ABSTRACT Objectives: The prevalence of end-stage renal disease is rising among older adults worldwide. Despite kidney transplantation being considered the best renal replacement therapy, it presents unique challenges in elderly patients. This study aims to describe deceased donor kidney transplantation in our center, analyze outcomes namely delayed graft function (DGF), acute rejection, bacterial infections, and death-censored allograft loss in patients aged 65 years or older, and compare graft and patient survival with recipients younger than 65 years old. Methods: A single-center retrospective cohort study of kidney transplantation from a deceased donor between 2016 and 2020 was conducted. Data on donor, recipient, and transplant characteristics were collected, and outcomes after transplantation were analyzed. Univariate Cox regression was used to compare patient and death-censored allograft survival between older and younger patients. Results: Of the 294 deceased-donor transplants performed, 48 were allocated to recipients aged 65 years or older. These patients had a significantly higher prevalence of extended criteria donors (ECD) when compared to younger recipients (p < 0.001). The mean recipient age in the elderly group was 68 ± 2 years, with a median follow-up of 29 months (interquartile range [IQR] 18-49). During the 1st year, five (10.4%) patients were diagnosed with biopsy-proven acute rejection and 24 (50%) with bacterial infections. DGF was observed in 27 (56.3%) patients and was associated with a higher proportion of high-risk donors (ECD and uncontrolled circulatory death donors with normothermic regional perfusion) (p = 0.034), longer cold ischemic times (p = 0.031), and hospitalization duration (p < 0.001). Death-censored allograft survival at 1, 3, and 5 years was 89.1, 89.1, and 84.6%, respectively, which was not statistically different from the group of younger recipients (p = 0.56). Throughout follow-up, five patients died, three (60%) of whom had a functioning allograft. Patient survival at 1, 3, and 5 years was 100, 97.6, and 79.2%, respectively, again showing no notable differences compared to younger recipients (p = 0.12). Conclusion: Even though an individualized approach and careful pre-transplant evaluation are key for the success of kidney transplantation in the elderly population, our 1, 3, and 5-year death-censored allograft and patient survival in older patients were similar to younger recipients. Objectives endstage end stage worldwide therapy center DGF, , (DGF) infections deathcensored censored 6 old Methods singlecenter single 201 202 conducted collected analyzed Results deceaseddonor performed 4 ECD p 0.001. 0001 0.001 . 0 001 0.001) followup follow up interquartile IQR [IQR 1849. 1849 18 49 18-49) st year 10.4% 104 10 (10.4% biopsyproven biopsy proven 50% 50 (50% 56.3% 563 56 3 (56.3% highrisk high risk perfusion 0.034, 0034 0.034 034 0.034) 0.031, 0031 0.031 031 0.031) Deathcensored Death 1 891 89 89.1 846 84 84.6% respectively 0.56. 056 0.56 0.56) followup, up, died 60% 60 (60% 100 976 97 97.6 792 79 79.2% 0.12. 012 0.12 12 0.12) Conclusion pretransplant pre population 5year (DGF 20 000 0.00 00 184 18-49 10.4 (10.4 (50 56.3 (56.3 003 0.03 03 8 89. 84.6 05 0.5 (60 9 97. 7 79.2 01 0.1 0.0 18-4 10. (10. (5 56. (56. 84. 0. (6 79. 18- (10 ( (56 (1

