Familial Amyloidotic Polineuropathy and Systemic Lupus

Familial amyloidotic polineuropathy is a genetic disorder, leading to systemic amyloid deposits, manifested as sensory-motor and autonomic neuropathy. In the Portuguese classical form, the disease is evident at a young age, and causes death if no specific treatment is received. Variability in penetrance, age of onset and clinical course has been published; environmental and genetic factors are believed to contribute to this variability. The authors report a case of a 51-year-old white female, with a medical history of acquired angioedema, late-onset familial amyloidotic polineuropathy and systemic lupus erythemathosus. The authors consider that these associated diseases could modulate their expression

Alocação Renal: Novas Contribuições para um Desafio Permanente

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Alternativas na Colocação de Cateteres em Hemodiálise

Background: Although vascular access is essential for adequate haemodialysis delivery, the systematic use of a patient's venous patrimony may eventually lead to exhaustion of suitable sites for placement of a new vascular access. Case Report: We present two cases of such patients. In the first one we inserted a 55cm catheter through the left external iliac vein, and a 40cm translumbar catheter was placed in the second one. Both interventions were performed percutaneously under radiological guidance. Both patients were anticoagulated after the procedure. Conclusion: Unusual sites for haemodialysis catheter placement may be life saving in selected situations and offer safe and viable alternatives for adequate haemodialysis delivery

Anticoagulant-Related Nephropathy in a Patient with IgA Nephropathy

Anticoagulant-related nephropathy is a type of acute kidney injury caused by overcoagulation. We describe a case of an 84-year-old man with arterial hypertension, coronary heart disease and atrial fibrillation treated with acenocoumarol, who presented with haematoproteinuria and acute kidney injury during a phase of excessive anticoagulation. In addition to IgA nephropathy, renal biopsy also revealed acute tubular necrosis, red blood cell casts and positive iron staining in tubular cells. After this acute episode, renal function improved and proteinuria decreased below the nephrotic range.info:eu-repo/semantics/publishedVersio

Validation of Blood Gas Analysis in Arteriovenous Fistula for Hemodialysis

Introduction: Arterial blood gas analysis is a minimally invasive technique used in our daily practice but is not a complication free technique. The aim of this study was to validate results from blood gas analysis obtained from the arteriovenous fistula (AVF)/graft as a surrogate marker of the arterial blood gas analysis. Methods: A prospective observational study was made in 45 patients. We performed arterial and AVF/graft blood gas analysis and results were compared by a paired sample t Student test. Results: Most of our subjects was male (68.9%) and the mean age was 67 years (±14). Hemodialysis vintage was 63 months (±66), and vascular access age was 62 months (±56). The more prevalent vascular access was left radiocephalic AVF (n = 16; 35.6%) and the main puncture artery was right radial artery (n = 27; 60.0%). There were no statistically significant differences between the samples collected. Conclusions: Our results suggest a possible alternative of arterial blood gas analysis in AVF/graft for hemodialysis patients. This could result in making an uncomfortable procedure almost painless and reducing complications. Future research should take place to include anatomical characteristics of the AVF or the circulation of recirculation.info:eu-repo/semantics/publishedVersio

Zero-Time Kidney Histology Predicts Early Graft Function

Our purposes are to determine the impact of histological factors observed in zero-time biopsies on early post transplant kidney allograft function. We specifically want to compare the semi-quantitative Banff Classification of zero time biopsies with quantification of % cortical area fibrosis. Sixty three zero-time deceased donor allograft biopsies were retrospectively semiquantitatively scored using Banff classification. By adding the individual chronic parameters a Banff Chronic Sum (BCS) Score was generated. Percentage of cortical area Picro Sirius Red (%PSR) staining was assessed and calculated with a computer program. A negative linear regression between %PSR/ GFR at 3 year post-transplantation was established (Y=62.08 +-4.6412X; p=0.022). A significant negative correlation between arteriolar hyalinosis (rho=-0.375; p=0.005), chronic interstitial (rho=0.296; p=0.02) , chronic tubular ( rho=0.276; p=0.04) , chronic vascular (rho= -0.360;P=0.007), BCS (rho=-0.413; p=0.002) and GFR at 3 years were found. However, no correlation was found between % PSR, Ci, Ct or BCS. In multivariate linear regression the negative predictive factors of 3 years GFR were: BCS in histological model; donor kidney age, recipient age and black race in clinical model. The BCS seems a good and easy to perform tool, available to every pathologist, with significant predictive short-term value. The %PSR predicts short term kidney function in univariate study and involves extra-routine and expensive-time work. We think that %PSR must be regarded as a research instrument

Uma Avaliação Prospectiva do Transplante Renal Versus Hemodiálise: na Perspectiva Social, Qual a Melhor Modalidade Terapêutica?

Background: Economic evaluations help health authorities facing budget constraints. This study compares the health-related quality of life (HRQOL) and costs in patient subgroups on haemodialysis (HD) and renal transplantation (KT). Methods: In a prospective study with follow-up of 1-3 years, we performed a costutility analysis of KT vs. HD, adopting a lifetime horizon. A societal perspective was taken. Costs for organ procurement, KT eligibility, transplant surgery and follow-up of living donors were included. Key clinical events were recorded. HRQOL was assessed using the EuroQol instrument. Results: The HRQOL remained stable on HD patients. After KT, mean utility score improved at 3 months while mean EQ-VAS scores showed a sustained improvement. Mean annual cost for HD was 32,567.57€. Mean annual costs for KT in the year-1 and in subsequent years were, 60,210.09€ and 12,956.77€ respectively. Cost for initial hospitalization averaged 18,740.74€. HLA-mismatches increased costs by 75% for initial hospitalization (p < 0.001) and 41% in the year-1 (p < 0.05), and duplicate the risk of readmission in the year-1 (p < 0.05). The incremental costutility ratio was 5,534.46€/QALY, increasing 35% when costs for organ procurement were added. KT costs were 41,541.63€ more but provided additional 7.51 QALY. Conclusions: The KT is cost-effective compared with HD. Public funding should reflect the value created by the intervention and adapt to the organ demand

Calcifilaxia: Revisão da Literatura a Propósito de 2 Casos Clínicos

Calciphylaxis is a rare and devastating obliterative vasculopathy, leading to ischemia and subcutaneous necrosis. In most cases it affects patients with renal disease and is associated with high morbidity and mortality. We present two case reports followed recently in our department, and a literature review on this topic. Case one refers to an 80 -year -old Caucasian woman with chronic kidney disease stage 5 and primary hyperparathyroidism with secondary brown tumour and calciphylaxis. Case two refers to a 59 -year -old Caucasian woman admitted with severe nephrotic syndrome associated with amyloidosis, that developed a catastrophic picture of calciphylaxis, ending in the patient’s death. There is a critical need to understand the pathogenesis of calciphylaxis. Its comprehension is the only way to improve the survival of these patients, and may help to elucidate the pathophysiology of vascular calcification in general. Educating physicians in the prevention and early detection of calciphylaxis is crucial. Only by increasing the knowledge about risk factors, pathophysiology, response to treatment and outcome, will we be able to improve prophylaxis and therapy of patients with calciphylaxis, decreasing the high mortality of this entity