ABSTRACT Introduction: Delayed graft function (DGF), an outcome for which inflammation is critical, has been associated with worse outcomes after kidney transplantation (KT). The neutrophil to lymphocyte and platelet (NLP) ratio is a biomarker of systemic inflammation. We assessed postoperative NLP ratio applicability as an early predictor of DGF in KT patients. Methods: Retrospective cohort of adult patients submitted to KT at our unit, between 1 January 2010 and 31 December 2020. NLP was calculated at 24 hours post-KT. Primary outcome was development of DGF. Logistic regression was calculated to determine significant factos which may have contributed to DGF. Results: We included 527 patients with a mean age of 49.9 ± 12.8 years. In 47.8% of patients expanded criteria donors were used, and in 3.6% non-heart-beating donors. DGF occurred in 17.8% of patients. Mean post-KT NLP was higher in patients submitted to induction therapy with lymphocyte depleting antibodies (50.2±40.3 vs 11.9±7.4 with basiliximab, p<0.001), but it was found to be higher even before KT (5.2±1.8 vs 1.9±1.2, p=0.001). Grafts from non-heart-beating donors (OR 13.989, 95% CI 4.741, 41.274, p=0.000), longer warm ischemia time (OR 1.035, 95% CI 1.007, 1.064, p=0.014) and higher NLP ratio 24 hours after transplantation (OR 1.009, 95% CI 1.002, 1.016, p=0.015) were independently associated with DGF. Conclusion: Higher NLP ratio at 24 hours after KT was independently associated with DGF. This reflects the impact of inflammation on KT outcomes and highlights the role of the NLP ratio as a sensitive marker of systemic inflammatory response after KT.

ABSTRACT Introduction: Sodium-glucose cotransporter 2 inhibitors (SGLT2i) have shown positive renal outcomes in diabetic patients. There is also emerging evidence in non-diabetic patients. This review was conducted to analyse the renal outcomes of SGLT2i in patients without diabetes mellitus (DM). Methods: A systematic review was performed in a Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) compliant manner. We included only randomized trials that examined the effect of SGLT2i on renal outcomes in non-diabetic patients. Results: A total of ten randomized trials were included with a combined cohort of 26 298 patients. There was a tendency for lesser risk of adverse renal outcomes in heart failure (HF) patients with reduced ejection fraction (EF) (hazard ratio (HR) 0.50-0.71). Lesser effect on adverse renal outcomes was seen in HF patients with preserved EF (HR 0.95). For both preserved and reduced EF HF, there was a statistically significant reduction in the rate of decline in estimated glomerular filtration rate (eGFR) (p<0.001). On a short follow-up, there was a significant reversible reduction in GFR. In the long term, chronic kidney disease (CKD) proteinuric patients had a statistically significant lesser risk of adverse renal outcomes (p<0.001) and a significant reduction in albuminuria (p=0.0016). CKD patients, including non-proteinuric, had a significant reduction in the rate of kidney disease progression (HR = 0.71). Conclusion: Treatment with SGLT2i significantly reduces the rate of kidney disease progression in CKD non-diabetic patients with eGFR ≥ 20 mL/min/1.73 m2. Benefit seems greater in proteinuric patients. Benefit for eGFR < 20 mL/min/1.73 m2, dialysis and kidney transplant patients is yet to be defined.

ABSTRACT Introduction: Acute kidney injury (AKI) is a major complication in critically ill septic patients and is associated with increased morbidity and mortality. A recent study suggested a risk score based on patient’s chronic comorbidities and acute events at intensive care unit (ICU) admission as a reliable tool for predicting AKI in critically ill adult population. The aim of this study was to adapt this score to septic-AKI patients and evaluate its prognostic value as predictor of the need for renal replacement therapy (RRT) at ICU admission. Methods: This is a retrospective analysis of 399 septic-AKI patients admitted to the Division of Intensive Medicine of the Centro Hospitalar Universitário Lisboa Norte between January 2008 and December 2014. The Kidney Disease Improving Global Outcomes (KDIGO) classification was used to define AKI. The Renal Replacement Therapy Risk Score was adapted from the AKI risk prediction score proposed by Malhotra et al (Nephrol Dial Transplant. 2017;32:814-22). Results: Fifty two percent of patients were KDIGO stage 3, 25.8% KDIGO stage 2 and 22.3% KDIGO stage 1. Twenty seven percent of patients required RRT. Patients requiring RRT had higher risk score than those who did not (6.6±2.5 vs 5.1 ±2.6, p<0.001). An optimal cut-off value of ≥ 6 in this score predicted the need for RRT with sensitivity 0.630 and specificity 0.391. Conclusion: The RRT risk score at ICU admission was independently associated with the requirement of RRT septic-AKI patients. The assessment of this ratio is simple and can prove useful in identifying patients at risk for need of RRT.

