Portuguese Journal of Nephrology & Hypertension

Hyperinfection syndrome with hypereosinophilia and chronic kidney disease: case report and review

Chronic kidney disease (CKD) is associated to significant infection incidence and severity, the cause of death of, approximately, 20% of end-stage renal disease patients. Close to 80% of all infections in these patients can be related to organ or tissue other than vascular access, reflecting immune system dysfunction related to several mechanisms. Strongyloides stercoralis is an endemic nematode found especially in tropical and subtropical areas. The clinical manifestations of stercoralis infection vary according to the acuity of infection and the host response, from asymptomatic to the disseminated presentation, frequently lethal. Risk factors for severe presentations are related to immunosuppression states. The authors report a case of a patient with advanced CKD of unknown etiology, who developed an exuberant eczema, respiratory symptoms and severe hypereosinophilia. The etiological study revealed a disseminated form of Strongyloides stercoralis infection with myocardia involvement, diagnosed solely by serologic testing, despite microbiological tests to identify the infective agent. In this case, the patient has evolved favorably, with remission of symptoms and cardiac features, after adequate anti-helminthic treatment. The importance of parasitic colonization and its potential harm becomes clinically more relevant when patients become immunosuppressed, as in CKD progression or in the immunosuppressive therapy setting, such as solid organ transplant rejection therapy. A brief literature review related to strongyloidiasis and immunosuppression in the CKD setting is also presented

IgG4-tubulointerstitial nephritis: a literature review following 5 cases in a single centre

Immunoglobulin G4-related disease is a recently described systemic fibro-inflammatory disease, characterized by an infiltration of abundant IgG4+ plasma cells and lymphocytes, leading to tumour-like swellings of the involved organs, and variable degrees of fibrosis. Organ involvement can occur metachronously, and as IgG4 expressionin other tissues is less specific, renal biopsy often plays a critical role in diagnosis leading to prompt treatment, which can prevent renal damage and other organ failure. Tubulointerstitial nephritis is the most common renal feature. IgG4-renal involvement is challenging to diagnose and remains under-recognized, particularly in patients with single-organ involvement. The authors describe the only 5 cases of IgG4-RD with kidney involvement identified in the last 10 years in a tertiary referral nephrology centre that serves a population of 3 million people. These cases underline the importance of an early diagnosis of IgG4-related disease, since early treatment is usually effective. They also corroborate that clinical and histopathological features are heterogeneous and that complement may play a role in its pathogenesis

Factors associated with early fistula failure

Introduction: Around the world, risk factors for fistula failure have been considered in vascular access planning in order to improve results. However, primary fistula failure rates seem to be increasing. Considering this, we conducted a study to identify relevant factors for early fistula failure in a Portuguese cohort with end-stage kidney disease. Subjects and Methods: Retrospective case-control study which included patients from a hospital center who underwent fistula construction between 2012 and 2015. Patients with fistula failure at 6 weeks were matched with consecutive controls in a proportion of 1:1. Clinical and laboratory data were retrieved. Multiple regression analysis was performed to identify factors associated with early complications. Results: Total of 100 predialysis patients with fistula failure at 6 weeks. Mean age of 67.7±11.9 years; most were women (n=54). Factors associated with overall risk of complications were distal location of fistula (OR 2.8; p<0.05) and diabetes mellitus (OR 3.8; p<0.05). Congestive heart failure (OR 7.2; p=0.06) was associated with a tendency for greater risk of inflow complications. Conclusions: In this cohort, despite improvements in vascular access planning, traditional risk factors still have a significant impact on fistula outcomes. The role of new factors is still undefined and further studies are needed. An adequate patient education, an organized vascular access program with nurses, nephrologists and surgeons with expertise, with systematic use of Doppler ultrasound, are key factors for better outcomes

Predicting early mortality in incident hemodialysis patients: strengthening a shared decision-making process

Introduction: The benefits of dialysis in the elderly are dubious. A shared decision-making process, helped by adequate prognostic tools, is essential to determine which patients are better candidates for conservative care. Based on a recent USRDS validated score, this study aimed to identify risk factors associated with early mortality (first 90 days) in a Portuguese cohort of patients. Methods: A total of 197 patients who initiated hemodialysis treatments in a Portuguese facility between 2005 and 2015 were included. Clinical and laboratory data were collected at time of admission to center. Multiple regression models were performed and fitted to identify potential predictors of early mortality. Findings: Total of 93 (47.2%) deaths with 23 (11.7%) deaths occurring in first year. In the first three months, there were 15 (7.6%) deaths. Of those who died in first three months, most were men (n=10; 5.1%), mean age 73.5 ± 6.82 years. Almost half (n=7; 3.6%) were dependent and the majority (n=12; 6.1%) had history of hospitalizations in previous year before admission. They had a higher prevalence of hypoalbuminemia and cardiovascular risk factors. Mortality associated factors were albumin level low (<3.5 g/dL) or unknown (OR 5.73; p<0.05), ischemic cardiomyopathy (OR 4; p<0.05) and history of hospitalizations in previous year before admission (OR 4.3; p<0.05). Absence of history hypertension was associated with a reduction of risk (OR 0, 18, p<0.05). Discussion: Some elements of USRDS score were associated with greater risk for early mortality in this Portuguese cohort of patients. Further investigations are needed in order to validate a specific prognostic tool in Europeans