RESUMO Objetivos: A prevalência de doença renal em estágio terminal está aumentando entre os adultos mais velhos em todo o mundo. Apesar de o transplante renal ser considerado a melhor terapia de substituição renal, ele apresenta desafios únicos em pacientes idosos. Este estudo pretende descrever o transplante renal com doador falecido em nosso centro, analisar os resultados, nomeadamente, função tardia do enxerto (FTE), rejeição aguda, infecções bacterianas e perda do aloenxerto censurada para a morte em pacientes com 65 anos ou mais, e comparar a sobrevida do enxerto e do paciente com receptores com menos de 65 anos. Métodos: Foi realizado um estudo de coorte retrospectivo em um único centro sobre transplante renal de doador falecido entre 2016 e 2020. Foram coletados dados sobre as características do doador, do receptor e do transplante, e os resultados após o transplante foram analisados. A regressão univariada de Cox foi usada para comparar a sobrevida do paciente e do aloenxerto censurada para a morte entre pacientes mais velhos e mais jovens. Resultados: Dos 294 transplantes de doadores falecidos realizados, 48 foram alocados para receptores com 65 anos ou mais. Esses pacientes tiveram uma prevalência significativamente maior de doadores com critérios estendidos (DCE) em comparação com receptores mais jovens (p < 0,001). A idade média dos receptores no grupo de idosos foi de 68 ± 2 anos, com um acompanhamento mediano de 29 meses (intervalo interquartil [IQR] 18-49). Durante o primeiro ano, cinco (10,4%) pacientes foram diagnosticados com rejeição aguda comprovada por biópsia e 24 (50%) com infecções bacterianas. A FTE foi observada em 27 (56,3%) pacientes e foi associada a uma proporção maior de doadores de alto risco (doadores com DCE e morte circulatória não controlada com perfusão regional normotérmica) (p = 0,034), tempos isquêmicos frios mais longos (p = 0,031) e maior duração de hospitalização (p < 0,001). A sobrevida do aloenxerto censurado para a morte em 1, 3 e 5 anos foi de 89,1, 89,1 e 84,6%, respectivamente, o que não foi estatisticamente diferente do grupo de receptores mais jovens (p = 0,56). Durante o acompanhamento, cinco pacientes morreram, três (60%) dos quais tinham um aloenxerto funcional. A sobrevida dos pacientes em 1, 3 e 5 anos foi de 100, 97,6 e 79,2%, respectivamente, mais uma vez sem diferenças notáveis em comparação com os receptores mais jovens (p = 0,12). Conclusão: Embora uma abordagem individualizada e uma cuidadosa avaliação pré-transplante sejam fundamentais para o sucesso do transplante renal na população idosa, nossa sobrevida do aloenxerto e do paciente censurado para a morte em 1, 3 e 5 anos em pacientes idosos foi semelhante à dos receptores mais jovens. Objetivos mundo nomeadamente FTE, , (FTE) 6 Métodos 201 2020 analisados Resultados realizados 4 (DCE p 0,001. 0001 0,001 . 0 001 0,001) intervalo IQR [IQR 1849. 1849 18 49 18-49) ano 10,4% 104 10 (10,4% 50% 50 (50% 56,3% 563 56 (56,3% normotérmica 0,034, 0034 0,034 034 0,034) 0,031 0031 031 1 891 89 89, 846 84 84,6% respectivamente 0,56. 056 0,56 0,56) morreram 60% 60 (60% funcional 100 976 97 97, 792 79 79,2% 0,12. 012 0,12 12 0,12) Conclusão prétransplante pré idosa (FTE 20 202 000 0,00 00 184 18-49 10,4 (10,4 (50 56,3 (56,3 003 0,03 03 8 84,6 05 0,5 (60 9 7 79,2 01 0,1 0,0 18-4 10, (10, (5 56, (56, 84, 0, (6 79, 18- (10 ( (56 (1

ABSTRACT Parvovirus B19 (PVB19)-associated pure red cell aplasia (PRCA) is an important diagnosis to consider in kidney transplant (KT) recipients experiencing persistent anemia, in whom other etiologies have been excluded. Its management poses a challenge since reducing immunosuppression (IS) to address the viral infection needs to be carefully balanced with the increased risk of allograft rejection. The authors describe a case of a 43-year-old male with a history of chronic kidney disease of unknown etiology who underwent a KT from a deceased donor after circulatory death in January 2021. In the 1st year post-KT, the patient was repeatedly admitted due to the recurrence of PVB19-associated PRCA, requiring blood transfusions and intravenous immunoglobulin (IVIG). IS was initially adjusted with mycophenolate mofetil (MMF) suspension and later transitioned to a TRANSFORM scheme with prednisolone, tacrolimus, and everolimus. Due to deteriorating kidney function, a kidney biopsy was performed and revealed borderline acute T-cell mediated rejection with significant signs of chronicity (50% of interstitial fibrosis and tubular atrophy). To guarantee infection control, IS was not increased. Considering the recurrence of PVB19-associated PRCA, despite the use of a TRANSFORM IS scheme in a patient with chronic allograft dysfunction and high immunologic risk, maintenance therapy with IVIG (0,4 mg/kg) every 4 weeks was started. After 9 months of maintenance therapy, no relapse was identified. To promote an individualized IS prescription, an ImmunoBiogram® was recently performed and an IS reduction is planned, followed by an ImmunoBiogram® control, which will potentially allow the suspension of IVIG maintenance therapy. The diagnosis and management of PVB19-associated PRCA is challenging. Regarding recurrent disease, prolonged IVIG treatment appears to be a useful treatment strategy, but more studies are necessary to ascertain its role. It is also fundamental to tailor the IS as much as possible to the individualized immunologic profile of the patients to prevent overimmunosuppression. B B1 PVB19associated PVBassociated PVB19 associated PVB (PRCA (KT anemia excluded (IS 43yearold yearold 43 old 2021 st postKT, postKT post KT, post-KT IVIG. . (IVIG) MMF (MMF prednisolone tacrolimus everolimus function Tcell T 50% 50 (50 atrophy. atrophy atrophy) control 0,4 04 0 (0, mg/kg mgkg mg kg started identified prescription ImmunoBiogram planned challenging strategy role overimmunosuppression PVB1 202 (IVIG 5 (5 0, (0 20 ( 2