Resumo Introdução: A lesão renal aguda (LRA) foi descrita em pacientes com doença do Coronavírus 2019 (COVID-19) e é considerada um marcador de gravidade da doença e fator prognóstico negativo para sobrevivência. Neste estudo, os autores visaram estudar o impacto da lesão renal aguda transitória e persistente (LRAp) na mortalidade hospitalar em pacientes com COVID-19. Métodos: Estudo observacional retrospectivo de pacientes internados com COVID-19 no Departamento de Medicina do Centro Hospitalar Universitário Lisboa Norte, Lisboa, Portugal, entre Março-Agosto de 2020. Realizou-se análise multivariada para prever desenvolvimento de LRA e mortalidade hospitalar. Resultados: De 544 pacientes com COVID-19, 330 desenvolveram LRA: 166 LRA persistente (LRAp), 164, LRA transitória. Pacientes com LRA eram mais velhos, apresentaram mais comorbidades prévias, maior necessidade de serem medicados com inibidores do SRAA, apresentaram creatina sérica basal mais elevada (CrS) (1,60 mg/dL vs 0,87 mg/dL), maior razão NL, e acidemia mais grave na admissão hospitalar, e necessitaram mais frequentemente de internação na UTI, ventilação mecânica, e uso de vasopressores. Pacientes com LRA persistente apresentaram maior nível de CrS (1,71 mg/dL vs 1,25 mg/dL) na admissão hospitalar. A mortalidade hospitalar foi de 14,0% e foi maior em pacientes com LRA (18,5% vs 7,0%). A DRC e ferritina sérica foram preditores independentes de LRA. A LRA não previu mortalidade, mas a LRAp foi um preditor independente de mortalidade, assim como idade e nível de lactato. Conclusão: A LRAp foi associada independentemente à mortalidade hospitalar em pacientes com COVID-19, mas seu impacto no acompanhamento de longo prazo ainda precisa ser determinado.

Abstract Introduction: Acute kidney injury (AKI) has been described in Coronavirus Disease 2019 (COVID-19) patients and is considered a marker of disease severity and a negative prognostic factor for survival. In this study, the authors aimed to study the impact of transient and persistent acute kidney injury (pAKI) on in-hospital mortality in COVID-19 patients. Methods: This was a retrospective observational study of patients hospitalized with COVID-19 in the Department of Medicine of the Centro Hospitalar Universitario Lisboa Norte, Lisbon, Portugal, between March 2020 and August 2020. A multivariate analysis was performed to predict AKI development and in-hospital mortality. Results: Of 544 patients with COVID-19, 330 developed AKI: 166 persistent AKI (pAKI), 164 with transient AKI. AKI patients were older, had more previous comorbidities, had higher need to be medicated with RAAS inhibitors, had higher baseline serum creatine (SCr) (1.60 mg/dL vs 0.87 mg/dL), higher NL ratio, and more severe acidemia on hospital admission, and more frequently required admission in intensive care unit, mechanical ventilation, and vasopressor use. Patients with persistent AKI had higher SCr level (1.71 mg/dL vs 1.25 mg/dL) on hospital admission. In-hospital mortality was 14.0% and it was higher in AKI patients (18.5% vs 7.0%). CKD and serum ferritin were independent predictors of AKI. AKI did not predict mortality, but pAKI was an independent predictor of mortality, as was age and lactate level. Conclusion: pAKI was independently associated with in-hospital mortality in COVID-19 patients but its impact on long-term follow-up remains to be determined.

