Abstract Epithelioid hemangioendothelioma is a very rare vascular neoplasm, which is often multifocal or metastatic at diagnosis. Most frequently arises in the liver, followed by the lung and bones. The authors present a case of a liver transplant recipient who developed a pattern of hepatic cholestasis associated with the appearance of a proliferative hepatic lesion with infiltrative growth. Histological examination and immunohistochemical study were compatible with the diagnosis of epithelioid hemangioendothelioma. Pulmonary micronodules were detected and lung metastases were hypothesized. Therefore, bronchoscopy was performed, which turned out to be normal, and cytology was negative for neoplastic cells. After a multidisciplinary discussion, liver retransplantation was decided. After 8 years of follow-up, the patient is clinically stable, with no graft dysfunction, no neoplastic recurrence, and dimensional stability of the pulmonary micronodules. Patients with organ transplant have higher risk of developing carcinoma compared to the general population. The development of cancer is a multifactorial process and little is known about the etiology of epithelioid hemangioendothelioma. No standard treatment strategy has been defined yet, and the natural course of the disease is heterogenous and the individual prognosis unpredictable. Complete surgical resection is offered to patients with unifocal disease, and those with unresectable disease should be evaluated for orthotopic liver transplantation.

Resumo O hemangioendotelioma epitelióide é uma neoplasia vascular extremamente rara, muitas vezes multifocal ou metastática ao diagnóstico. O local mais frequente afetado é o fígado, seguido pelo pulmão e ossos. Os autores apresentam o caso de uma doente com antecedentes de transplante hepático que desenvolveu um padrão de colestase associado ao aparecimento de uma lesão hepática proliferativa e de crescimento infiltrativo. O exame histológico e o estudo imuno-histoquímico foram compatíveis com hemangioendotelioma epitelióide. Foram detetados micronódulos pulmonares, tendo sido colocada a hipótese de se tratarem de metástases pulmonares. Assim, foi realizada broncoscopia, que não revelou alterações, estando a citologia negativa para células neoplásicas. Após discussão multidisciplinar, foi decidido o retransplante hepático. Após 8 anos de seguimento, a doente encontra-se clinicamente estável, sem disfunção do enxerto, sem recidiva neoplásica e com estabilidade dimensional dos micronódulos pulmonares. Doentes submetidos a transplante têm maior risco de desenvolver neoplasias em comparação com a população geral. O desenvolvimento da neoplasia é um processo multifatorial, sendo a etiologia do hemangioendotelioma epite-lióide ainda pouco compreendida. Não existe uma estratégia terapêutica standard, sendo o curso natural da doença heterogêneo e o prognóstico individual imprevisível. A ressecção cirúrgica é a primeira opção terapêu-tica nos doentes com doença unifocal, aqueles com doença irressecável devem ser avaliados para transplante hepático.