RESUMO A aplasia eritrocitária pura (PRCA) associada à infeção por parvovírus B19 (PVB19) é um importante diagnóstico a considerar em recetores de transplante renal (TR) que se apresentam com anemia persistente, nos quais outras etiologias foram excluídas. A gestão desses doentes representa um desafio, uma vez que a redução da imunossupressão (IS) para tratar a infeção vírica deve ser cautelosa pelo potencial aumento do risco de rejeição do aloenxerto. Os autores descrevem um caso de um homem de 43 anos com antecedentes de doença renal crónica de etiologia indeterminada que foi submetido a TR de um dador falecido após morte circulatória em janeiro de 2021. No primeiro ano pós-TR, o paciente foi internado repetidamente devido a PRCA associada ao PVB19 e necessitou de transfusões de glóbulos vermelhos e tratamento com imunoglobulina humana intravenosa (IVIG). A IS foi inicialmente ajustada com suspensão do micofenolato de mofetil (MMF) e posteriormente alterada para um esquema TRANSFORM com prednisolona, tacrolimus e everolimus. Devido ao agravamento da função renal, foi realizada uma biópsia renal que revelou rejeição aguda mediada por células T borderline com sinais significativos de cronicidade (50% de fibrose intersticial e atrofia tubular). Para garantir o controlo da infeção, a IS não foi aumentada. Considerando a recorrência da PRCA associada ao PVB19 apesar do uso de um esquema IS TRANSFORM num paciente com disfunção crónica do aloenxerto e alto risco imunológico, foi iniciada terapêutica de manutenção com IVIG (0,4 mg/kg) a cada 4 semanas. Após 9 meses de tratamento não foi identificada nenhuma recidiva. De forma a promover uma prescrição individualizada de IS, um ImmunoBiogram® foi recentemente realizado, estando a ser considerada uma redução da IS que potencialmente permitirá a suspensão da terapia de manutenção com IVIG. O diagnóstico e tratamento da PRCA associada ao PVB19 são desafiadores. Relativamente à doença recidivante, o tratamento prolongado com IVIG parece ser útil, mas mais estudos são necessários para estabelecer o seu papel. Também é fundamental adaptar, tanto quanto possível, a IS ao perfil imunológico individual dos doentes para prevenir o uso excessivo de imunossupressão. (PRCA B B1 PVB (PVB19 (TR persistente excluídas desafio (IS 2021 pósTR, pósTR pós TR, pós-TR PVB1 . (IVIG) MMF (MMF prednisolona everolimus 50% 50 (50 tubular. tubular tubular) aumentada 0,4 04 0 (0, mg/kg mgkg mg kg semanas recidiva ImmunoBiogram realizado desafiadores recidivante útil papel adaptar possível (PVB1 202 (IVIG 5 (5 0, (0 (PVB 20 ( 2

