, with the exception of the patient with the generalised erythroderma reaction, who suspended the perfusion and was later switched to bosentan. After a mean follow-up time of 6.9 (+/-) 4.0 years of treatment (range 0.06-22), 24 patients (40%) stopped the therapy, 14 (58.3%) of whom due to clinical improvement. The overall 5-, and 10-year survival rates of IV iloprost were 68.2% and 55.6%, respectively. CONCLUSION: SSc patients who received this flexible IV iloprost regimen achieved clinical improvement, reflected in the high persistence rate of the drug, with a good tolerability profile. In addition, most side effects were mild and easily managed.

Clinical features and outcome of 1054 patients with Systemic Sclerosis: analysis of Reuma.pt/SSc registry.

BACKGROUND: Systemic sclerosis (SSc) is a rare connective tissue disorder with heterogeneous manifestations and outcomes. Besides differences in disease characteristics among distinct ethnic groups and geographical regions, several questions regarding the impact of the disease and the effectiveness of treatments remain unanswered. To address these questions, the Rheumatic Diseases Portuguese Register (Reuma.pt) launched a specific protocol for the prospective follow-up of SSc patients. OBJECTIVES: To describe the baseline characteristics, disease subsets, treatments used and survival of SSc patients registered in Reuma.pt/SSc. METHODS: Data from adult patients with SSc included in Reuma.pt up to November 2020 were analysed. Demographic features, SSc subsets, fulfilment of classification criteria, main clinical and immunological features, comorbidities, treatments used and survival data were described and compared between diffuse cutaneous (dc) and limited cutaneous (lc) disease subsets. Survival was calculated for patients included in Reuma.pt within the first two years of diagnosis. RESULTS: In total, 1054 patients were included, 87.5% female, with a mean age at diagnosis of 52.7 +/- 14.8 years. The most common subset was lcSSc (56.3%), followed by dcSSc (17.5%), preclinical SSc (13%), overlap syndrome (9.8%) and SSc sine scleroderma (3.3%). Raynaud's phenomenon (93.4%) and skin thickening (76.9%) were the most frequently observed clinical manifestations. Gastrointestinal (62.8% versus 47.8%), pulmonary (59.5% versus 23%) and cardiac (12.8% versus 6.9%) involvements were significantly more prevalent in dcSSc than lcSSc. Ninety per-cent of patients were Antinuclear antibody positive, 52.5% were Anti-centromere antibody positive and 21% anti-topoisomerase positive, with significant differences between lcSSc and dcSSc. One-third of patients were treated with immunomodulators, 53.6% with vasodilators, 23% with glucocorticoids and 2.3% with biologics. During follow-up, 83 deaths (7.9%) were reported. The overall 1-, 2- and 5-year survivals were 98.0%, 96.8% and 92.6%, respectively, without significant differences between lcSSc and dcSSc. CONCLUSION: Reuma.pt/SSc data highlights the importance of registries in improving knowledge about rare and complex diseases, such as SSc. Clinical features of Portuguese SSc patients are similar to those of other populations. In recently diagnosed patients, 5-year survival is over 92%. To the best of our knowledge, this is the first study showing that clinical features of Portuguese SSc are similar to those of other cohorts.

Gender differences in clinical features and outcomes of a Portuguese systemic sclerosis cohort.

Evidence for the role of sex in the clinical manifestations of systemic sclerosis (SSc) patients is emerging. Some multicenter cohorts have shown that male SSc patients have more severe disease and worse survival. To assess the differences in clinical manifestations and survival in Portuguese SSc patients according to gender. Data from male and female adult SSc patients included in the Rheumatic Diseases Portuguese Register (Reuma.pt) were analysed and compared. Survival was calculated for patients included in Reuma.pt. within the first two years of diagnosis (inception cohort). In total, 1054 adult patients with SSc were included, 12.5% males. No differences in demographic features and comorbidities were found between the sexes, except for a higher rate of cigarette smokers among men. Diffuse cutaneous SSc and anti-topoisomerase antibodies were more prevalent in males than females. Additionally, male patients presented significantly more myositis, interstitial lung disease and gastric involvement. There were no differences in the patterns of drug use between the sexes. During follow-up, more deaths were reported in men than women (12.1% vs 7.3%, p = 0.04). The overall 1-, 3-, and 5-year survivals from diagnosis of the inception cohort (N = 469) for men vs women were 96.4% vs 98.2%, 93% vs 95.9%, and 75.8% vs 93.2%, respectively, with statistically significant differences (p < 0.01). This study confirms the existence of gender differences in clinical and immunological SSc features. Although SSc is less common in men than women, men have a more severe expression of skin and internal organ involvement and worse survival. Key Points • There are differences in SSc disease manifestations between sexes. • Males more commonly have diffuse cutaneous SSc, anti-topoisomerase antibodies, pulmonary and musculoskeletal involvement. • In the inception cohort, men had worse survival rates than women.