Are we building too many arteriovenous fistulas?: A single-center experience

Introduction: Arteriovenous fistula has been associated with improved morbimortality in hemodialysis patients. This has resulted in the fistula First, catheter last initiative. Nonetheless, the survival benefit of arteriovenous fistula has been questioned. Methods: We conducted a retrospective observational study of all patients with non-end stage renal disease referred for first vascular access building between January 2014 and December 2015 in our hospital center. Our main goal was to evaluate the clinical impact and burden of building fistula in predialysis patients. Results: During this period, of 178 first arteriovenous accesses placed, 87 patients remained in predialysis and 91 patients started a chronic hemodialysis program. Median follow-up time by a nephrologist was 3.9 (2.5, 9.7) years. The mean age was 65.8±14.7 years, with 50.6% (n=90) of male patients. A higher rate of thrombosis in the predialysis group (26% vs 13%, p=0.037) was observed, but vascular access survival did not differ significantly (55% vs 67%, p=0.12). Mean vascular access placing was higher in the predialysis group (1.4±0.7 vs 1.2±0.4, p=0.006) and less interventions were requested (0.2±0.5 vs 0.3±0.6, p=0.10). Median time from vascular access placement to hemodialysis start was 22 (13, 41) months. At hemodialysis initiation, 10 (10.9%) patients used a central venous catheter; 80 (87.9%) patients an arteriovenous fistula, and one patient a graft. A total of 227 vascular accesses were built; 121 (53.3%) in predialysis vs 106 (46.7%) in incident hemodialysis patients. In a multivariate model, the presence of a functional arteriovenous fistula at hemodialysis start was only associated with a trend to survival benefit (HR 0.38, 95% CI 0.14-1.00, p=0.05). Conclusions: Our results stress the need for an individual approach and for future tools to assess the risk of death and progression to end-stage renal disease, therefore helping reduce the number of unutilized vascular accesses and rising cost of interventions

Lecithin-cholesterol acyltransferase deficiency: a review for clinical nephrologists

Lecithin-cholesterol acyltransferase (LCAT) is the enzyme responsible for esterification of free cholesterol on the surface of lipoproteins, particularly in high-density lipoproteins (HDL), and is also involved in the reverse transport of cholesterol from peripheral tissues to the liver. LCAT is synthesized in the liver and circulates in plasma reversibly bound to lipoprotein particles, or in a lipid-free form. Primary LCAT deficiency is a rare inherited metabolic condition caused by homozygous or compound heterozygous mutation in the LCAT gene. It is associated with two distinct clinical syndromes, Familial LCAT Deficiency (FLD) and Fish-Eye Disease (FED), respectively caused by complete and partial deficiency of LCAT activity, but having in common markedly reduced plasma levels of HDL and apolipoprotein A-I. FLD is characterized by corneal opacities, haemolytic anaemia and chronic kidney disease (CKD), which may progress to end-stage renal disease (ESRD). The pathogenesis of FLD nephropathy is not clear, but accumulation of lipoprotein-X in the kidneys might be a major contributing factor. Corneal opacification is the only clinical hallmark of FED. In line with several reports of intermediate phenotypes, we have recently described an incomplete FLD phenotype in two Portuguese brothers, homozygous for a novel LCAT mutation, presenting with proteinuric CKD but no haemolytic anaemia, who developed noticeable corneal clouding only many years afterwards. Such a phenotype poses a diagnostic challenge to nephrologists, which will have to rely on accurate appraisal of the lipid profile abnormalities and a high index of suspicion to consider the right diagnosis. Further studies are needed to confirm whether recombinant human LCAT is effective in halting CKD progression in FLD patients. Meanwhile, renoprotective therapy by inhibition of renin-angiotensin-aldosterone system should be initiated as soon as possible. Despite early histological recurrence of the nephropathy in kidney grafts, renal transplantation remains a suitable therapy for FLD patients with ESRD

The role of bone biomarkers and new imaging techniques in the management of patients with CKD-MBD

Chronic kidney Disease - Mineral and Bone Disorder (CKD-MBD) encompasses abnormalities in bone turnover, volume and mineralization, compromising bone quantity and quality. Bone biopsy is the gold standard to access bone changes, but is not available in many centers and neither is it included in routine practice. The need for a noninvasive method to evaluate bone disease has promoted the development of multiple bone biomarkers and imaging techniques to assess bone metabolism, to clarify the risk of bone loss and fractures and to guide therapeutic decisions. However, all of these have important limitations and presently no noninvasive method either isolated or in association can replace bone biopsy. This article will discuss the major groups of bone biomarkers and new imaging techniques and their role in CKD-MBD

