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- Accidental Diagnosis of Isolated Persistent Left Superior Vena Cava After an Elective Central Venous Access Procedure for Chronic Hemodialysis: Clinical Implications and Precautions From a Case ReportPublication . Coimbra, Miguel T; Braga, Beatriz; Silva, Adriana; Sousa, Fernanda; Queirós, JoséPersistent left superior vena cava (PLSVC) is the most frequent thoracic venous anatomical variant in the general population. Isolated PLSVC, without formation of the right superior vena cava, is described in 10% of cases of PLSVC only. While it can be associated with congenital heart disease, arrhythmias, and premature death, adult patients with PLSVC are mostly asymptomatic, and the diagnosis is usually accidental. We present the case of a 72-year-old male with end-stage renal disease who was started on urgent hemodialysis through a temporary non-tunneled femoral central venous catheter (CVC) in the SLED (slow low-efficiency dialysis) modality and later remained dependent on hemodialysis. At this stage, placement of a tunneled CVC in the right internal jugular vein was necessary and fluoroscopy guidance was not available. There were no complications during the procedure, but postoperative conventional chest radiography revealed an inadequate positioning of the CVC tip in the left hemithorax, crossing the midline. Subsequently, the diagnosis of PLSVC was obtained by performing a thoracic angio-CT scan, confirming CVC tip positioning inside the PLSVC, and also excluded the presence of cardiac defects or additional anatomical variations of the great vessels of the thorax. Early evaluation for the creation of autologous vascular access was started under our care, and there were no mechanical or other complications associated with hemodialysis sessions during early follow-up after discharge.
- ANCA-positive vasculitis: Clinical implications of ANCA types and titersPublication . Domingues, V.; Machado, B.; Santos, J.INTRODUCTION: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is an autoimmune disease that can affect multiple organs, the kidney being one of the most affected. Apart from the diagnostics value of ANCA, they have also been advocated as biomarkers of the disease activity. Recently, the genetic changes found in polyangiitis associated with serine-protease proteinase 3 (PR3)-ANCA or myeloperoxidase (MPO)-ANCA raised the possibility of immune-pathogenic and therapeutic differences. OBJECTIVE: To identify differences in the number of relapses, inflammatory markers, outcomes and renal histology related to the types of ANCA. To analyze the implications of ANCA titers in prognosis. METHOD: A retrospective observational study in a Portuguese tertiary hospital. RESULTS: There were no differences in the progression of renal function, histological pattern and initial treatment with regard to ANCA subtypes. As for the evaluated parameters, there were no significant differences according to the types of ANCA, except for mean CRP values within the normal range, which was 6.3±1.3 mg/L for MPO-ANCA and 12.4±10.14 mg/L for PR3-ANCA (p=0.04). We found that 66.7% of the MPO-ANCA-positive showed no relapses versus 40% in the case of PR3-ANCA-positive. There was no correlation between the ANCA titers at presentation, during remission, and in the last evaluation, and the number of relapses. CONCLUSION: PR3-ANCA patients have a mean CRP value within the normal range significantly higher than that of MPO-ANCA patients (p=0.04), which seems to reveal greater inflammatory activity in the first.
- Association of the Calcification Score of the Abdominal Aorta, Common Iliac, and Renal Arteries with Outcomes in Living Kidney DonorsPublication . Ribeiro, Luís Costa; Almeida, Manuela; J, Malheiro; Silva, Filipa; Nunes-Carneiro, Diogo; Martins, La Salete; Pedroso, Sofia; Silva-Ramos, MiguelBackground: Vascular calcification is an ever-more-common finding in protocoled pre-transplant imaging in living kidney donors. We intended to explore whether a connection could be found between the Agatston calcification score, prior to kidney donation, and post-donation renal function. Methods: This is a retrospective analysis of 156 living kidney donors who underwent living donor nephrectomy between January 2010 and December 2016. We quantified the total calcification score (TCaScore) by calculating the Agatston score for each vessel, abdominal aorta, common iliac, and renal arteries. Donors were placed into two different groups based on their TCaScore: <100 TCaScore group and ≥100 TCaScore group. The relationship between TCaScore, 1-year eGFR, proteinuria, and risk of 1 measurement of decreased renal function (eGFR < 60 mL/min/1.73 m2) over 5 years of follow-up was investigated. Results: The ≥100 TCaScore group consisted of 29 (19%) donors, with a median (interquartile range) calcification score of 164 (117-358). This group was significantly older, 56.7 ± 6.9 vs. 45.5 ± 10.6 (p < 0.001), had a higher average BMI (p < 0.019), and had a lower preoperative eGFR (p < 0.014). The 1-year eGFR was similarly diminished, 69.9 ± 15.7 vs. 76.3 ± 15.5 (p < 0.048), while also having an increased risk of decreased renal function during the follow-up, 22% vs. 48% (p < 0.007). Conclusions: Our study, through univariate analyses, found a relationship between a TCaScore > 100, lower 1-year eGFR, and decreased renal function in 5 years. However, a higher-than-expected vascular calcification should not be an excluding factor in donors, although they may require closer monitoring during follow-up.