Chronic thromboembolic pulmonary hypertension: Initial experience of patients undergoing pulmonary thromboendarterectomy.

INTRODUCTION AND OBJECTIVES: Pulmonary endarterectomy (PEA) is a potentially curative procedure in patients with chronic thromboembolic pulmonary hypertension (CTEPH). This study reports the initial experience of a Portuguese PH center with patients undergoing PEA at an international surgical reference center. METHODS: Prospective observational study of consecutive CTEPH patients followed at a national PH center, who underwent PEA at an international surgical reference center between October 2015 and March 2019. Clinical, functional, laboratory, imaging and hemodynamic parameters were obtained in the 12 months preceding the surgery and repeated between four and six months after PEA. RESULTS: 27 consecutive patients (59% female) with a median age of 60 (49-71) years underwent PEA. During a median follow-up of 34 (21-48) months, there was an improvement in functional class in all patients, with only one cardiac death. From a hemodynamic perspective, there was a reduction in mean pulmonary artery pressure from 48 (42-59) mmHg to 26 (22-38) mmHg, an increase in cardiac output from 3.3 (2.9-4.0) L/min to 4.9 (4.2-5.5) L/min and a reduction in pulmonary vascular resistance from 12.1 (7.2-15.5) uW to 3.5 (2.6-5,2) uW. During the follow-up, 44% (n=12) of patients had no PH criteria, 44% (n=12) had residual PH and 11% (n=3) had PH recurrence. There was a reduction of N-terminal pro-B-type natriureticpeptide from 868 (212-1730) pg/mL to 171 (98-382) pg/mL. Rright ventricular systolic function parameters revealed an improvement in longitudinal systolic excursion and peak velocity of the plane of the tricuspid ring from 14 (13-14) mm and 9 (8-10) cm/s to 17 (16-18) mm and 13 (11-15) cm/s, respectively. Of the 26 patients with preoperative right ventricular dysfunction, 85% (n=22) recovered. The proportion of patients on specific vasodilator therapy decreased from 93% to 44% (p<0.001) and the proportion of those requiring oxygen therapy decreased from 52% to 26% (p=0.003). The six-minute walk test distance increased by about 25% compared to the baseline and only eight patients had significant desaturation during the test. CONCLUSION: Pulmonary endarterectomy performed at an experienced high-volume center is a safe procedure with a very favorable medium-term impact on functional, hemodynamic and right ventricular function parameters in CTEPH patients with operable disease. It is possible for PH centers without PEA differentiation to refer patients safely and effectively to an international surgical center in which air transport is necessary.

Ten years of a systemic sclerosis clinic in a tertiary referral centre - insights and future directions.

Systemic sclerosis (SSc) is an uncommon condition, with a wide range of manifestations, characterized by specific antibody production, vasculopathy and fibrosis of the skin and other internal organs. It is a complex disease, which is estimated to be rare in Portugal, although specific incidence data are missing. The aetiology of SSc remains unknown, but is likely to be multifactorial, involving genetic and environmental aspects. Its management is challenging and often requires a multidisciplinary approach. In 2011, we established a dedicated outpatient clinic for patients with SSc. Clinical data of every patient with a confirmed diagnosis of SSc is prospectively registered in Reuma.pt/SSc. In this manuscript, we aim to describe the general functioning of our SSc outpatient clinic, and to characterise the population of patients with SSc who are followed herein.