Resumo Introdução: A colonoscopia é eficaz a detetar e remover lesões do colon e reto. Contudo, após uma colonoscopia normal, podem ser detetadas neoplasias durante o intervalo de vigilância recomendado entre colonoscopias. O objetivo do estudo foi identificar características e fatores de risco para o desenvolvimento de cancro colorretal pós-colonoscopia - subtipo de intervalo. Material e Métodos: Estudo retrospetivo e unicêntrico realizado entre janeiro de 2018 e dezembro de 2019 que incluiu todos os doentes diagnosticados de novo com cancro colorretal. Variáveis clínicas, demográficas e endoscópicas foram obtidas após consulta do processo clínico. Doentes com diagnóstico de cancro colorretal entre duas colonoscopias consecutivas, realizadas no intervalo de vigilância recomendado, foram considerados como tendo cancro colorretal pós-colonoscopia - subtipo de intervalo. Foi, então, realizada a comparação entre o grupo de doentes com cancro color-retal não pós colonoscopia - subtipo de intervalo e o grupo de doentes com cancro colorretal pós colonoscopia - subtipo de intervalo. Resultados: Durante o período de estudo, 491 doentes foram diagnosticados de novo com cancro colorretal. Destes, 61 (12.4%) foram considerados como tendo cancro colorretal pós-colonoscopia - subtipo de intervalo. O cancro colorretal pós-colonoscopia - subtipo de intervalo foi três vezes mais prevalente no colon proximal (p = 0.014) e associou-se a presença de dois ou mais fatores de risco cardiovasculares (aOR = 0.45; p = 0.016), colecistectomia no passado (aOR = 10.09; p = 0.0.19) e história familiar de cancro colorretal num familiar de primeiro grau (aOR = 4.25; p = 0.006). Aquando da análise dos fatores de risco cardiovasculares isolados, observou-se um fator protetor aquando da ausência de to-dos os fatores de risco cardiovasculares (aOR = 20; p = 0.034). A curva ROC associada ao modelo multivariado revelou um poder preditivo de 77.8% (p < 0.001). Conclusão: O cancro colorretal pós-colonoscopia - subtipo de intervalo é mais comum no colon proximal e em doentes com história familiar (em familiares de primeiro grau) de cancro colorretal, dois ou mais fatores de risco cardiovasculares e história de colecistectomia. Todos estes fatores de risco são facilmente detetáveis na prática clínica e podem ser de extrema importância no controlo, a curto e longo prazo, do cancro colorretal pós-colonoscopia.

Abstract Background and Aims: Colonoscopy is effective to detect and remove colorectal lesions. However, after a negative colonoscopy, cancers could be detected during the interval follow-up. This study was designed to identify characteristics and risk factors for postcolonoscopy colorectal cancer - interval type. Methods: Medical records of individuals who were newly diagnosed with colorectal cancer between January 2018 and December 2019 were reviewed. Clinical, demographic, and endoscopic variables were analyzed. Those with the diagnosis of colorectal cancer between two consecutive colonoscopies performed within the appropriated surveillance range were considered to have postcolonoscopy colorectal cancer - interval type. A comparison between the group of patients with non-postcolonoscopy colorectal cancer - interval type and the group of patients with post-colonoscopy colorectal cancer - interval type was then performed. Results: During the study period, 491 patients were newly diagnosed with colorectal cancer. Among them, 61 (12.4%) had postcolonoscopy colorectal cancer - interval subtype. Postcolonoscopy colorectal cancer - interval type was three times more prevalent on the proximal colon (p = 0.014) and was associated with the presence of two or more cardiovascular risk factors (aOR = 4.25; p = 0.016), cholecystectomy in the past (aOR = 10.09; p = 0.019), and family history of colorectal cancer on a first-degree relative (aOR = 4.25; p = 0.006). Moreover, isolated cardiovascular risk factors revealed a protective effect for the absence of all cardiovascular risk factors (aOR = 20; p = 0.034). The ROC curve associated with the multivariate model revealed a predictive power of 77.8% (p < 0.001). Conclusions: Postcolonoscopy colorectal cancer - interval type is more common in the proximal colon and in patients with a family history (first-degree relative) of colorectal cancer, two or more cardiovascular risk factors, and a history of cholecystectomy. All of these are easily detectable in clinical practice and may be of extreme importance in the control of postcolonoscopy colorectal cancer in the near future.