Resumo Introdução: COVID-19 é atualmente um problema de saúde global e uma causa importante de mortalidade. Doença renal crônica (DRC) é um dos fatores de risco para infecção, morbilidade e mortalidade por SARS-CoV-2. Neste estudo, objetivamos avaliar a apresentação clínica e os outcomes de doentes com DRC com COVID-19, bem como identificar preditores de mortalidade. Métodos: Estudo retrospetivo de doentes com DRC internados num hospital terciário português entre Março-Agosto/2020. As variáveis foram submetidas a análise univariada e multivariada para determinar fatores preditivos de mortalidade hospitalar. Resultados: analisámos 130 pacientes com DRC (média de idades 73,9 anos; 60,0% homens). Hipertensão (81,5%), doença cardiovascular (36,2%) e diabetes (54,6%) foram comorbilidades frequentes. Tosse, dispneia, febre e insuficiência respiratória também foram comuns. Quase 60% apresentavam anemia, 50% hipoalbuminemia e 13,8% hiperlactacidemia, 17% acidemia. A ferritina sérica média foi 1531 µg/L, PCR média 8,3 mg/dL, LDH médio 336,9 U/L. A maioria foi tratada com lopinavir/ritonavir, hidroxicloroquina ou corticosteroides e apenas 2 com remdesivir. Oitenta por cento tiveram lesão renal aguda; 16,2% necessitaram de internamento na unidade de cuidados intensivos. Os 34 pacientes que faleceram eram mais velhos e mais propensos a ter insuficiência cardíaca. Estes apresentaram razão neutrófilos/linfócitos e níveis de ferritina, lactato e LDH mais elevados. A análise multivariada identificou uma associação entre idade avançada [OR 1,1 (IC 1,01-1,24), p=0,027], níveis de ferritina [OR 1,0 (IC 1,00-1,00), p=0,009] e LDH mais elevados [OR 1,0 (IC 1,00-1,01), p=0,014] e mortalidade. Conclusão: Na nossa coorte de doentes com DRC com COVID-19, a idade avançada e níveis mais elevados de ferritina e LDH foram fatores de risco independentes para mortalidade.

Abstract Introduction: COVID-19 is currently a global health issue and an important cause of mortality. Chronic kidney disease (CKD) is one of the risk factors for infection, morbidity and mortality by SARS-CoV-2. In our study, we aimed to evaluate the clinical presentation and outcomes of CKD patients with COVID-19, as well as identify predictors of mortality. Methods: This was a retrospective study of CKD patients admitted in a tertiary-care Portuguese hospital between March and August of 2020. Variables were submitted to univariate and multivariate analysis to determine factors predictive of in-hospital mortality. Results: 130 CKD patients were analyzed (median age 73.9 years, male 60.0%). Hypertension (81.5%), cardiovascular disease (36.2%), and diabetes (54.6%) were frequent conditions. Cough, dyspnea, fever and respiratory failure were also common. Almost 60% had anemia, 50% hypoalbuminemia, 13.8% hyperlactacidemia and 17% acidemia. Mean serum ferritin was 1531 µg/L, mean CRP 8.3 mg/dL and mean LDH 336.9 U/L. Most patients were treated with lopinavir/ritonavir, hydroxychloroquine or corticosteroids and only 2 with remdesivir. Eighty percent had acute kidney injury and 16.2% required intensive care unit admission. The 34 patients who died were older and more likely to have heart failure. They had higher neutrophils/lymphocytes ratio, ferritin, lactate, and LDH levels. Multivariate analysis identified an association between older age [OR 1.1 (CI 1.01-1.24), p=0.027], higher ferritin [OR 1.0 (CI 1.00-1.00), p=0.009] and higher LDH levels [OR 1.0 (CI 1.00-1.01), p=0.014] and mortality. Conclusion: In our cohort of CKD patients with COVID-19, older age, higher ferritin, and higher LDH levels were independent risk factors for mortality.

