ABSTRACT Introduction: Optimal fluid balance in peritoneal dialysis requires adequate urinary and peritoneal water and sodium removal and restriction of dietary sodium intake (<2 g/day). We aim to study the relationship between sodium intake, blood pressure control and medication burden in peritoneal dialysis patients. Methods: Observational, cross-sectional study of chronic peritoneal dialysis patients followed in our hospital in March 2022. We estimated patient’s sodium intake according to the equations proposed by Kim et al. Mean ambulatory blood pressure was categorized in controlled, stage one, stage two and stage three hypertension. A qualitative evaluation of sodium intake was done by asking patients about their compliance with a low-salt diet. Results: Eighty-two patients: mostly men, with a mean age of 54.1±14.7 years and dialysis vintage of 26.2±18.7 months. Most patients were on continuous ambulatory peritoneal dialysis (63.4%) and the mean weekly Kt/V was 2.2±0.4. A percentage of 85.4% of our patients had residual diuresis, averaged at 1257±867 mL/day. Mean dietary sodium intake was 3.5±1.1 g; it was higher in men and patients with uncontrolled hypertension, regardless of age, dialysis vintage and other comorbidities. We found a strong correlation between dietary sodium intake and residual diuresis, systolic blood pressure, diastolic blood pressure and the number of anti-hypertensive drugs. Patients with a sodium intake ≥ 3.3 g had higher risk of uncontrolled BP and higher medication burden. Qualitative evaluation of this sub-population revealed that only 2.3% (n=1) admitted non-compliance with a low-sodium diet. Conclusion: We found a strong correlation between estimated dietary sodium consumption, blood pressure control and medication burden. Most importantly, there was a striking difference between patient’s perception of sodium consumption and the actual results. Our results highlight the importance of dietary salt restriction in blood pressure control and reinforce the need for a dietary consultation in peritoneal dialysis patients.

Abstract Introduction: Coronavirus disease 19 (COVID-19) has caused a direct increase in mortality but not all of it is entirely attributable to COVID-19, meaning there was a higher rate of mortality in non-COVID-19 patients due to the pandemic. The aim of this study was to compare two groups of patients: group 1, which included all of the patients admitted to the Internal Medicine ward during the month of April in the year 2019 and group 2, composed of all of the patients admitted to the same ward in April 2020 (a non-COVID ward) and to identify factors which were closely related with worse renal function in two group of patients. Methods: Retrospective observational study carried out through the analysis of digital data of all non-COVID-19 patients admitted at an Internal Medicine ward in April 2019 (group 1) and April 2020 (group 2). Results: A total of 162 patients were included. Group 1 represented 63.6% of the patients and group 2 represented 36.4%. Although there was a significant difference (p<0.001) between baseline creatinine and that presented at admission for hospitalization in both groups, the difference was markedly greater in the second group when compared to the first group. The worsening of renal function, measured by the CKD-EPI formula, was associated with higher mortality, which was higher in the 2020 group. Conclusion: There was a sense of fear imposed by the pandemic, which delayed resorting to medical care. This increased the severity of medical conditions. In the post-pandemic aftermath, it is essential that General and Family Medicine coordinate effectively with different medical specialties to jointly improve the follow-up of our patients.