Chronic thromboembolic pulmonary hypertension: initial experience of patients undergoing pulmonary thromboendarterectomy. / Hipertensão pulmonar tromboembólica crónica: experiência inicial de doentes submetidos a tromboendarterectomia pulmonar.

INTRODUCTION AND OBJECTIVES: Pulmonary endarterectomy (PEA) is a potentially curative procedure in patients with chronic thromboembolic pulmonary hypertension (CTEPH). This study reports the initial experience of a Portuguese PH center with patients undergoing PEA at an international surgical reference center. METHODS: Prospective observational study of consecutive CTEPH patients followed at a national PH center, who underwent PEA at an international surgical reference center between October 2015 and March 2019. Clinical, functional, laboratory, imaging and hemodynamic parameters were obtained in the 12 months preceding the surgery and repeated between four and six months after PEA. RESULTS: 27 consecutive patients (59% female) with a median age of 60 (49-71) years underwent PEA. During a median follow-up of 34 (21-48) months, there was an improvement in functional class in all patients, with only one cardiac death. From a hemodynamic perspective, there was a reduction in mean pulmonary artery pressure from 48 (42-59) mmHg to 26 (22-38) mmHg, an increase in cardiac output from 3.3 (2.9-4.0) L/min to 4.9 (4.2-5.5) L/min and a reduction in pulmonary vascular resistance from 12.1 (7.2-15.5) uW to 3.5 (2.6-5, 2) uW. During the follow-up, 44% (n=12) of patients had no PH criteria, 44% (n=12) had residual PH and 11% (n = 3) had PH recurrence. There was a reduction of N-terminal pro-B-type natriureticpeptide from 868 (212-1730) pg/mL to 171 (98-382) pg/mL. Rright ventricular systolic function parameters revealed an improvement in longitudinal systolic excursion and peak velocity of the plane of the tricuspid ring from 14 (13-14) mm and 9 (8-10) cm/s to 17 (16-18) mm and 13 (11-15) cm/s, respectively. Of the 26 patients with preoperative right ventricular dysfunction, 85% (n=22) recovered. The proportion of patients on specific vasodilator therapy decreased from 93% to 44% (p<0.001) and the proportion of those requiring oxygen therapy decreased from 52% to 26% (p=0.003). The six-minute walk test distance increased by about 25% compared to the baseline and only eight patients had significant desaturation during the test. CONCLUSION: Pulmonary endarterectomy performed at an experienced high-volume center is a safe procedure with a very favorable medium-term impact on functional, hemodynamic and right ventricular function parameters in CTEPH patients with operable disease. It is possible for PH centers without PEA differentiation to refer patients safely and effectively to an international surgical center in which air transport is necessary.

Epidemiology of bacteremia in a pediatric population - A 10-year study.

INTRODUCTION: With the widespread introduction of conjugate meningococcal and pneumococcal vaccines, the prevalence and etiology of invasive bacterial infections have changed. We aimed to review all cases of bacteremia in a level II pediatric department over a ten-year period in the post-pneumococcal conjugate vaccine era. METHODS: We reviewed all positive blood cultures (BC) obtained in our department between 2007 and 2016. Results were classified as contaminants, potential pathogens or confirmed pathogens, based on species, number of positive BC in the episode and the patients' medical history. Demographic and clinical data were collected for patients with identified pathogens. RESULTS: A total of 638 positive BC were identified (6.6% of total BC); 120 (1.2%) were considered to represent true bacteremia. The most frequently identified microorganism was Streptococcus pneumoniae (29.2%), with a decrease in the number of cases between 2008 and 2015. Staphylococcus aureus was the second most common organism (19.2%) being 21.7% of these methicillin-resistant. Escherichia coli was the most common isolate in children aged less than three months. CONCLUSION: We found a rate of true bacteremia in children similar to recent studies. Although Streptococcus pneumoniae remains the most common microorganism, its prevalence may be declining. Monitoring microbiological data in children has implications in practice, particularly in local antibiotic prescription.