ABSTRACT Introduction: Kidney transplantation is the preferred treatment for end-stage renal disease, but organ scarcity can result in long waiting times. Living-donor kidney transplantation offers an alternative to deceased donation, but HLA or AB0 incompatibility can pose a significant obstacle. This study aimed to show the results achieved by a portuguese hospital since its integration into an international cross-over program, the South Alliance for Transplants (SAT). Methods: The SAT program was founded in 2017 and is composed of ten Spanish hospitals, three Italian hospitals and one Portuguese hospital. The program runs every 4 months and enrolled only pairs that were incompatible. Organ transportation is carried out in partnership with the Portuguese Air Force. Results: Three distinct cross-over kidney transplants were performed in partnership with three Spanish hospitals, culminating in the transplantation of three Portuguese patients out of a total of seven patients. The first swap was performed in March 2020, at the beginning of the COVID-19 pandemic, in partnership with two Portuguese and one Spanish hospital. It involved one donor/recipient pair from each country, with AB0 incompatibility between the Portuguese donor and recipient, and no complications were reported. The second swap occurred in December 2021, with three donor/recipient pairs (one Portuguese, where the recipient presented anti-donor antibodies and positive crossmatch with the potential donor, and two from two Spanish hospitals). It was complicated by type-IB cellular rejection in the Portuguese recipient, one week after transplantation, which was treated with corticosteroid therapy. The third swap, also in December 2021, involved two donor/recipient pairs (one Portuguese and one Spanish). It was complicated by delayed renal function due to acute tubular necrosis (histological diagnosis) in the Portuguese recipient. At follow-up, the patients‘ serum creatinine levels were within normal limits, and no other unexpected outcomes were recorded. Conclusion: SAT program has allowed some successful cross-over kidney transplants, probably improving the outcomes of kidney transplantation in Portugal. The expansion of such programs may contribute to a more efficient use of the available resources, increasing the number of transplants performed and reducing waiting times. Introduction endstage end stage disease times Livingdonor Living donation AB obstacle crossover cross over SAT. . (SAT) Methods 201 incompatible Force Results 2020 COVID19 COVID 19 COVID-1 pandemic donorrecipient country reported 2021 antidonor anti hospitals. hospitals) typeIB type IB therapy Spanish. Spanish) histological diagnosis followup, followup follow up, up follow-up limits recorded Conclusion Portugal resources (SAT 20 202 COVID1 1 COVID- 2

RESUMO Introdução: O transplante renal é o tratamento preferencial da doença renal crônica terminal, porém, a escassez de órgãos pode resultar em longos tempos de espera. O transplante renal de doador vivo oferece uma alternativa ao doador cadáver, mas a incompatibilidade HLA ou AB0 pode representar um obstáculo significativo. Este estudo teve como objetivo mostrar os resultados alcançados por um hospital português desde sua integração num programa internacional de doação cruzada, o South Alliance for Transplants (SAT). Métodos: O programa SAT foi fundado em 2017 e é composto por dez hospitais espanhóis, três hospitais italianos e um hospital português. O programa ocorre a cada 4 meses e inscreve apenas pares que são incompatíveis. O transporte de órgãos é realizado em parceria com a Força Aérea Portuguesa. Resultados: Foram realizados três cruzamentos distintos em parceria com três hospitais espanhóis, culminando no transplante de três doentes portugueses de um total de sete doentes. O primeiro cruzamento foi realizado em março de 2020, no início da pandemia de COVID-19, com a parceria de dois hospitais portugueses e um hospital espanhol, envolvendo 1 par doador/recetor de cada país, em que o português apresentava incompatibilidade AB0 e onde não se registraram complicações. O segundo ocorreu em dezembro de 2021 com 3 pares doador/receptor (1 português em que o recetor apresentava anticorpos anti-dador e crossmatch positivo com o potencial doador; e 2 de dois hospitais espanhóis), complicado com rejeição celular tipo-IB uma semana após o transplante no receptor português, tratada com corticoterapia. O terceiro cruzamento ocorreu também em dezembro de 2021 com 2 pares doador/recetor (1 português e 1 espanhol), complicado com atraso na função renal por necrose tubular aguda (diagnóstico histológico) na receptora portuguesa. Durante o follow-up, os níveis de creatinina sérica dos doentes mantiveram-se dentro dos limites normais, e não foram registradas outras intercorrências. Conclusão: O programa SAT permitiu a realização de alguns transplantes renais cruzados bem-sucedidos, provavelmente melhorando os resultados do transplante renal em Portugal. A expansão de programas semelhantes pode contribuir para um uso mais eficiente dos recursos disponíveis, aumentando o número de transplantes realizados e reduzindo os tempos de espera. Introdução terminal porém espera cadáver AB significativo cruzada SAT. . (SAT) Métodos 201 espanhóis incompatíveis Portuguesa Resultados 2020 COVID19, COVID19 COVID 19, 19 COVID-19 espanhol doadorrecetor país complicações 202 doadorreceptor ( antidador anti dador , espanhóis) tipoIB tipo IB corticoterapia espanhol) diagnóstico histológico portuguesa followup, followup follow up, up follow-up mantiveramse mantiveram normais intercorrências Conclusão bemsucedidos, bemsucedidos bem sucedidos, sucedidos bem-sucedidos Portugal disponíveis (SAT 20 COVID1 COVID-1 COVID-