Cortical nephrocalcinosis asymmetrically involving the kidneys: a case report documenting the development via imaging

We report the case of a 38-year-old man with known human immunodeficiency virus infection who presented with severe Bordetella Bronchiseptica pneumoniae that led to septic shock and acute renal insufficiency, after which he developed bilateral cortical nephrocalcinosis, affecting most severely the right kidney with associated atrophy. The onset and progressive development of the findings were documented via both ultrasound and computed tomography. As far as we know, this is the first time that the development of cortical nephrocalcinosis has been documented via imaging, interestingly revealing a gradual decrease of the right kidney size, together with a change from a punctate pattern of cortical calcifications to a rim of calcifications

Alport Syndrome - A rare presentation

Introduction: Alport syndrome is a glomerular genetic disease progressing to chronic renal failure associated with deafness and ocular changes. Clinical presentation is usually in the first decade of life with microscopic haematuria and/or persistent proteinuria without hypertension or renal dysfunction. Case Report: Male, 4.5 years old, with an acute nephritic syndrome characterized by macroscopic haematuria, oedema, non-oliguric acute renal failure (maximal urea and creatinine of 25mmol/L and 150μmol/L, respectively), anaemia and proteinuria. Blood pressure normal. Normal immunoglobulins and complement fractions; anti-neutrophil cytoplasmic antibody, anti-nuclear antibody and anti-basal membrane antibody negative. History of recurrent episodes of macroscopic haematuria since the age of eighteen months associated with respiratory infections. No family history of renal disease or deafness. Renal biopsy showed proliferative glomerulonephritis with extracapillary crescentic activity, complete fragmentation of glomerular basement membranes and negative immunofluorescence. Pulse methylprednisolone was given followed by oral prednisolone and cyclophosphamide. Renal function recovered, microscopic haematuria persisted. At age 5, there was reappearance of proteinuria, worsening progressively and two years later he started treatment with enalapril. At age 11, a second renal biopsy revealed mesangial proliferative glomerulonephritis, small foci of glomerular sclerosis and few deposits of IgM on immunofluorescence. He started oral corticosteroids with partial response. Nine months later, bilateral sensorineural deafness was detected. At age 16, he maintains normal renal function, microscopic haematuria with manifest proteinuria. A mutation in homozygosity in the COL4A3 gene, compatible with autosomal recessive Alport syndrome, was identified. Conclusions: This case draws attention to an uncommon early course and clinical/pathological findings of a patient later diagnosed with Alport syndrome, with an initial good response to corticosteroids and cyclophosphamide. The case also illustrates the importance of kidney biopsy, including electron microscopy, in the diagnostic, classification and therapy in kidney diseases with unusual clinical course

72-year-old man with acute kidney injury, hypercalcemia and metastatic prostate câncer

Paraproteinemias are characterized by the abnormal expansion of a plasma cell clone with overproduction of a monoclonal (M) immunoglobulin. In rare cases (1%) two distinct M proteins can be identified (biclonal gammopathy). Renal manifestations are frequent and can present with several histological patterns. Prognosis and treatment are similar to monoclonal gammopathies varying according to extent of disease and response to therapy. We report a case of a 72-year-old man with a prior history of hypertension, dyslipidemia, and prostate cancer with bone metastasis under treatment with leuproline, cyproterone, and nonsteroidal anti-inflammatory drugs who was found to have anemia, acute kidney injury, and hypercalcemia. After clinical evaluation and workup, a biclonal multiple myeloma (IgG kappa and IgA lambda) and a cast nephropathy were diagnosed. The patient was started on renal replacement therapy and on CyBorDex treatment cycle protocol for two years with remission of multiple myeloma but without renal function recovery. During this period, there was no prostate cancer progression. This case report alerts us to the rarity of a biclonal multiple myeloma especially in a patient with advanced prostate cancer but also to the fact that not all osteolytic lesions are secondary lesions

Crescentic IgA nephropathy with preserved renal function

Crescentic glomerulonephritis is a severe form of glomerular inflammation. IgA nephropathy (IgAN) is the most common primary glomerulonephritis and rarely presents as crescentic glomerulonephritis with rapidly progressive glomerulonephritis. Treatment of IgAN includes renin-angiotensin system blockade and immunosuppression in select cases with persistent proteinuria and/or renal failure, which are also indications for biopsy. Prognosis markers guide treatment and crescents are recognized as an indication of powerful immunosuppression as they are associated with rapid decline of renal function. We describe a case of crescentic IgA nephropathy with preserved renal function that was associated with several episodes of severe tonsillitis. Renin-angiotensin system blockade and corticosteroids were used and renal function remains stable. To our knowledge, this is the third case described in the literature of a patient with crescents and IgA glomerular deposition but with preserved renal function. A more benign aspect of crescents and association with tonsillitis episodes may explain a more favorable prognosis