[Venous Thromboembolism in Pediatric Age: A 15 Year Retrospective Review]. / Tromboembolismo Venoso em Idade Pediátrica: Estudo Retrospetivo de 15 Anos.

INTRODUCTION: Pulmonary thromboembolism and deep venous thrombosis occur in pediatric age, with unknown incidence, morbidity and mortality. Our aim is to review the epidemiology, clinical presentation, complementary diagnostic tests and prognosis of patients with pulmonary thromboembolism and deep venous thrombosis. MATERIAL AND METHODS: Retrospective, descriptive and analytical study of pediatric patients admitted to a Level II hospital for pulmonary thromboembolism and deep venous thrombosis, between 2000 and 2014. Demographic characteristics, clinical history, comorbidities and risk factors were studied. RESULTS: Eleven patients (n = 7 pulmonary thromboembolism, n = 5 deep venous thrombosis, n = 1 both), 64% females and with 16 years old average, were admitted. All patients with pulmonary thromboembolism presented symptoms of chest pain and/or dyspnea, 25% syncope/palpitations and 25% fever. All patients with deep venous thrombosis reported localized pain at the site of obstruction, 83% edema/cyanosis of the affected limb and 17% fever. The study of positive thrombophilia was the most frequent risk factor in both entities. The mean value of D-dimers was 3252 ug/dL and 2660 ug/dL in pulmonary thromboembolism and deep venous thrombosis, respectively. All patients started anticoagulation, three required intensive care, two had sequelae and one died. DISCUSSION: All patients had at least one risk factor, and hereditary hypercoagulability was most commonly established. CONCLUSIONS: The increased incidence in the pediatric population described in some studies can be attributed to an increased awareness of this pathology, medical advances and increasing survival of chronic diseases. There is a lack of evidence-based recommendations identifying patients at risk of thrombosis so that decisions can be made carefully, balancing the risk and benefit in each case.

Introdução: O tromboembolismo pulmonar e trombose venosa profunda ocorrem em idade pediátrica com incidência, morbilidade e mortalidade desconhecidas. O objetivo foi rever epidemiologia, apresentação clínica, exames complementares de diagnóstico e prognóstico de doentes com tromboembolismo pulmonar e trombose venosa profunda.Material e Métodos: Estudo retrospetivo, descritivo e analítico de doentes pediátricos internados num hospital de nível II por tromboembolismo pulmonar e trombose venosa profunda, entre 2000 e 2014. Estudaram-se características demográficas, história clínica, comorbilidades e fatores de risco.Resultados: Foram internados 11 doentes (sete com tromboembolismo pulmonar, cinco com trombose venosa profunda e um com ambos), 64% do género feminino e idade média de 16 anos. Todos os doentes com tromboembolismo pulmonar referiam toracalgia/dispneia, 25% síncope/palpitações e 25% febre. Todos os doentes com trombose venosa profunda referiam dor no local da obstrução, 83% edema/cianose do membro afetado e 17% febre. O estudo da trombofilia positivo foi o fator de risco mais frequente nas duas entidades. O valor médio dos D-dímeros foi 3252 ug/L e 2660 ug/L no tromboembolismo pulmonar e trombose venosa profunda, respetivamente. Todos os doentes iniciaram anticoagulação, três necessitaram de cuidados intensivos, três apresentaram sequelas e houve um óbito.Discussão: Todos os doentes tinham pelo menos um fator de risco associado e as condições de hipercoagulabilidade herdadas foram o fator de risco mais frequentemente encontrado nos nossos adolescentes.Conclusão: O aumento da incidência na população pediátrica descrito na literatura pode ser atribuído à crescente sensibilização para esta patologia, aos avanços médicos e aumento da sobrevida de doenças crónicas. Escasseiam recomendações baseadas na evidência que identifiquem os doentes com risco de trombose, para que as decisões possam ser tomadas de forma cuidadosa, equilibrando o risco e benefício em cada caso.