ABSTRACT For patients with end stage renal disease, kidney transplant offers significant survival and quality-of-life advantages compared with dialysis. But for patients seeking transplant who are highly sensitized, waiting times have traditionally been long and options limited. We present the case of a 34-year-old hypersensitized female who underwent renal retransplantation. Histocompatibility tests revealed a calculated panel-reactive antibody of 99.53% with multiple antibodies against class I and II human leucocyte antigens and an eplet analysis was performed. The donor’s potential unacceptable antigens were re-defined and the calculated panel-reactive antibody decreased to 88.38%. After one month the patient received a deceased-donor kidney transplant. Complement dependent cytotoxicity crossmatch was negative; virtual crossmatch and flow cytometry crossmatch with historical serum were positive. High-dose intravenous immunoglobulin and rituximab were added to the thymoglobulin-based induction immunosuppression. Three donor-specific antibodies were detected and plasmapheresis was performed. Renal allograft biopsy revealed no manifestations of rejection. Repeated testing observed a decrease in donor-specific antibodies median fluorescence intensity values. Four months post-transplant, the patient remained with normal graft function without proteinuria. She is receiving a standard maintenance immunosuppression regime with prednisolone, mycophenolate mofetil and tacrolimus. The careful discussion among the transplantation center and histocompatibility laboratory in association with intense immunosuppression and close laboratory monitoring allowed a successful human leukocyte antigen-incompatible deceased donor kidney transplantation in the most critical phase for the occurrence of humoral rejection. It is noteworthy that the new histocompatibility and immunogenetics methodologies provide a more affirmative and comprehensive assessment of mismatch acceptability.

ABSTRACT Operational tolerance in kidney transplantation is characterized by stable serum creatinine < 1.7 mg/dL and proteinuria < 1 g/day in the absence of immunosuppression or immunodeficiency for over one year. However, simultaneous donor specific antibodies are common and serum creatinine is a poor surrogate of early lesions. Consequently, subclinical rejections will meet operational tolerance criteria if sérum creatinine remains stable. We report a patient with operational tolerance criteria followed by biopsy-proven chronic active antibody mediated rejection, discussing the intricate challenges of immunosuppression management.

ABSTRACT Nephrotic syndrome in systemic lupus erythematosus patients with histological evidence of minimal change disease, mesangial proliferation or focal and segmental glomerulosclerosis on light microscopy, represents a distinct clinical entity - lupus podocytopathy. This entity is characterized by a diffuse foot process effacement on electron microscopy and by absence of subepithelial or subendothelial immune‑complex deposition. We report the case of a 49‑year‑old woman admitted on suspicion of lupus nephritis flare, characterized by nephrotic syndrome and acute kidney injury, whose renal biopsy revealed histological features of lupus podocytopathy. Six months after discharge, under prednisolone and azathioprine, she presented 300 mg/day proteinuria, normal kidney function, without hematuria. A review of the pathogenesis, clinical features, diagnostic criteria, treatment and prognosis of lupus podocytopathy is provided. This case highlights the mounting evidence that lupus podocytopathy encompasses distinct clinical and morphological features, that should be included in the upcoming pathological classification of lupus nephritis.

RESUMO Objetivo: Investigar se a hiperemia reativa correlaciona-se com marcadores de disfunção endotelial e pode ser utilizada para identificar sepse na doença crítica. Métodos: Trata-se de estudo prospectivo em uma coorte de pacientes críticos. A disfunção endotelial foi avaliada quando da admissão, por meio da quantificação de hiperemia por tonometria arterial periférica e níveis plasmáticos de endotelina 1, E-selectina solúvel, endocana e sindecano 1. Os pacientes sépticos foram comparados com pacientes sem evidência de infecção. Resultados: Cinquenta e oito pacientes sépticos foram comparados com 28 controle. O logaritmo natural da tonometria arterial periférica teve correlação negativa com comorbidades cardiovasculares, severidade da doença e níveis plasmáticos de E-selectina solúvel (p = 0,024) e sindecano 1 (p < 0,001). O logaritmo natural da tonometria arterial periférica foi mais baixo nos pacientes sépticos quando comparado com os de pacientes controle (0,53 ± 0,48 versus 0,69 ± 0,42, respectivamente) e, quando ajustado à idade, o modelo multivariado predisse que cada 0,1 de diminuição em unidades de logaritmo natural da tonometria arterial periférica levou a aumento de 14,6% na probabilidade de infecção. Conclusão: A hiperemia reativa avaliada por tonometria arterial periférica tem estreita relação com E-selectina solúvel e sindecano 1, o que sugere associação entre ativação endotelial, degradação de glicocálix e reatividade vascular. A hiperemia reativa por tonometria arterial periférica parece estar comprometida em pacientes críticos, especialmente os com sepse.

