Blueprint for a European calciphylaxis registry initiative: the European Calciphylaxis Network (EuCalNet)
Calcific uraemic arteriolopathy (CUA) is a rare disease and continues to be a clinical challenge. The typical course of CUA is characterized by painful skin discolouration and induration evolving to necrotic ulcerations. Medial calcification of cutaneous arterioles and extensive extracellular matrix remodelling are the hallmarks of CUA. The epidemiology and risk factors associated with this disease are still not fully understood. Moreover, CUA treatment strategies vary significantly among centres and expert recommendations are heterogeneous. Registries may provide important insights and information to increase our knowledg...
Source: CKJ: Clinical Kidney Journal - September 24, 2015 Category: Urology & Nephrology Authors: Brandenburg, V., Adragao, T., van Dam, B., Evenepoel, P., Frazao, J. M., Ketteler, M., Mazzaferro, S., Urena Torres, P., Ramos, R., Torregrosa, J.-V., Cozzolino, M., on behalf of the CKD-MBDWG ERA-EDTA, collaborators: Ziad Massy, Adrian Covic, Marc Vervlo Tags: CKD-MBD Source Type: research
Albuminuria, renal dysfunction and circadian blood pressure rhythm in older men: a population-based longitudinal cohort study
Conclusions
UAER associates with circadian BP rhythm variation and non-dipper progression in elderly men. Concurrent renal dysfunction modifies and exacerbates these associations. (Source: CKJ: Clinical Kidney Journal)
Source: CKJ: Clinical Kidney Journal - September 24, 2015 Category: Urology & Nephrology Authors: Xu, H., Huang, X., Riserus, U., Cederholm, T., Sjogren, P., Lindholm, B., Arnlov, J., Carrero, J. J. Tags: HYPERTENSION Source Type: research
Pathophysiological role of different tubular epithelial cell death modes in acute kidney injury
The histological substrate of many forms of intrinsic acute kidney injury (AKI) has been classically attributed to tubular necrosis. However, more recent studies indicate that necrosis is not the main form of cell death in AKI and that other forms such as apoptosis, regulated necrosis (i.e. necroptosis and parthanatos), autophagic cell death and mitotic catastrophe, also participate in AKI and that their contribution depends on the cause and stage of AKI. Herein, we briefly summarize the main characteristics of the major types of cell death and we also critically review the existing evidence on the occurrence of different ...
Source: CKJ: Clinical Kidney Journal - September 24, 2015 Category: Urology & Nephrology Authors: Sancho-Martinez, S. M., Lopez-Novoa, J. M., Lopez-Hernandez, F. J. Tags: AKI Source Type: research
Collagenofibrotic glomerulopathy
Conclusion
Collagenofibrotic glomerulopathy is a rare disease that appears to occur more frequently in adult Indian populations in a sporadic, non-familial manner. To our knowledge, this is the largest cases series of collagenofibrotic glomerulopathy in an adult population. (Source: CKJ: Clinical Kidney Journal)
Source: CKJ: Clinical Kidney Journal - September 24, 2015 Category: Urology & Nephrology Authors: Kurien, A. A., Larsen, C. P., Cossey, L. N. Tags: HEREDITARY AND RARE NEPHROPATHIES Source Type: research
Variability in phenotype induced by the podocin variant R229Q plus a single pathogenic mutation
Conclusions
These cases highlight the phenotypic variability associated with the NPHS2 R229Q variant plus pathogenic mutation. Individuals may present with early aggressive disease. (Source: CKJ: Clinical Kidney Journal)
Source: CKJ: Clinical Kidney Journal - September 24, 2015 Category: Urology & Nephrology Authors: Phelan, P. J., Hall, G., Wigfall, D., Foreman, J., Nagaraj, S., Malone, A. F., Winn, M. P., Howell, D. N., Gbadegesin, R. Tags: HEREDITARY AND RARE NEPHROPATHIES Source Type: research
Understanding the physical and emotional impact of early-stage ADPKD: experiences and perspectives of patients and physicians
Conclusions
Early-stage ADPKD can have a significant physical and emotional impact on patients. Whilst some physicians have an awareness of patient experience during early-stage disease, most underestimate the impact of ADPKD. Both patients and physicians are negatively affected by their inability to alter disease progression. (Source: CKJ: Clinical Kidney Journal)
Source: CKJ: Clinical Kidney Journal - September 24, 2015 Category: Urology & Nephrology Authors: Baker, A., King, D., Marsh, J., Makin, A., Carr, A., Davis, C., Kirby, C. Tags: HEREDITARY AND RARE NEPHROPATHIES Source Type: research
Etiological diagnosis of granulomatous tubulointerstitial nephritis in the tropics
Conclusion
Etiological diagnosis of GIN is essential for timely and appropriate therapy. Tuberculosis is the commonest etiology (53%) in the tropics. Necrosis in granuloma, demonstration of acid fast bacilli, blood interferon gamma release assay and urine culture is not sensitive for the diagnosis of tuberculosis in GIN. Our findings suggest that tissue PCR for tuberculosis performed in an appropriate clinical setting is useful in the diagnostic evaluation of GIN. (Source: CKJ: Clinical Kidney Journal)
Source: CKJ: Clinical Kidney Journal - September 24, 2015 Category: Urology & Nephrology Authors: Agrawal, V., Kaul, A., Prasad, N., Sharma, K., Agarwal, V. Tags: GRANULOMATOUS INTERSTITIAL NEPHRITIS Source Type: research
Granulomatous interstitial nephritis
We describe a patient with a history of multiple spinal surgeries complicated by wound infection who presented with confusion and rash with subsequent development of acute kidney injury. Urinalysis demonstrated pyuria and eosinophiluria, and renal biopsy revealed acute interstitial nephritis with granulomas. These findings were attributed to doxycycline treatment of his wound infection. This review explores the clinical associations, presentation, diagnosis, and treatment of this uncommon cause of acute kidney injury. (Source: CKJ: Clinical Kidney Journal)
Source: CKJ: Clinical Kidney Journal - September 24, 2015 Category: Urology & Nephrology Authors: Shah, S., Carter-Monroe, N., Atta, M. G. Tags: GRANULOMATOUS INTERSTITIAL NEPHRITIS Source Type: research
Granulomatous interstitial nephritis: a chameleon in a globalized world
(Source: CKJ: Clinical Kidney Journal)
Source: CKJ: Clinical Kidney Journal - September 24, 2015 Category: Urology & Nephrology Authors: Aleckovic-Halilovic, M., Nel, D., Woywodt, A. Tags: GRANULOMATOUS INTERSTITIAL NEPHRITIS Source Type: research
A European multicentre and open-label controlled randomized trial to evaluate the efficacy of Sequential treatment with TAcrolimus-Rituximab versus steroids plus cyclophosphamide in patients with primary MEmbranous Nephropathy: the STARMEN study
Background
Patients with primary membranous nephropathy (MN) and persistent nephrotic syndrome have a high risk of progression to end-stage renal disease. The Ponticelli protocol (steroids with alkylating agents) is the most effective immunosuppressive therapy for this condition, but it has severe adverse effects. Tacrolimus and rituximab have demonstrated efficacy for remission of nephrotic syndrome in MN with a safer profile. However, the published evidence is largely based on small or short-term observational studies, historical cohorts, comparisons with conservative therapy or clinical trials without appropriate contro...