Recém-nascidos com risco de toxoplasmose congênita, revisão de 16 anos / Newborns at risk for congenital toxoplasmosis, review of 16 years

OBJETIVOS: Analisar os dados de gestações com risco de toxoplasmose congênita e investigar a evolução dos recém-nascidos, em um hospital de nível II em Portugal. MÉTODOS: Um estudo transversal retrospetivo incluiu recém-nascidos com risco de toxoplasmose congênita e suas mães, cujo parto ocorreu entre janeiro de 2000 e dezembro de 2015. Os critérios de inclusão foram mãe com soroconversão para toxoplasmose durante a gestação ou primeira amostra sérica com IgM e IgG específicas positivas. O diagnóstico de toxoplasmose congênita foi definido por IgM específica positiva ao nascimento e/ou reação em cadeia da polimerase positiva no líquido amniótico e/ou persistência de IgG específica até os 12 meses de vida. A toxoplasmose congênita foi definida como sintomática quando os achados clínicos foram atribuídos à doença. Os testes Qui-quadrado ou Exato de Fisher foram usados para testar associações entre variáveis, assumindo-se significado estatístico quando p<0,05. RESULTADOS: Ocorreram 39.585 nascimentos vivos no período em estudo e foram identificados 98 casos com risco de toxoplasmose congênita, dos quais 89 completaram o seguimento. A prevalência de IgG para T. gondii nas gestantes foi de 26% (intervalo de confiança [IC] 95% 24-27%). Foram confirmados 22 casos de toxoplasmose congênita (5,6 por 10.000 nascidos vivos, IC95% 3,5-8,5 por 10.000). Dos 22 recém-nascidos, 18 (82%, IC95% 61-93%) eram sintomáticos. Os achados clínicos mais frequentes foram calcificação intracraniana (64%), hepatomegalia e/ou elevação das transaminases (32%) e retinocoroidite (14%). As lesões cerebrais foram mais frequentes quando a infeção materna foi documentada no primeiro e segundo trimestres em comparação com o terceiro (p=0,018). Em 31 casos (35%), as gestantes foram tratadas desde o momento do diagnóstico até o parto com espiramicina, não se tendo encontrado diferenças relativamente à taxa de transmissão vertical ou ao aparecimento de manifestações clínicas entre os recém-nascidos de mães tratadas e não tratadas. CONCLUSÕES: A prevalência de toxoplasmose congênita foi superior à reportada em outros países da Europa. A prevalência de calcificações intracranianas foi maior do que a descrita na literatura, enquanto que a de retinocoroidite e estrabismo foi menor. As alterações cerebrais nos recém-nascidos foram mais frequentes nas infecções de primeiro e segundo trimestre. Não houve diferença na taxa de transmissão vertical e na ocorrência de manifestações clínicas entre os recém-nascidos cujas mães receberam espiramicina na gestação ou não receberam tratamento.

AIMS: To analyze the data on pregnancies with risk of congenital toxoplasmosis and to investigate the newborns' outcome, in a level II hospital in Portugal. METHODS: A cross-sectional retrospective study included newborns at risk for congenital toxoplasmosis and their mothers, whose delivery occurred between January 2000 and December 2015. The inclusion criteria were mother with seroconversion to toxoplasmosis during pregnancy or first serum sample with positive specific IgM and IgG. The diagnosis of congenital toxoplasmosis was defined by positive specific IgM at birth and/or polymerase chain reaction positive in amniotic fluid and/or persistence of specific IgG up to 12 months of life. Congenital toxoplasmosis was defined as symptomatic when clinical findings were attributed to the disease. Chi-square or Fisher's exact tests were used to test associations between variables, assuming statistical significance when p<0.05. RESULTS: There were 39,585 live births in the study period and 98 cases with risk of congenital toxoplasmosis were identified, of which 89 completed the follow-up. The prevalence of anti-T. gondii IgG in the pregnant women was 26% (95% confidence interval [CI] 24-27%). Twenty-two cases of congenital toxoplasmosis (5.6 per 10,000 live births, 95%CI 3.5-8.5 per 10,000) were confirmed. Of the 22 newborns, 18 (82%, 95%CI 61-93%) were symptomatic. The most frequent clinical findings were intracranial calcification (64%), hepatomegaly and / or elevation of transaminases (32%) and retinochoroiditis (14%). Cerebral lesions were more frequent when maternal infection was documented in the first and second trimester compared to the third trimester (p=0.018). In 31 cases (35%), the pregnant women were treated from the time of diagnosis until delivery with spiramycin, and no differences were found regarding the rate of vertical transmission or the appearance of clinical manifestations among the newborns of treated and non-treated mothers treated. CONCLUSIONS: The prevalence of congenital toxoplasmosis was higher than that reported in other European countries. The prevalence of intracranial calcifications was higher than that described in the literature, whereas retinochoroiditis and strabismus occurrence was lower. Brain lesions in newborns were more frequent in first- and second-trimester infections. There was no difference in the rate of vertical transmission and in the occurrence of clinical manifestations among newborns whose mothers received spiramycin during gestation and those whose mothers did not receive treatment.