Abstract Objective: To investigate whether reactive hyperemia measured by peripheral arterial tonometry correlates with markers of endothelial dysfunction and may be used to identify sepsis in critical illness. Methods: A prospective study was performed using a cohort of critically ill patients. Endothelial dysfunction was assessed on admission by quantifying reactive hyperemia-peripheral arterial tonometry and plasma levels of endothelin-1, soluble E-selectin, endocan and syndecan-1. Septic patients were compared to patients without evidence of infection. Results: Fifty-eight septic patients were compared to 28 controls. The natural logarithm of reactive hyperemia-peripheral arterial tonometry was negatively correlated with cardiovascular comorbidities, disease severity and plasma levels of soluble E-selectin (p = 0.024) and syndecan-1 (p < 0.001). The natural logarithm of reactive hyperemia-peripheral arterial tonometry was lower in septic patients than in controls (0.53 ± 0.48 versus 0.69 ± 0.42, respectively). When adjusted for age, the multivariable model predicted that each 0.1-unit decrease in natural logarithm of reactive hyperemia-peripheral arterial tonometry increased the odds for infection by 14.6%. m. Conclusion: Reactive hyperemia-peripheral arterial tonometry is closely related to soluble E-selectin and syndecan-1, suggesting an association between endothelial activation, glycocalyx degradation and vascular reactivity. Reactive hyperemia-peripheral arterial tonometry appears to be compromised in critically ill patients, especially those with sepsis.

IgA Nephropathy (IgAN) is the most prevalent biopsy-proven primary glomerular disease worldwide. Historically, thrombotic microangiopathy (TMA) was associated with IgAN in cases of severe hypertension or advanced chronic kidney disease, but recent data suggest that complement activation plays a crucial role in the development of TMA in IgAN. We report a case of Crescentic IgAN with complement-mediated TMA (C-TMA) in a 27-year old male patient with a pathological missense mutation in heterozygosity in the CFH gene and a rare variant in the C3 gene, treated with steroids, cyclophosphamide and plasmapheresis without recovery of kidney function. We also discuss other treatment possibilities and kidney transplant options. Additionally, we will review the latest advances that are enhancing our understanding of the association between IgAN and TMA

Hyperoxaluria is an important complication of short bowel syndrome (SBS) and a non-negligible cause of chronic kidney disease (CKD). We describe the case of a 56-year-old woman with polyarteritis nodosa (PAN) and SBS who was admitted for acute kidney injury (AKI) 2 months after a successful deceased donor kidney transplantation. The diagnostic investigation was consistent with the diagnosis of oxalate nephropathy (OxN) and a set of lifestyle and therapeutic measures was implemented aiming at the reduction of intestinal oxalates absorption. Despite the short-term benefit of this strategy, a progressive and inexorable deterioration of renal function was observed. In this report, we discuss the limited therapeutic options, the ominous prognosis and the risk of recurrence of this rare entity after renal transplantation. Additionally, we reflect on the value of patient compliance in the successful management of the disease and review potential new strategies that may improve the clinical course of this entity in the future

Lupus nephritis is a common complication of systemic lupus erythematosus (SLE), usually developing early in the course of the disease. Almost all patients with lupus nephritis present with positive ANA and anti-dsDNA, Hypocomplementaemia and renal manifestations (haematuria, proteinuria and/or renal failure). However, in a small number of patients, the initial presentation is lupus nephritis with absent serology or clinical features of systemic lupus erythematosus