- Autoimmune diabetes recurrence should be routinely monitored after pancreas transplantationPublication . Martins, L.Autoimmune type 1 diabetes recurrence in pancreas grafts was first described 30 years ago, but it is not yet completely understood. In fact, the number of transplants affected and possibly lost due to this disease may be falsely low. There may be insufficient awareness to this entity by clinicians, leading to underdiagnosis. Some authors estimate that half of the immunological losses in pancreas transplantation are due to autoimmunity. Pancreas biopsy is the gold standard for the definitive diagnosis. However, as an invasive procedure, it is not the ideal approach to screen the disease. Pancreatic autoantibodies which may be detected early before graft dysfunction, when searched for, are probably the best initial tool to establish the diagnosis. The purpose of this review is to revisit the autoimmune aspects of type 1 diabetes and to analyse data about the identified autoantibodies, as serological markers of the disease. Therapeutic strategies used to control the disease, though with unsatisfactory results, are also addressed. In addition, the author's own experience with the prospective monitoring of pancreatic autoantibodies after transplantation and its correlation with graft outcome will be discussed.
- Biomarkers and Imaging Findings of Anderson-Fabry Disease-What We Know NowPublication . Beirão, I.; Cabrita, A.; Torres, M.; Silva, F.; Aguiar, P.; Laranjeira, F.; Gomes, A.Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder, caused by deficiency or absence of the alpha-galactosidase A activity, with a consequent glycosphingolipid accumulation. Biomarkers and imaging findings may be useful for diagnosis, identification of an organ involvement, therapy monitoring and prognosis. The aim of this article is to review the current available literature on biomarkers and imaging findings of AFD patients. An extensive bibliographic review from PubMed, Medline and Clinical Key databases was performed by a group of experts from nephrology, neurology, genetics, cardiology and internal medicine, aiming for consensus. Lyso-GB3 is a valuable biomarker to establish the diagnosis. Proteinuria and creatinine are the most valuable to detect renal damage. Troponin I and high-sensitivity assays for cardiac troponin T can identify patients with cardiac lesions, but new techniques of cardiac imaging are essential to detect incipient damage. Specific cerebrovascular imaging findings are present in AFD patients. Techniques as metabolomics and proteomics have been developed in order to find an AFD fingerprint. Lyso-GB3 is important for evaluating the pathogenic mutations and monitoring the response to treatment. Many biomarkers can detect renal, cardiac and cerebrovascular involvement, but none of these have proved to be important to monitoring the response to treatment. Imaging features are preferred in order to find cardiac and cerebrovascular compromise in AFD patients.
- Bone Mineral Density After Simultaneous Kidney–PancreasPublication . Pereira, S.; Pedroso, S.; Martins, L.; Santos, P.; Almeida, M.; Freitas, C.; Dias, L.; Dores, J.; Almeida, R.; Henriques, A.C.; Teixeira, M.ABSTRACT Bone disease and an high risk of fractures are major problems in transplantation. Among diabetic patients undergoing simultaneous kidney–pancreas (SKP) transplantation, there are few studies assessing long-term effects on bone mass. The aim of this study was to evaluate bone mineral density (BMD) over 4 years follow-up after SKP transplantation. Fifty-seven patients had 22.8 5.3 years of prior diabetes, 65% were female, and the overall mean age was 24.3 5.93 years. At the time of transplantation, the lumbar spine and femoral neck T-scores were 1.75 1.05 and 1.95 0.73, respectively; 28% of subjects had evidence of osteoporosis. One year after transplantation, 77.6% of patients displayed improved lumbar T-scores to 1.33 0.94 (.044) with stable femoral neck T-scores. Bone densitometry enhanced gradually through the 4 years follow-up: lumbar T-score to 1.04 0.67 (.004) and femoral neck T-score to 1.69 0.49 (.12). At year 4, no osteoporosis cases were detected but 86.7% of patients did not receive steroids in the immunosuppressive regimen. The graft function remained stable (serum creatinine, 1.2 mg/dL; fasting glucose, 87.7 mg/dL). During the follow-up, BMD improved more significantly at cortical sites. Our study reports a reduced prevalence of fractures (8.7%) compared with the literature, which could be related to a steroid-sparing protocol and/or aggressively treatment of osteoporosis.
- Chemical peritonitis in a patient treated with icodextrin and intraperitoneal vancomycinPublication . Freitas, C.; Rodrigues, A.; Carvalho, M.; Cabrita, A.