Que lugar para a corporeidade no cenário dos saberes e práticas psis ? / What place for corporeality in the scene of psis knowledge and practices?

Resumo O presente artigo realizado por pesquisadores do Laboratório de Corporeidade e Subjetividade (CORPOREILABS - Universidade Federal Fluminense [UFF], Faculdade Angel Vianna [FAV], Universidade Federal do Rio de Janeiro [UFRJ], Universidade Federal do Ceará [UFC]) surge da necessidade de explicitar o tema da corporeidade nos saberes e práticas psis na contemporaneidade. O sujeito moderno emerge de uma radical experiência de separação eu-mundo e a subjetividade, entendida por uma perspectiva representacional, sofre uma cisão dos processos sensíveis do corpo. No intuito de afirmar uma outra política de percepção a respeito da subjetividade, afirma-se nesse artigo a necessidade de incluir nos estudos da subjetividade sua relação intrínseca com os processos de corporeidade. Desde esta perspectiva se abre uma clínica e um modo de pesquisar como processualidades, em que a dimensão sensorial, ética, estética e política são convocadas.(AU)

Abstract The present article carried out by researchers of the Laboratory of Corporeality and Subjectivity (CORPOREILABS - Fluminense Federal University [UFF], Angel Vianna College [FAV], Federal University of Rio de Janeiro [UFRJ], Federal University of Ceará [UFC]) arises from the need to explain the subject of corporeity in Psis knowledges and practices in the contemporary world. The modern subject emerges from a radical experience of separation from the world and subjectivity, understood by a representational perspective, undergoes a split of the sensory processes of the body. To affirm another policy of perception regarding subjectivity, it is stated in this article the need to include in the studies of subjectivity its intrinsic relation with the processes of corporeality. From this perspective opens a clinic and a way of researching as processualities, in which the sensorial, ethical, aesthetic and political dimension are summoned.(AU)

Corposições entre o ver, o dizer e o agir / Corposições between seeing, saying and acting

Resumo Este artigo enuncia as questões que mobilizam o coletivo de investigação "Corposições: Profanações entre afeto, performatividade e território", explicitando o "como", "onde" e "quando" os campos de atuação de seus integrantes se imbricam para construir um corpo comum. A partir de suas principais ferramentas de trabalho - a Composição em Tempo Real de João Fiadeiro e o Modo Operativo AND de Fernanda Eugenio - o jogo proposto pelo grupo é o de partilhar suas experiências, dando visibilidade ao que desestabiliza, ventila e amplia suas práticas e discursos. Tal proposição implica modos de ver específicos - a articulação entre corporeidade, afeto, território e performatividade - e modos de agir - uma investigação que é experimentação e laboratório, na qual são performadas intervenções entre dança e clínica.(AU)

Abstract This article states the questions that mobilize the research group "Corposições: Profanations between affection, performativity and territory". It highlights "how", "where" and "when" the fields of action of its members imbricate to build up a common body. Considering its main work tools - João Fiadeiro's Real Time Composition and Fernanda Eugenio's AND Operative Mode - the game proposed by the group is to share their experiences, giving visibility to what destabilizes, ventilates and broadens its practices and discourses. This proposition implies specific ways of seeing - the articulation between corporeity, affection, territory and performativity - and ways of acting - an investigation which is experimentation and laboratory where interventions between dance and clinic are performed.(AU)