Introdução: O transplante renal em adultos com alterações congénitas do trato urinário inferior (ACTUI) apresenta complicações específicas. O estudo urológico de bexigas com baixa capacidade e pouco complacentes pode determinar a necessidade de cirurgia vesical reconstrutiva e soluções derivativas. Além disso, esta população apresenta maior risco de infeções do trato urinário (ITUs). Objetivo: Caraterizar os doentes com ACTUI seguidos na Unidade de Transplante Renal do Centro Hospitalar de São João e avaliar complicações pós -transplante renal. Os doentes com refluxo vesicoureteral (RVU) primário foram excluídos da análise. Materiais e Métodos: Foram avaliados 8 doentes com ACTUI com idades entre 13 e 39 anos. A doença renal crónica estabeleceu -se por RVU secundário e ITUs de repetição em contexto de mielomeningocelo (3 casos), válvulas da uretra posterior (1 caso), bexiga neurogénica com ânus imperfurado (1 caso), megaureter congénito (2 casos) e bexiga neurogénica idiopática com dissenergia vesicoesfincteriana (1 caso). Resultados: Todos os doentes foram submetidos a intervenções cirúrgicas antes do transplante: nefrectomia dos rins nativos (2 casos), ablação das válvulas da uretra posterior (1 caso), cistoplastia de aumento (6 casos) e ureteroileostomia (1 caso). Sete doentes mantiveram-se continentes, 6 praticando auto-algaliação intermitente para esvaziamento vesical e 1 usando a manobra de Valsalva. Verificou-se uma taxa de pielonefrites agudas do enxerto renal com necessidade de internamento de 31 episódios por 100 doentes -ano, bastante superior à descrita para transplantados renais por qualquer causa, apesar da sobrevida do enxerto não ter sido afetada. Após um seguimento médio de 104 ± 11.8 meses, 5 doentes mantêm enxertos funcionantes com creatinina sérica média de 1.17 ± 0.40 mg/dl. Conclusão: O transplante renal em doentes com ACTUI apresenta bons resultados após cirurgia prévia para correção das alterações urológicas, apesar da frequência elevada de pielonefrites do enxerto

Introduction: Renal transplantation in adults with congenital abnormalities of the lower urinary tract (CALUT) carries particular complications. Careful urologic evaluation of abnormal bladders, due to neuropathy or outlet obstruction, may indicate the need for surgical correction before transplantation. This population is also at increased risk for urinary tract infections. Purpose: We assessed the outcome of renal transplantation in patients with CALUT at our Transplant Unit. Patients with primary vesicoureteral reflux (VUR) were excluded from our analysis. Materials and Methods: A total of eight patients with CALUT, 13 to 39 years old, were evaluated. End -stage renal disease was caused by secondary VUR and recurrent urinary tract infections in patients with myelomeningocele (three cases), posterior urethral valves (one case), neurogenic bladder associated with imperforate anus (one case), congenital megaureter (two cases) and idiopathic neuropathic bladder and sphincter dyssynergia (one case). Results: All patients underwent surgical interventions before transplantation: ureteronephrectomy of the native kidneys (two cases), ablation of posterior urethral valves (one case), augmentation cystoplasty (six cases) and construction of an ileal conduit (one case). Seven patients remained continent, six were able to empty the bladder by clean intermittent selfcatheterization and the remaining by using the Valsalva maneuver. A rate of 31 episodes per 100 patient-years of graft pyelonephritis was found, much higher when compared to other transplant recipients, although graft survival was not affected. After a mean follow -up of 104 ± 11.8 months, five patients have kept functioning grafts with mean serum creatinine of 1.17 ± 0.40 mg/dl. Conclusion: Renal transplant in patients with CALUT has good outcomes after preparatory urologic surgical corrections although graft pyelonephritis remains a frequent complication