Resumo Introdução: A doença de coronavírus 19 (COVID-19) causou um aumento direto na mortalidade, mas nem tudo é totalmente atribuível ao COVID-19, o que significa que houve uma taxa mais elevada de mortalidade em doentes não COVID-19 devido à pandemia. O objetivo deste estudo foi comparar dois grupos de doentes: grupo 1, incluiu todos os doentes internados na enfermaria de Medicina Interna durante o mês de abril do ano de 2019 e o grupo 2, composto pelos doentes internados na mesma enfermaria em abril de 2020 (enfermaria não COVID) e identificar o que se associou, de forma significativa, a alteração da função renal. Métodos: Estudo observacional retrospetivo realizado através da análise de dados digitais de todos os doentes não COVID-19 internados numa enfermaria de Medicina Interna em abril de 2019 (grupo 1) e abril de 2020 (grupo 2). Resultados: Foram incluídos 162 doentes; dos quais 63,6% eram do grupo 1 e 36,4% do grupo 2. Embora houvesse diferença significativa (p<0,001) entre a creatinina basal e a apresentada à admissão para internamento em ambos grupos, a diferença foi marcadamente maior no segundo grupo quando comparado ao primeiro grupo. O agravamento da função renal, medida pela fórmula CKD-EPI, associou-se a maior mortalidade que foi mais elevada no grupo de 2020. Conclusão: A pandemia COVID-19 impôs um sentimento de medo na população, que levou ao atraso no recurso aos serviços de saúde, levando a uma maior gravidade das condições médicas. No rescaldo pós-pandemia, é essencial uma coordenação eficaz entre a Medicina Geral e Familiar e as diferentes especialidades médicas para melhorar, conjuntamente, o acompanhamento dos nossos doentes, de forma a eliminar o medo imposto pela COVID-19.

ABSTRACT The diagnosis of antibody-mediated rejection has become more common and represents a major cause of kidney graft loss. The ideal treatment remains unknown, with little evidence supporting the use of a specific therapy. The definition of antibody-mediated rejection is based on serologic (presence and/or development of donor-specific antibodies) and histologic (evidence of acute tissue injury with C4d deposition or other signs of antibody interaction with the vascular endothelium) criteria. Antibody-mediated rejection without donor‐specific antibodies is a subcategory with a defying diagnosis. This particularly entity is increasingly being reported as an important cause of allograft dysfunction in kidney transplantation. We describe the case of a kidney transplant recipient with a low immunological risk and delayed graft function due to an antibody-mediated rejection with negative donor-specific antibodies.

ABSTRACT Introduction: Sleep disorders affect up to 70% of peritoneal dialysis (PD) patients and appear to have significant negative effects on their quality of life (QoL). We compared life and sleep quality between patients on automated PD (APD) and continuous ambulatory PD (CAPD). Material and Methods: Cross-sectional study in chronic PD patients followed in our Dialysis Unit in February 2022. We evaluated sleep quality with the Pittsburgh Sleep Quality Index (PSQI) and the Epworth Sleepiness Scale (ESS) and QoL with EUROHIS-QOL-8. Results: Fifty-two patients, mostly men (65.4%, n=32), with a mean age of 52.7±14.2 years and PD vintage of 24.3±17.1 months. Our sample had similar distribution between APD and CAPD; we found no difference between groups regarding demographic data, PD-related quality parameters or prevalence of previous sleep disturbances. Mean QoL index was 69.9%±9.3% and similar between APD and CAPD patients; EUROHIS-QOL-8 negatively correlated with the scores on ESS (p=0.004) and PSQI (p=0.011). Regarding sleep quality, the mean ESS was 8.8±4.2 and PSQI was 7.6±3.6. APD patients had similar scores on ESS, but higher prevalence of severe daytime sleepiness (ESS≥18) (p=0.04) and higher scores on PSQI (p=0.002). Within the PSQI, sleep’ latency (p=0.003), duration (p=0.002) and efficiency (p=0.013) were the most affected parameters. Higher PSQI scores also correlated with worse blood pressure control; a cut-off value for PSQI of 6 was found to increase the risk of uncontrolled hypertension. Conclusion: In our series, APD patients had worse global sleep quality (PSQI) when compared to ACPD patients. This difference was mainly due to worse sleep latency, duration and efficiency. The assessment of the quality of life showed no difference between groups, however there was a clear link between quality of life and sleep quality.