ABSTRACT Introduction: Healthcare facilities are among the greatest contributors to resource consumption and waste generation. Nephrology is one of the most polluting medical areas, mostly due to hemodialysis (HD). Incremental hemodialysis (iHD) has gained attention due to several clinical advantages, by decreasing the number of sessions while the patient still has residual kidney function. Beyond these benefits, this regímen might also be both more economical and environmentally friendly. The aim of this study was to evaluate the potential reduction of resource consumption, waste generation and carbon footprint associated with the implementation of iHD. Methods: We conducted a single-center retrospective analysis of 157 incidental patients initiating HD in our centre in 2019 (pre-pandemic). Retrospectively, a set of criteria identifying eligibility for an iHD program were applied to those patients. Results: Twenty-three patients (15%) would have been eligible for iHD instead of starting a standard HD program. In that scenario, iHD implementation would have reduced from 36 to 85 tons of CO2 equivalent emissions and 4186 kg of contaminated waste yearly, at our unit. iHD would also allow savings of almost 60 000€ and a reduction in water consumption of 418 600 L in one year. Conclusion: This study shows that iHD can significantly attenuate the environmental and economic impact of HD. These findings encourage kidneycare programs and policymakers to adopt greener options but should never be detrimental to the clinical decision of referencing a patient to iHD.

ABSTRACT The rising prevalence of patients living with kidney disease and the recognition of the huge environmental impact of kidney care (mainly dialysis treatments) points to the unsustainability of nephrology in the near future, which has forced the nephrology field to define its sustainability as a priority for this century. In order to face this issue, nephrologists need to recognize the dimension of the environmental footprint of their activities and what topics are associated with a bigger carbon footprint or resource consumption. Nephrologists should be in the center and the driving force for the changes needed.

ABSTRACT Introduction: Healthcare services have a significant environmental impact (EI). The increasing prevalence of chronic kidney disease makes nephrology one of the clinical areas with higher EI. The aim of this study was to document Portuguese nephrologists’ current practice regarding environmental sustainability. Methods: Two surveys were developed: one for chiefs of departments and another for all nephrologists registered in the Portuguese Society of Nephrology. Results: There were 153 responders (25.5% response rate): 17 (11%) were chiefs of departments, 36 (23.5%) nephrologists in coordination roles, 60 (39%) nephrologists and 40 (26%) nephrology fellows. The median priority attributed to environmental sustainability was 4 (IQR 3-5). Only one department had established a green group, but 13 were currently undertaking at least one green project (76.5%). Only 6% of responders (8/136) had received formal training on this topic, but more than 90% would like to have more information. The most reported barriers to environmental sustainability practices were inadequate information, lack of adequate facilities, concerns about security and legal norms. Only two facilities (11.8%) declared that environmental sustainability was considered in procurement decisions. More than 70% of the Units did not have regular access to any kind of environmental sustainability audit. Only 3 surveyed units (17.6%) have set up systems to reuse the reverse osmosis rejected water and only half had local on-line dialysate production (9/17, 53%). Conclusion: Portuguese nephrologists wish to incorporate environmental sustainability in their daily practice, however there is a big gap between their intention and current behavior, likely because of the identified barriers. Keywords: Environment; Medical Waste; Nephrology; Renal Dialysis

Resumo Introdução: Danos tubulares são comuns em doenças glomerulares (DG). Glicosúria é um marcador de disfunção tubular e pode detectar lesão tubular e progressão da DRC. O objetivo deste estudo foi avaliar a prevalência e o valor prognóstico da glicosúria no diagnóstico em glomerulopatias primárias (GP). Métodos: Realizamos estudo retrospectivo de 24 meses em pacientes diagnosticados com GP em nosso centro entre 2009-2020. Excluímos pacientes diabéticos, uso de inibidores de SGLT2, pacientes transplantados e DG secundárias. Os pacientes dividiram-se em dois grupos de acordo com seu estado de glicosúria no diagnóstico. Resultados: Estudamos 115 pacientes. A prevalência global de glicosúria foi de 10% (n=11) e a nefropatia membranosa (NM) teve maior prevalência (n=5, 17,9%). Constatamos que pacientes com glicosúria apresentavam creatinina sérica mais elevada (2,4 vs. 1,2 mg/dL, p=0,030), albuminúria mais alta (4,8 vs. 1,9 g/g, p=0,004), e albumina sérica mais baixa (2,3 vs. 3,2 g/dL, p=0,021). Não encontramos associação com fatores prognósticos histológicos. Ao final do acompanhamento, pacientes com glicosúria tiveram maior prevalência do desfecho composto de DRC estágio 5D ou aumento de 50% na CrS basal (45,5% vs. 17,3%, p=0,037). Em pacientes com NM, os resultados foram semelhantes, mas encontramos uma associação de glicosúria com fibrose intersticial mais grave e atrofia tubular (25,0 vs. 0,0 %, p=0,032). Conclusão: 10% de nossos pacientes com GP têm glicosúria. A glicosúria no diagnóstico foi associada a uma apresentação clínica mais grave e pior desfecho renal. A associação com albuminúria mais elevada sugere que a função tubular tem um impacto na gravidade e nos desfechos da GP.