ABSTRACT Introduction: Dysproteinemia is associated with kidney damage and treatment of underlying conditions is crucial to minimize kidney damage. The aim of this study was to describe and analyze the characteristics and outcome of the patients diagnosed with dysproteinemia-associated kidney disease with initial renal presentation. Methods: We performed a retrospective observational study of the patients admitted with initial dysproteinemia-associated kidney disease in the Department of Nephrology and Renal Transplantation at Centro Hospitalar Universitário Lisboa Norte in Lisbon over five years. Results: Thirty patients were diagnosed with dysproteinemia-associated kidney disease: twenty-six (86.7%) with multiple myeloma, one of these with concomitant amyloidosis; three (10.0%) with gammopathy of renal significance and one (3.0%) with Waldenström macroglobulinemia. Mean age was 71.5 (± 12.44) years and half patients were male. Anemia, hypercalcemia, hyperphosphatemia, and hypoalbuminemia was found in twenty-three (76.7%), ten (30.0%), fourteen (46.7%) and seventeen (56.7%) patients, respectively. Bence Jones proteinuria was found in twenty-one (70.0%) patients and thirteen (43.3%) patients presented with creatinine >6 mg/dL. Twelve (40.0%) patients were submitted to kidney biopsy and light chain cast nephropathy was the most common finding. Twenty-nine (96.7%) patients were treated with chemotherapy. Twenty-two (72.3%) patients required renal replacement therapy and six of these (27.3%) patients were submitted to high cut-off dialyzers. Four (13.3%) patients had full recovery of renal function, sixteen (53.3%) remained dialysis-dependent at hospital discharge. At one-year follow-up, eleven (36.7%) patients remained dialysis-dependent and six (20.0%) had died. Conclusion: Dysproteinemia-associated kidney disease can present heterogeneously, and diagnostic suspicion is crucial to start prompt directed therapy to improve kidney and overall outcomes. Renal replacement therapy requirement and dependence at discharge appear to be related to higher mortality in these patients. In the future, routine screening of dysproteinemia may be particularly relevant in older patients to prevent irreversible kidney damage.

Resumo Introdução: O uso de Rituximab (RTX) em doenças glomerulares (DG) aumentou nos últimos anos, embora ainda utilizado apenas em uma pequena fração de pacientes. Métodos: Conduzimos em nosso centro, de 2010-2020, um estudo retrospectivo de único centro de pacientes adultos com nefropatia membranosa (NM), glomeruloesclerose segmentar focal (GESF), nefrite lúpica (NL) e vasculite tratada com RTX como terapia de primeira ou segunda linha. Resultados: Identificamos 19 pacientes; 36,8% tinham NM; 25,0% cada apresentava GESF, NL e vasculite. RTX foi terapia de primeira linha em 26,3% dos pacientes e em 73,7% foi terapia de segunda linha. O tempo médio de acompanhamento foi 7,7 ± 7,2 anos. Em NM, 2 pacientes (28,6%) tiveram remissão completa (RC), 2 pacientes (28,6%) remissão parcial (RP), e 3 pacientes (42,9%) não tiveram resposta (NR). Na GESF, 2 pacientes (50,0%) apresentaram RC, 1 paciente (25,0%) não teve resposta e, 1 paciente, deterioração renal. Dois pacientes (50,0%) apresentaram NL classe IV com RC após RTX, 1 paciente com NL classe IIIC/V não teve resposta, e 1 paciente com NL classe II apresentou deterioração renal. Na vasculite, 3 pacientes (75,0%) apresentaram RC e 1 paciente RP. Reações à infusão ocorreram em 2 pacientes (10,5%) e um paciente apresentou múltiplas complicações infecciosas. Conclusões: A eficácia do RTX em tratar diferentes tipos de DG imunomediada tem sido demonstrada com diferentes taxas de resposta, mas com perfil geral seguro. Em nossa série de casos, os resultados também são encorajadores. Estudos longitudinais são necessários para compreender melhor o efeito do RTX na DG.

Abstract Introduction: The use of Rituximab (RTX) in glomerular diseases (GD) has increased in the past years, although it is still only used in a small fraction of patients. Methods: A single center retrospective study of adult patients with membranous nephropathy (MN), focal segmental glomerulosclerosis (FSGS), lupus nephritis (LN), and vasculitis treated with RTX as first or second-line therapy was conducted at our center from 2010 to 2020. Results: We identified 19 patients; 36.8% had MN and 25.0% each had FSGS, LN, and vasculitis. RTX was first-line therapy in 26.3% of patients and in 73.7% it was second-line therapy. Mean follow-up time was 7.7 ± 7.2 years. In MN, 2 patients (28.6%) had complete remission (CR), 2 patients (28.6%) had partial remission (PR), and 3 patients (42.9%) had no response (NR). In FSGS, 2 patients (50.0%) presented CR, 1 patient (25.0%) had no response, and 1 patient had renal deterioration. Two patients (50.0%) had a LN class IV with a CR after RTX, 1 patient with LN class IIIC/V had no response, and 1 patient with LN class II had renal deterioration. In vasculitis, 3 patients (75.0%) presented CR and 1 patient had PR. Infusion reactions were present in 2 patients (10.5%) and one patient had multiple infectious complications. Conclusions: The efficacy of RTX in treating different types of immune-mediated GD has been demonstrated with different response rates, but an overall safe profile. In our case series, the results are also encouraging. Longitudinal studies are needed to better understand the effect of RTX in GD.