ABSTRACT A 43-year-old male patient followed for 27 years in a Nephrology consultation with negative autoimmune diagnostic workup and a histologically proven membranous nephropathy (MN), interpreted as a primary MN. During the follow-up he had multiple relapses, requiring continuous immunosuppression. Eventually, end-stage renal disease developed and the patient started hemodialysis (HD) in January 2018. Soon after initiating HD, he was admitted to the ward due to persistent fever of unknown origin, refractory to antipyretic and antibiotic therapy, but responsive to prednisolone (40 mg/day). Again, the diagnostic workup was negative and the patient was discharged with a corticosteroid taper over 16 weeks. In a posterior admission, after prednisolone suspension, the patient presented to the emergency room with persistente fever, involuntary weight loss, pruriginous macular rash and inflammatory joint pain. As the anti-nuclear antibody and anti-dsDNA came up positive and there was complement consumption, a diagnosis of a systemic lupus flare was established. His stay was complicated by sudden respiratory distress associated with hemoptysis and hemoglobin drop, with a confirmed diffuse alveolar hemorrhage. Daily plasma Exchange and intensive immunosuppression was necessary and the patient evolved into complete clinical remission with partial serological response. Usually, patients with systemic lupus erythematosus (SLE) develop clinical and serological remission upon initiating HD, reducing the number of flares and need for medication. This case depicts a lupus flare after starting HD, on a previously serologically negative patient, complicated with a rare type of lung parenchyma involvement, which is associated with a high mortality and required intensive immunosuppression for disease control. One can speculate whether or not the initial glomerulopathy was the first manifestation of SLE, previously controlled due to the continuous use of immunosuppression.

ABSTRACT Introduction: ANCA associated vasculitis commonly affects the kidneys. The intense inflammatory damage disrupts the glomerular architecture. Induction immunosuppressive therapy is responsible for a large part of the morbidity and mortality of these patients, due to infectious complications. This highlights the importance of defining risk factors associated with a worse renal prognosis in order to select the patients who would benefit the most from immunosuppressive therapy when it comes to renal limited vasculitis. Methods: Retrospective collection of data from patients admitted to the Coimbra’s University Hospital Nephrology Department with the diagnosis of de novo ANCA vasculitis, between 01-06-2009 and 01-06-2019. Data were analysed with SPSS v26® using parametric and nonparametric tests, Kaplan Meyer survival and ROC curve analysis. Results: There were 81 cases of de novo vasculitis, mainly due to anti-MPO antibody associated vasculitis (N=69; 85.2%). Mean age at diagnosis was 67.4 ± 15.7 years and most patients were male (N=47; 58%). At admission, patients had a mean serum creatinine (sCr) of 6.8 ± 4.0 mg/dL. Dialysis was required in 23 (28.4%) patients at admission and 44.4% were dependent on renal replacement therapy at discharge. Among dialysis-dependent patients at discharge treated with immunosuppression for at least 3 months (N=15) we observed partial kidney recovery in 2 (13.3%). Deaths were mostly (N=8/11; 72.7%) caused by infections. Discussion: Our results call into question the need for aggressive induction treatment in dialysis-dependent patients without extrarenal manifestations.

ABSTRACT We admitted a 21‑year‑old female to the Nephrology ward due to proteinuria and generalized oedema, which started soon after consuming clonixin 300mg thrice a day for two weeks. Her initial blood work showed nephrotic‑range proteinuria, hypoalbuminaemia, hypercholesterolaemia and normal kidney function. We conducted a full auto‑immune and serological study: there were no signs of infection, complemente consumption or underlying auto‑immune diseases. Suspecting minimal change disease, we started prednisolone 1mg/kg/day, with good clinical response and a reduction of urinary protein losses. By the third day, a kidney biopsy was performed: the glomeruli were morphologically normal and no immune deposits were found, which suggested minimal change disease. After seven days, the patient was discharged from the hospital with a corticoid taper until her Nephrology consultation. In the following weeks she maintained clinical and laboratorial remission and prednisolone was stopped after six months. Two months after, the patient noticed a growing bilateral cervical mass and went to the emergency room. The diagnostic workup was inconclusive, so an ultrasound‑guided biopsy was requested. The biopsy revealed lymphoid cell proliferation, positive for CD30, CD15, MUM‑1 and BCl6. These findings confirmed a Hodgkin’s Lymphoma diagnosis and the patient started radiotherapy and chemotherapy (ABVD‑doxorubicin, bleomycin, vinblastin, dacarbazine). In the following months her clinical status improved and the cervical mass had a significant size reduction. The kidney disease remained in remission, without therapeutic adjustments. A post‑treatment PET scan was requested, showing only a residual mediastinal mass.