Abstract Introduction: Tubular damage is common in glomerular diseases (GD). Glycosuria is a marker of tubular dysfunction and may be used to detect tubular lesion and CKD progression. The aim of this study was to evaluate the prevalence and prognostic value of glycosuria at the time of diagnosis in primary glomerulopathies (PG). Methods: We conducted a 24-month retrospective study in patients diagnosed with PG in our center between 2009 and 2020. We excluded diabetic patients, use of SGLT2 inhibitors, transplant patients, and secondary GD. Patients were divided in two groups according to their glycosuria status at diagnosis. Results: We studied 115 patients. Global prevalence of glycosuria was 10% (n=11) and membranous nephropathy (MN) had the highest prevalence (n=5, 17.9%). We found that patients with glycosuria had higher serum creatinine (2.4 vs. 1.2 mg/dL, p=0.030), higher albuminuria (4.8 vs. 1.9 g/g, p=0.004), and lower serum albumin (2.3 vs. 3.2 g/dL, p=0.021). We did not find association with histological prognostic factors. At the end of follow-up, patients with glycosuria had higher prevalence of the composite outcome of stage 5D CKD or 50% increase in basal SCr (45.5% vs. 17.3%, p=0.037). In patients with MN, results were similar but we were able to find an association of glycosuria with more severe interstitial fibrosis and tubular atrophy (25.0 vs. 0.0 %, p=0.032). Conclusion: Ten percent of our patients with PG have glycosuria. Glycosuria at the time of diagnosis was associated with more severe clinical presentation and worst renal outcome. The association with higher albuminuria suggests that tubular function has an impact on the severity and outcomes of PG.

Resumo: Introdução: A doença por coronavirus 19 (COVID-19) é uma pandemia global que afetou gravemente os serviços de saúde em todo o mundo. Durante o estado de emergência declarado em Portugal nos meses de Março e Abril de 2020, verificou-se uma redução importante na atividade médica. Este estudo teve como objetivo avaliar o impacto da COVID-19 nos Serviços de Gastroenterologia portugueses e as estratégias implementadas para fazer face a este desafio. Métodos: Tratou-se de um estudo transversal baseado num inquérito online. Um questionário pormenorizado acerca de diferentes aspetos da atividade dum Serviço de Gastroenterologia foi enviado por email para os Diretores de Serviço de Gastroenterologia dos Hospitais Distritais. Foram avaliados dois períodos: o estado de emergência e o período de recuperação entre maio e setembro. Foram registadas as respostas entre setembro e outubro 2020. Resultados: Responderam 21 Hospitais (taxa de resposta: 80,8%). Vinte e oito porcento dos inquiridos tiveram profissionais da sua unidade infectados com COVID-19. Pelo menos um elemento foi mobilizado para outro local de trabalho em 66,7% dos hospitais, maioritariamente internos de formação específica de Gastroenterologia. Durante o estado de emergência, 47,6% dos Hospitais só realizaram endoscopias urgentes/emergentes. Em 38,5%, a necessidade de racionamento de equipamento de proteção individual levou à suspensão do treino em endoscopia. Relativamente ao período de recuperação, os procedimentos não urgentes foram retomados na maioria dos centros. O mesmo se verificou para o programa de rastreio do cancro colorectal. Salienta-se que 81% dos inquiridos confirmaram que adiaram exames a pedido dos doentes por “medo de ficarem infetados”. Mantiveram-se consultas não-presenciais em 81% dos Hospitais. De modo geral, as atividades de formação dos internos foram progressivamente retomadas. Discussão/Conclusão: Este estudo oferece o retrato do impacto e consequências da primeira vaga da pandemia nos Hospitais portugueses. É importante compreender como os Serviços de Gastrenterologia nacionais lidaram com o primeiro impacto da COVID-19 e que estratégias foram implementadas de forma a melhor preparar o que se segue.