As amiloidoses são um grupo heterogéneo de doenças classificadas de acordo com a composição das suas proteínas amiloidogénicas. Frequentemente, os tecidos preservados em parafina são usados para identificação imunohistoquímica. A análise de ADN deve ser sempre considerada se houver suspeita de amiloidose hereditária. Dado que os rins são um dos órgãos mais frequentemente envolvidos nas amiloidoses sistémicas, procedemos à classificação imunohistoquímica de 102 casos consecutivos de doença amiloide confirmada por biópsia renal. A análise de ADN foi realizada para confirmar o diagnóstico de amiloidose hereditária. As características demográficas, doença subjacente e dados clínicos à data da biópsia foram obtidos pela revisão retrospetiva dos registos médicos. O tipo de amiloidose obtido por identificação imunohistoquímica foi AA em 60 (58,8%) doentes, AL em 21 (20,6%), AFib em quatro (3,9%), ATTR em dois (2,0%), AApoAI em um (2,0%), ALys em um (2,0%), e em um (2,0%) coexistiam os tipos AL e AA. Em 12 (11,7%) não foi identificado o tipo de amiloide: oito (7,8%) por imunohistoquímica negativa e quatro (3,9%) devido a amostra insuficiente. A análise de ADN confirmou os casos AFib e ATTR pela identificação das mutações pontuais FGA p.Glu545Val e TTR p.Met51Val, respetivamente. A média de idade à data do diagnóstico foi 53,3 anos (49,4 para AA, 63,0 para AL e 53,9 para AFib). As infeções crónicas foram a principal causa de amiloidose AA, sobretudo a tuberculose, e foi apenas identificada uma AA familiar associada a síndrome de Muckle-Wells. A síndrome nefrótica foi a manifestação clínica mais frequente, independentemente do tipo de amiloide. Na nossa série, a amiloidose AA continua a ser a amiloidose sistémica mais frequente. Seis doentes tiveram amiloidose hereditária inequívoca. A imunohistoquímica não identificou a proteína precursora em quase 8% dos doentes; contudo, a utilização de um painel de anticorpos mais alargado poderá melhorar o diagnóstico.

Amyloidoses are a group of heterogeneous diseases classified according to the nature of their causative amyloid proteins. Commonly, paraffin-embedded tissue is used for the typing of amyloid by immunohistochemistry. DNA analysis should always be considered if hereditary amyloidosis is suspected. Since the kidneys are one of the organs that are most commonly involved in amyloid deposition in systemic amyloidoses, we screened 102 consecutive cases with biopsy-proven amyloid disease by immunohistochemistry. DNA analysis was performed to confirm a diagnosis of hereditary amyloidosis. Demographic characteristics, underlying disease and clinical data at the time of renal biopsy were obtained by retrospective review of medical records. The amyloidosis type according to immunohistochemical amyloid protein identification was AA in 60 (58.8%) patients, AL in 21 (20.6%), AFib in four (3.9%), ATTR in two (2.0%), AApoAI in one (1.0%), ALys in one (1.0%) and combined AL and AA in one (1.0%). The type of protein could not be classified in 12 (11.7%) patients: eight (7.8%) because of negative immunohistochemistry and four (3.9%) due to the lack of adequate tissue. DNA analysis confirmed AFib and ATTR cases by the identification of the point mutations FGA p.Glu545Val and TTR p.Met51Val, respectively. Mean age at diagnosis was 53.3 years (49.4 for AA, 63.0 for AL and 53.9 for AFib). Chronic infections were the most frequent disorder associated with AA amyloidosis, mainly tuberculosis, and only one patient had familial AA associated with Muckle-Wells syndrome. Nephrotic syndrome was the most frequent clinical manifestation, independently of the amyloid type. In our series, AA amyloidosis is still the most frequent type of systemic amyloidoses. Six patients had unequivocal hereditary amyloidosis. Immunohistochemistry did not establish the precursor protein in almost 8% of patients; however, an improvement could be obtained using a wider panel of amyloid antibodies.

A sarcoidose e uma doença inflamatória cronica, de atingimento multissistemico, cuja etiologia permanece ainda por identificar. Caracteriza-se, do ponto de vista histopatológico, pela formação de granulomas não caseosos em qualquer órgão envolvido. Os mecanismos mais frequentemente implicados na disfunção renal devem-se aos distúrbios no metabolismo do cálcio; mais raramente podem ocorrer nefrite intersticial granulomatosa e doença glomerular. Os autores apresentam três casos de nefrite intersticial com o achado de insuficiência renal como manifestação inaugural de sarcoidose. Foi efectuado tratamento com prednisolona na dose de 1 mg/Kg/dia, verificando-se recuperação parcial e sustentada da função renal.

Sarcoidosis is a chronic inflammatory disorder of unknown cause. The histopathological feature is the presence of the non-caseating granuloma in any organ involved. The mechanisms most frequently implicated in renal dysfunction are due to disturbances in calcium metabolism; more rarely, granulomatous interstitial nephritis and glomerular disease may occur. The authors report three cases of sarcoidosis presented as interstitial nephritis with renal failure. Prednisolone treatment was performed at a dose of 1 mg/kg/day with partial and sustained recovery of renal function.