Resumo Introdução: Lesão renal aguda (LRA) é uma complicação comum em pacientes submetidos a grandes cirurgias abdominais, e está associada a considerável morbimortalidade. Vários estudos investigando a associação entre débito urinário intraoperatório e LRA pós-operatória mostraram resultados conflitantes. Neste trabalho investigamos a associação de oligúria intraoperatória com LRA pós-operatória em uma coorte de pacientes submetidos à grandes cirurgias abdominais. Métodos: Análise retrospectiva de centro único envolvendo pacientes adultos submetidos à grandes cirurgias abdominais, de janeiro de 2016 a dezembro de 2018. A LRA foi definida segundo critérios de creatinina da KDIGO. Definimos oligúria intraoperatória como débito urinário inferior a 0,5 mL/kg/h. Fatores de risco foram avaliados por análise de regressão logística multivariada. Resultados: Analisamos 165 pacientes. Nas primeiras 48 horas após a cirurgia, a incidência de LRA foi de 19,4%. LRA pós-operatória foi associada à mortalidade hospitalar (p = 0,011). 20% dos pacientes desenvolveram oligúria intraoperatória, sem associação com comorbidades preexistentes. Não houve correlação entre o tipo de anestesia e oligúria intraoperatória; entretanto, maior tempo de anestesia esteve associado à oligúria intraoperatória (p = 0,007). Maior creatinina sérica (Cr) inicial (p = 0,001), necessidade de drogas vasoativas (p = 0,007) e uso de AINEs (p = 0,022) foram associados à oligúria intraoperatória. Oligúria intraoperatória não foi associada ao desenvolvimento de LRA no pós-operatório (p = 0,772), à permanência hospitalar prolongada (p = 0,176) ou à mortalidade intra-hospitalar (p = 0,820). Conclusão: Demonstramos que a oligúria intraoperatória não prediz LRA pós-operatória em cirurgias abdominais de grande porte.

Abstract Background: Acute kidney injury (AKI) is a common complication in patients undergoing major abdominal surgery and is associated with considerable morbidity and mortality. Several studies investigating the association between intraoperative urine output and postoperative AKI have shown conflicting results. Here, we investigated the association of intraoperative oliguria with postoperative AKI in a cohort of patients submitted to elective major abdominal surgery. Methods: This was a single-center retrospective analysis of adult patients who underwent elective major abdominal surgery from January 2016 to December 2018. AKI was defined according to the serum creatinine criteria of the KDIGO classification. Intraoperative oliguria was defined as urine output of less than 0.5 mL/kg/h. Risk factors were evaluated using multivariate logistic regression analysis. Results: A total of 165 patients were analyzed. In the first 48 h after surgery the incidence of AKI was 19.4%. Postoperative AKI was associated with hospital mortality (p=0.011). Twenty percent of patients developed intraoperative oliguria. There was no association between preexisting comorbidities and development of intraoperative oliguria. There was no correlation between the type of anesthesia used and occurrence of intraoperative oliguria, but longer anesthesia time was associated with intraoperative oliguria (p=0.007). Higher baseline SCr (p=0.001), need of vasoactive drugs (p=0.007), and NSAIDs use (p=0.022) were associated with development of intraoperative oliguria. Intraoperative oliguria was not associated with development of postoperative AKI (p=0.772), prolonged hospital stays (p=0.176) or in-hospital mortality (p=0.820). Conclusion: In this cohort of patients we demonstrated that intraoperative oliguria does not predict postoperative AKI in major abdominal surgery.

Resumo O distúrbio mineral e ósseo é uma característica comum da doença renal crônica. A síndrome da face leonina é uma complicação rara do hiperparatireoidismo grave em pacientes com doença renal terminal, que tem sido menos relatada devido aos avanços na diálise e tratamento médico na última década. O reconhecimento precoce da deformidade facial característica é crucial para estimular o tratamento precoce e prevenir a desfiguração severa. Os autores apresentam um caso raro de hiperparatireoidismo grave, apresentando síndrome da face leonina e fraturas ósseas.