ABSTRACT A 56-year-old male with a poorly controlled human immunodeficiency virus infection presents to the emergency room due to anorexia and weight loss. The patient was emaciated, hypertensive and his laboratory tests showed thrombocytopenia, anaemia, an elevated lactate dehydrogenase and an acute kidney injury. The patient was admitted to the Infecciology ward and by the fourth day, as the kidney function did not improve, a Nephrology consultation was requested. We collected a full auto-immune and serological panel and a urinalysis to rule out glomerular pathologies. Two days later, the patient developed a hypertensive emergency associated with an acute respiratory distress, responsive to furosemide and anti-hypertensive medications. The complementary study showed a high LDH, unmeasurable haptoglobin, thrombocytopenia and schizocytes on peripheral blood smear. These findings suggested a thrombotic microangiopathy associated with human immunodeficiency virus infection and so, he restarted antiretroviral therapy. The kidney function deteriorated and, despite starting haemodialysis, the patient died shortly after.

ABSTRACT Introduction: Diabetes is the leading cause of chronic kidney disease and end-stage kidney disease worldwide. A kidney biopsy in a diabetic patient must be considered when non-diabetic renal disease is suspected, such as in the presence of a rapid decline in renal function or severe unexplained proteinuria. However, the timing and criteria of a biopsy remain controversial in these patients. We aimed to identify clinical and histological markers that could help differentiate diabetic and non-diabetic renal disease and decide if this invasive approach is needed or not. Subjects and Methods: We reviewed 30 years of biopsies from diabetic patients performed at a tertiary hospital. We collected patient demographic data, biopsy indications, histological findings, and clinical and analytical data both at the moment of the biopsy and extensive followup. Based on kidney biopsy findings, patients were categorized as isolated diabetic nephropathy, non-diabetic kidney disease, or non-diabetic kidney disease superimposed on diabetic nephropathy (diabetic kidney disease). Results and Discussion: We enrolled 92 patients, mostly with type 2 diabetes, with a mean age of 62.9 ± 13.2 years. Nearly half of them had isolated diabetic nephropathy (53.3%), and 15.2% had diabetic nephropathy superimposed on non-diabetic kidney disease, comprising a total of 63 patients (68.5%) with diabetic kidney disease. Twenty-nine patients (31.5%) were considered to have non-diabetic kidney disease. These last patients were significantly less likely to need insulin therapy (p=0.002), had more frequently an acute deterioration of renal function (p=0.01), lower albumin levels (p=0.03), and a higher prevalence of microhematuria (p=0.001). We found the latter to be an independent predictor of non-diabetic kidney disease. Further, patients with the primary diagnosis of diabetic nephropathy had higher survival than those who had nondiabetic kidney disease, contradicting published data. Conclusions: The criteria for performing a biopsy in diabetic patients still lack consensus, although the priority to identify non-diabetic kidney disease prevails. We believe the non-diabetic kidney disease predictors we describe may prove helpful for determining the need for a histological assessment in diabetic patients.

ABSTRACT Eosinophilic granulomatosis with polyangiitis is an ANCA vasculitis characterized by asthma, rhinosinusitis and peripheral eosinophilia. The kidney is infrequently involved, usually in the form of necrotizing crescentic glomerulonephritis. We present the case of a 60-year-old man who presented with painless sudden loss of visual acuity, purpuric exanthem in his legs, asthenia and myalgia. CT-scan ruled out acute vascular and intracranial space occupant lesions. Optical coherence tomography showed signs of left central retinal artery occlusion and perfusion deficits in the right arterial retinal blood supply. Complementary study showed prominent peripheral eosinophilia (24.500 cel/uL), increased serum IgE (1260U/L) and increased C-reactive protein (10.6mg/dl). During admission, the patient presented with acute kidney failure (serum creatinine of 4.7mg/dl) and an exceptionally high p-ANCA MPO titer (>600U/L). Eosinophilic granulomatosis with polyangiitis was diagnosed and plasmapheresis, pulse steroid therapy and intravenous cyclophosphamide were provided. Kidney biopsy showed interstitial nephritis with high eosinophil content while the glomerulus was relatively spared, with only mild endocapillary proliferation. The patient didn’t require dialysis. Kidney function was normal at discharge, although the visual deficit did not improve.