Source: CKJ: Clinical Kidney Journal - September 24, 2015 Category: Urology & Nephrology Authors: Rojas-Rivera, J., Fernandez-Juarez, G., Ortiz, A., Hofstra, J., Gesualdo, L., Tesar, V., Wetzels, J., Segarra, A., Egido, J., Praga, M., on behalf of the STARMEN Investigators Tags: GLOMERULONEPHRITIS Source Type: research
Unmet medical needs in lupus nephritis: solutions through evidence-based, personalized medicine
Lupus nephritis (LN) remains a kidney disease with significant unmet medical needs despite extensive clinical and translational research over the past decade. These include the need to (i) predict the individual risk for LN in a patient with systemic lupus erythematosus, (ii) identify the best therapeutic option for an individual patient, (iii) distinguish chronic kidney damage from active immunologic kidney injury, (iv) develop efficient treatments with acceptable or no side effects and improve the design of randomized clinical trials so that effective drugs demonstrate efficacy. This review discusses the underlying reaso...
Source: CKJ: Clinical Kidney Journal - September 24, 2015 Category: Urology & Nephrology Authors: Anders, H.-J., Weidenbusch, M., Rovin, B. Tags: GLOMERULONEPHRITIS Source Type: research
Use of eculizumab in crescentic IgA nephropathy: proof of principle and conundrum?
We report here a 16-year-old male with the vasculitic form of IgAN who failed to respond to aggressive conventional therapy including high-dose steroids, cyclophosphamide and plasma exchange and who was treated with four weekly doses of 900 mg eculizumab followed by a single dose of 1200 mg. He responded rapidly to this treatment and has had a stable creatinine around 150 µmol/L (1.67 mg/dL) for >6 months. However, proteinuria was unabated on maximal conventional anti-proteinuric treatment, and a repeat renal biopsy 11 months after presentation revealed severe chronic changes. We believe this case provides proof o...
Source: CKJ: Clinical Kidney Journal - September 24, 2015 Category: Urology & Nephrology Authors: Ring, T., Pedersen, B. B., Salkus, G., Goodship, T. H. J. Tags: GLOMERULONEPHRITIS Source Type: research
Clinical features of IgA nephropathy with serum ANCA positivity: a retrospective case-control study
Conclusions
IgAN patients with ANCA positivity showed more severe clinical and histological features when compared with ANCA-negative IgAN patients and were comparable to AASV patients. However, renal prognosis was relatively better in ANCA-positive crescentic IgAN patients after aggressive immunosuppressive therapy in the short term, compared with ANCA-negative patients. (Source: CKJ: Clinical Kidney Journal)
Source: CKJ: Clinical Kidney Journal - September 24, 2015 Category: Urology & Nephrology Authors: Yang, Y.-z., Shi, S.-F., Chen, Y.-Q., Chen, M., Yang, Y.-H., Xie, X.-F., Zou, R., Lv, J.-C., Liu, L.-J., Zhang, H. Tags: GLOMERULONEPHRITIS Source Type: research
Rapidly progressive IgA nephropathy: a form of vasculitis or a complement-mediated disease?
A rapidly progressive and crescentic IgA nephropathy (IgAN) is uncommon, but it has a high risk of progression to end-stage renal disease and variable response to immunosuppression. The importance of a positive anti-neutrophil cytoplasmic antibody (ANCA) serology in this group of patients is not fully understood but may have prognostic significance. On the other hand, there is growing evidence of the role of complement in the pathogenesis of IgAN, especially in cases of crescentic IgAN. Therapies directed against the complement system are a potential and rational therapeutic approach. In this issue, two clinical studies of...
Source: CKJ: Clinical Kidney Journal - September 24, 2015 Category: Urology & Nephrology Authors: Rojas-Rivera, J., Fernandez-Juarez, G., Praga, M. Tags: GLOMERULONEPHRITIS Source Type: research
Announcements
(Source: CKJ: Clinical Kidney Journal)
Source: CKJ: Clinical Kidney Journal - July 27, 2015 Category: Urology & Nephrology Tags: ANNOUNCEMENTS Source Type: research