Abstract Mineral bone disorder is a common feature of chronic kidney disease. Lion face syndrome is rare complication of severe hyperparathyroidism in end-stage renal disease patients, which has been less commonly reported due to dialysis and medical treatment advances in the last decade. The early recognition of the characteristic facial deformity is crucial to prompt management and prevent severe disfigurement. The authors present a rare case of severe hyperparathyroidism presenting with lion face syndrome and bone fractures.

Vascular access (VA) dysfunction is an important cause of morbidity and mortality in the hemodialysis (HD) population. Regardless of the increasing prevalence of HD patients with complex VA, the definitions of VA failure (VAF) or end-stage VA failure (ES-VAF) are still being discussed and there is no current guideline that provides recommendation for solving the problem of complicated vascular access. The aim of this study was to review the management approach for resuming the continuity of RRT in patients with problematic vascular accesses, based on our center experience. In a retrospective analysis of 30 patients with VAF or ES-VAF over an 11-year period, twenty patients were transferred to peritoneal dialysis (PD), seven patients remained in HD after the placement of a non-standard site VA and three patients had a renal transplant (RT). In half of these patients, the initial therapeutic option eventually failed, leading to multiple reinterventions and RRT changes. Mean survival was 47 ± 32.7 months. Ten patients died, the majority within the first year of VAF diagnosis. The management of end-stage renal disease patients includes vessel preservation, the combination of RRTs to extend life expectancy and VA care to maintain long-term function. Our approach stratifies patients according to exhausted VA sites in order to plan for RT, transition to PD or alternative VA site management. This allows for better management of the VAF patient and highlights the need to individualize patient care.

Membranous nephropathy (MN) is the most common cause of nephrotic syndrome in Caucasian adults aged over 60 years old. Approximately two thirds of cases of membranous nephropathy (MN) in adults are primary (idiopathic), and the remaining are secondary forms which have been attributed to a variety of agents or conditions. Recent advances in pathophysiology and the availability of new laboratory diagnostic methods reinforce the importance of differentiating idiopathic from secondary MN, given the repercussions on therapy. We present the case of a 71-year-old Caucasian male referred to Nephrology due to nephrotic syndrome. He had a history of gastric adenocarcinoma and of parathyroid cancer, but was considered cured. Investigation excluded immunologic disease, dysproteinaemia, chronic infection and active malignancy. Kidney biopsy was performed and confirmed MN. Histological characterization of IgG subtypes was not available, nor was biopsy study for the reactivity for PLA2 receptor. The patient was started on conservative treatment, but, after a one-year follow-up, presented persistent nephrotic proteinuria (5g/day) and renal function decline (serum creatinine of 1.6mg/dL). Serological determination of phospholipase A2 receptor antibody was positive, but with borderline titer. Considering his history of malignancy as well as kidney function deterioration, Rituximab was prescribed. At one year of follow-up the patient has proteinuria of 500 mg/day, stable renal function (serum creatinine of 1.2 mg/dL) and negative serum anti-PLA2 receptor antibody. Despite the recent availability of serological determination of phospholipase A2 receptor antibody and histological characterization of IgG subtypes, in this case they did not allow us to safely distinguish between idiopathic and secondary MN. Therefore the past medical history played a significant part in the therapeutic option. Recent studies suggest that Rituximab has similar results to non-specific immunosuppression, promoting remission in patients with severe and persistent MN, with less adverse effects, and thus can be considered as first-line therapy

The increasing prevalence of end-stage chronic kidney disease associated with advances in treatment is expected to lead to improved survival rates of chronic haemodialysis patients in Portugal. Establishing and maintaining vascular access patency is becoming a challenging issue in these patients. We present a case report of a multiple vascular access failure patient to whom a novel vascular access device was implanted which allowed successful haemodialysis, despite the presence of central venous stenosis and/or occlusion. To our knowledge the present case report describes the first utilization of Hemodialysis Reliable Outflow (HeRO) Graft device in Portugal. We present the case as well as a brief description of the device and available literature. This case report also underlines the importance of adopting a multidisciplinary approach in order to overcome complex and life-threatening clinical challenges