Abstract: Introduction: Coronavirus disease 2019 (COVID-19) is a global pandemic that has severely affected health care systems around the world. During the emergency state declared in Portugal in the months of March and April 2020, there was a severe reduction in medical activity in order to reduce the pressure on health systems. This study aimed to assess the impact of COVID-19 in gastroenterology departments across Portugal and the strategies developed to overcome this challenge. Methods: This was a cross-sectional study based on an online survey. A detailed questionnaire concerning different aspects of gastroenterology department activity was sent via e-mail to the heads of gastroenterology departments of Portuguese District Hospitals (Núcleo de Gastroenterologia dos Hospitais Distritais). Two periods were assessed, i.e., the emergency state and the recovery period between May and September. The responses were collected between September and October 2020. Results: A total of 21 hospitals were enrolled (80.8% response rate). Twenty-eight percent of the responders reported healthcare professionals from their unit infected with COVID-19. At least 1 member (mostly fellows) of the department was deployed to another workplace in 66.7% of the hospitals. During the emergency state, 47.6% of the hospitals only performed urgent/emergent endoscopic procedures. In 38.5% of the hospitals the need to ration personal protective equipment led to the suspension of endoscopic training. Regarding the recovery period, nonurgent procedures were restarted in almost all of the centers. The same was reported for the colorectal cancer screening program. Remarkably, 81% of the responders confirmed that they had postponed procedures at patients’ request for “fear of getting infected.” Remote consultation was maintained in 81% of the hospitals. Globally, the fellows had resumed their training. Discussion/Conclusion: This study provides a snapshot of the impact and consequences of the first wave of the COVID-19 pandemic across Portuguese hospitals. It is important to understand how the gastroenterology world dealt with the first impact of COVID-19 and what strategies were implemented in order to better prepare for what might follow.

Introduction: Retroperitoneal fibrosis is a rare disorder. The idiopathic (IRF) form of the disease accounts for most of cases. Renal failure is a common finding, and some patients present with non-functioning kidneys, as a result of long-lasting obstructive uropathy. The symptoms and signs associated with IRF are non-specific and, thus, its diagnosis requires a high degree of suspicion. Case Report: A 71-year-old man with multifactorial chronic kidney disease required initiation of haemodialysis on February 2014, due to disease progression to end-stage renal disease (ESRD). His past medical history included the diagnosis of optic neuritis, seronegative arthritis and biopsy-proven retroperitoneal fibrosis. The patient had been treated with glucocorticoids for 13 years, stopping this treatment at the time of haemodialysis (HD) initiation. Two months after initiation of HD, in April 2014, the patient complained of malaise and presented with recurrent fever. A thorough clinical and laboratory evaluation was unremarkable. An echocardiogram showed a minor to moderate pericardial effusion that led to prescription of HD intensification. Five months later, he was admitted to the Hospital due to worsening of the pericardial effusion, requiring pericardiocentesis. Pericardial fluid analysis showed exudative changes. A thorough laboratory evaluation was performed and only interferon gamma release assay (IGRA) was positive. Empirical anti-bacillary therapy was initiated, waiting for definite mycobacterial cultures. One month later, as the patient maintained complains of malaise and fatigue, and had recurrent fever, he was readmitted to the Hospital. Mycobacterial cultures ultimately became negative. Further work-up was performed, including positron emission tomography/computed tomography (PET/CT) scan, which detected recurrence of IRF along with aortic, pericardial and pleural involvement. Following initiation of glucocorticoid treatment, a clinical and laboratoryl improvement was documented, that persisted at 11 months of follow-up. Conclusion: IRF is characterized by a relapsing nature, and clinical presentation includes local and systemic symptoms and complications, making it difficult to diagnose. Treatment of IRF is often delayed because of difficulties in reaching the correct diagnosis