Resumo A doença de Hirschsprung é o distúrbio congénito de motilidade intestinal mais comum e habitualmente diagnosticado no período neonatal. É causado por um segmento intestinal aganglionar, que leva a ausência de peristaltismo intestinal e obstrução funcional. O diagnóstico deve ser considerado em todos os doentes com obstipação sem resposta ao tratamento convencional. A radiografia e o enema com contraste são exames importantes para o diagnóstico, mas este só é definitivamente estabelecido por análise histoquímica da biópsia do segmento aganglionar. O tratamento é cirúrgico e o reconhecimento precoce da doença é importante para evitar complicações e melhorar o prognóstico. É reportado o caso clínico de um lactente que se apresentou no Serviço de Urgência Pediátrica com náuseas, vómitos, obstipação, recusa alimentar e uma importante distensão abdominal. A radiografia abdominal e o enema com contraste foram compatíveis com doença de Hirschsprung. A análise histoquímica da biópsia confirmou o diagnóstico e foi efetuado tratamento cirúrgico.

Abstract Hirschsprung disease is the most common congenital gut motility disorder and usually diagnosed in the neonatal period. It is caused by an aganglionic bowel segment resulting in absence of intestinal peristalsis and functional obstruction. Diagnosis should be considered in all patients with constipation that does not respond to conventional treatment. Radiography and contrast enema are important diagnostic exams, but the definitive diagnosis is established through histochemical analysis of aganglionic segment biopsy. Treatment is surgical and early recognition is important to avoid complications and improve prognosis. Herein is reported the clinical case of a young infant presenting to the Pediatric Emergency Department with nausea, vomiting, constipation, refusal to feed, and important abdominal distension. Abdominal radiograph and contrast enema were compatible with Hirschsprung disease. Biopsy histochemical analysis confirmed the diagnosis and surgical treatment was performed.

Concomitant development of symptomatic amyloid light-chain (AL) amyloidosis and multiple myeloma (MM) is rare and has a poor survival rate. Introduction of thalidomide and bortezomib, a proteasome inhibitor, has improved the survival of patients with MM; however, it has not cured the disease. Nevertheless, monoclonal antibodies may treat the disease using an immunotherapeutic approach. This report describes the case of a 39-year-old man with MM IgG lambda, heavily treated with multiple QT cycles and two hematopoietic stem cell transplants, who developed light chain amyloidosis. After an interdisciplinary discussion, daratumumab was initiated. Approximately 24 months later, he presents complete remission of the disease, normal renal function and no associated symptoms.

Introduction: Central venous stenosis can be the main obstacle to the creation of a vascular access. The Surfacer® device was developed to solve the problem of complete occlusions of the right internal jugular vein, right brachiocephalic vein and superior vena cava, allowing for the placement of a vascular access in this venous axis. Case Report: We present the case of a 77-year-old patient on long-term hemodialysis with a long history of failed vascular accesses and of central vein stenosis, chronically dialyzed using a tunneled catheter placed through the left femoral vein. With use of the Surfacer® device it was possible to recreate, with an inside-out technique, a tract through the fibrous tissue of the completely occluded right brachiocephalic and right internal jugular veins, subsequently placing a tunneled catheter into the right atrium through this later vein. Conclusion: We report the first successful use in Portugal of a new vascular access device that allows us to rescue a previous unusable right central venous axis for the placement of a central venous catheter, thus avoiding the use of less advantageous options such as the femoral veins and preserving venous vascular capital.