- Combined Pancreas-Kidney Transplantation: A New Program in Portugal, Results From the First 12 CasesPublication . Martins, L.; Henriques, A.; Dias, L.; Ventura, A.; Seca, R.; Almeida, R.; Dores, J.; Bacelar, C.; Oliveira, F.; Lhamas, A.; Amil, M.; Rua, F.; Coelho, T.; Esteves, S.; Ribeiro, A.; Pereira, R.; Sarmento, A.; Teixeira, M.; Pereira, M.Transplant Proc. 2003 May;35(3):1107-8. Combined pancreas-kidney transplantation: a new program in Portugal, results from the first 12 cases. Martins L, Henriques A, Dias L, Ventura A, Seca R, Almeida R, Dores J, Bacelar C, Oliveira F, Lhamas A, Amil M, Rua F, Coelho T, Esteves S, Ribeiro A, Pereira R, Sarmento A, Teixeira M, Pereira M. Transplantation Department, Hospital Santo António, 4050, Porto, Portugal. lasalete@clix.pt PMID: 12947877 [PubMed - indexed for MEDLINE]
- Compared to mycophenolate mofetil, rapamycin induces significant changes on growth factors and growth factor receptors in the early days post‐kidney transplantationPublication . OLIVEIRA, J.G.; XAVIER, P.; SAMPAIO, S.M.; HENRIQUES, C.; TAVARES, I.; MENDES, A.A.; PESTANA, M.We found 1 article: Transplantation. 2002 Mar 27;73(6):915-20. Compared to mycophenolate mofetil, rapamycin induces significant changes on growth factors and growth factor receptors in the early days post-kidney transplantation. Oliveira JG, Xavier P, Sampaio SM, Henriques C, Tavares I, Mendes AA, Pestana M. Renal Department, Hospital S. João, Porto, Portugal. Abstract BACKGROUND: The new immunosuppressive drug Rapamycin (Rapa) is endowed with a mechanism of action that is distinct from that of calcineurin inhibitors. It has been claimed that Rapa does not significantly modulate either the cytokine expression or the transcription of several growth factors in mitogen-activated T cells. Previously, we reported that fine-needle aspiration biopsy (FNAB) sample cultures synthesize a large array of cytokines, and some of them may be powerful predictors of acute rejection in renal transplants. We hypothesized that Rapa may induce significant changes on cytokine production by FNAB sample cultures and on serum cytokine receptors when compared to other immunosuppressive drugs. METHODS: Kidney transplants treated with CsA-Rapa-Pred (Rapa group) were compared with transplants treated with CsA-mycophenolate mofetil-Pred (MMF group). They were studied on day 7 posttransplantation, and they remained rejection free for at least the first 6 months. FNAB samples were cultured and the supernatants were collected at 48 hr of incubation and analyzed by ELISA for interleukin 1 receptor antagonist (IL-1ra), IL-2, IL-6, IL-10, IL-18, monocyte chemotactic protein 1 (MCP-1), soluble tumor necrosis factor I, and transforming growth factor (TGF)-beta(1). The soluble receptors for IL-1, IL-2, IL-6, and tumor necrosis factor alpha, together with IL-2 and IL-18 were also measured in serum. RESULTS: Significant differences were observed when comparing Rapa with the MMF group. IL-18 and TGF-beta(1) synthesis were up-regulated, whereas IL-6 and MCP-1 were down-regulated in FNAB sample cultures. The Rapa group showed a significant down-regulation of each cytokine receptor and of IL-2 in serum. CONCLUSIONS: Rapa was associated with a decreased synthesis of primarily monocyte-derived cytokines and enhanced production of TGF-beta(1), which in an appropriate cytokine milieu may promote allograft tolerance. The down-regulation of cytokine receptors and IL-2 may be associated with a depressed immune response towards the kidney allograft. PMID: 11923692 [PubMed - indexed for MEDLINE]
- CT volumetry performs better than nuclear renography in predicting estimated renal function one year after living donationPublication . Almeida, Manuela; Pereira, Pedro R.; Ramos, Miguel; Nunes-Carneiro, Diogo; Mandaleno, Mariana; Silva, Filipa; Pedroso, Sofia; França, Manuela; Martins, La Salete; J, MalheiroThe evaluation of split renal function (SRF) is a critical issue in living kidney donations and can be evaluated using nuclear renography (NR) or computerized tomography (CT), with unclear comparative advantages. We conducted this retrospective study in 193 donors to examine the correlation of SRF assessed by NR and CT volumetry and compared their ability to predict remaining donor renal function at 1 year, through multiple approaches. A weak correlation between imaging techniques for evaluating the percentage of the remaining kidney volume was found in the global cohort, with an R2 = 0.15. However, the Bland-Altman plot showed an acceptable agreement (95% of the difference between techniques falling within - 8.51 to 6.11%). The predicted and observed eGFR one year after donation were calculated using the CKD-EPI, and CG/BSA equations. CT volume showed a better correlation than NR for both formulas (adjusted R2 of 0.42. and 0.61 vs 0.37 and 0.61 for CKD-EPI and CG/ BSA equations, respectively). In non-nested modeling tests, CT volumetry was significantly superior to NR for both equations. CT volumetry performed better than NR in predicting the estimated renal function of living donors at 1-year, independently from the eGFR equation.