A diálise peritoneal é uma opção válida para o tratamento da doença renal crónica estádio 5. Como este procedimento provoca um aumento da pressão intra-abdominal, pode causar uma “fuga” pleuroperitoneal (hidrotórax). Esta complicação não infecciosa, sendo rara, pode ser fatal para doentes em diálise peritoneal; provavelmente secundária à passagem trans-diafragmática de líquido através de uma comunicação pleuroperitoneal. A sua etiologia não está ainda definida, foram propostas diversas causas: defeitos congénitos do diafragma; gradiente de pressão pleuroperitoneal; distúrbios da drenagem linfática; defeitos adquiridos do diafragma, como o aparecimento de fenestrações ou a escassez de fibras musculares diafragmáticas, situações agravadas pela pressão intra-abdominal durante a permanência de dialisante na diálise peritoneal. A cintigrafia peritoneal (peritoneocintigrafia 99mTc-MAA) é uma ferramenta útil no diagnóstico de comunicação pleuroperitoneal. Apresentamos quatro casos de hidrotórax associados a comunicação pleuroperitoneal num total de 290 doentes tratados com diálise peritoneal entre 1999 e 2014, na nossa Unidade. Analisamos a incidência, estratégia diagnóstica e terapêutica desta complicação.

Peritoneal dialysis is an established treatment option for patients with end-stage renal disease. As this procedure causes high pressure in the abdomen, it might lead to peritoneopleural leakage (hydrothorax). The latter is an uncommon non-infectious complication of peritoneal dialysis patients, possibly secondary to trans-diaphragmatic fluid leakage through a pleuroperitoneal communication. The aetiology of this condition is still unclear. A variety of mechanisms have been proposed: congenital diaphragmatic defects; pleuroperitoneal pressure gradient; lymph drainage disorder; acquired diaphragmatic defects that are a consequence of increased intra-abdominal pressure during the dwell period of peritoneal dialysis, such as fenestrations or acquired scarcity of muscle fibres in the tendinous part of the diaphragm. Peritoneal scintigraphy (99mTc-MAA peritoneoscintigraphy) is a useful diagnostic tool in the management of suspected pleuroperitoneal communication. We report four cases of hydrothorax related to pleuroperitoneal communication in 290 patients treated with chronic peritoneal dialysis, between 1999 and 2014, at our PD Unit. Here we analyse our department’s incidence of this complication and also present own experience in diagnosing and treating it.

Q fever is a zoonotic disease caused by the highly infectious agent Coxiella burnetii which may manifest as a fairly common acute benign disease, or a much rarer and potentially lethal chronic condition. To our knowledge, chronic Q fever has previously only been described in two dialysis patients. We report a further two cases of haemodialysis patients with chronic Q fever and hypothesise as to its possible underdiagnosis in this population. Both patients had a prosthetic heart valve and although no vegetations were found by transoesophagic echocardiography, they met the Duke criteria for possible infective endocarditis. One patient, diagnosed about 11 months after the initial clinical manifestations, died, while the other, started on doxycycline and chloroquine about 3 months after the initial clinical manifestations, survived. We underline some key points that should lead to suspicion of chronic Q fever, namely the combination of a fluctuant febrile syndrome of unknown origin, anaemia and prior heart valve disease, especially in an immunocompromised patient