Familial vasculitides: granulomatosis with polyangitis and microscopic polyangitis in two brothers with differing anti-neutrophil cytoplasm antibody specificity
We report the unusual case of two brothers presenting with AAV with differing clinical pictures and differing ANCA specificity. There is a recently identified difference in genetic risk factors associated with ANCA specificity, making it surprising that first-degree relatives develop AAV with differing clinical and serological features. Our report illustrates the complex aetiology of AAV and suggests that further research on the interaction of genetic and environmental factors is needed. (Source: CKJ: Clinical Kidney Journal)
Source: CKJ: Clinical Kidney Journal - May 30, 2016 Category: Urology & Nephrology Authors: Prendecki, M., Cairns, T., Pusey, C. D. Tags: FAMILIAL NEPHROPATHIES Source Type: research
A novel heterozygous mutation in the ATP6V0A4 gene encoding the V-ATPase a4 subunit in an adult patient with incomplete distal renal tubular acidosis
A 40-year-old Japanese man who had a medical history of hypokalemic periodic paralysis 4 months prior was hospitalized to undergo a cholecystectomy. Hypokalemia, nephrocalcinosis and alkaluria suggesting distal renal tubular acidosis (dRTA) were detected, but metabolic acidosis was not evident. An ammonium chloride/furosemide–fludrocortisone/bicarbonate loading test demonstrated a remarkable disability in urinary H+ excretion. A novel heterozygous mutation in the ATP6V0A4 gene encoding the vacuolar H+-ATPase (V-ATPase) a4 subunit p.S544L was detected. Among cases of V-ATPase a4 mutations, this is the first case in wh...
Source: CKJ: Clinical Kidney Journal - May 30, 2016 Category: Urology & Nephrology Authors: Imai, E., Kaneko, S., Mori, T., Okado, T., Uchida, S., Tsukamoto, Y. Tags: FAMILIAL NEPHROPATHIES Source Type: research
Haematological malignancies and acute kidney injury requiring nephrology consultation: challenging the worst of the worst
Conclusions
Septic shock, IMV and allogeneic SCT were identified as independent predictors of death in patients with HM and AKI, with only a small chance of survival if all three were present. Depending on the combination of risk factors, the indication for aggressive life support therapies, such as RST, might be questionable. (Source: CKJ: Clinical Kidney Journal)
Source: CKJ: Clinical Kidney Journal - May 30, 2016 Category: Urology & Nephrology Authors: Chuva, T., Maximino, J., Barbosa, J., Silva, S., Paiva, A., Baldaia, J., Loureiro, A. Tags: ONCONEPHROLOGY Source Type: research
Severe acute interstitial nephritis after combination immune-checkpoint inhibitor therapy for metastatic melanoma
Immune-checkpoint inhibitors are emerging as revolutionary drugs for certain malignancies. However, blocking the co-inhibitory signals may lead to immune-related adverse events, mainly in the spectrum of autoimmune diseases including colitis, endocrinopathies and nephritis. Here, we report a case of a 75-year-old man with metastatic malignant melanoma treated with a combination of nivolumab (anti-PD1-antibody) and ipilimumab (anti-CTLA-4 antibody) who developed systemic rash along with severe acute tubulointerstitial nephritis after two doses of combination therapy. Kidney biopsy and peripheral blood immune profile reveale...
Source: CKJ: Clinical Kidney Journal - May 30, 2016 Category: Urology & Nephrology Authors: Murakami, N., Borges, T. J., Yamashita, M., Riella, L. V. Tags: ONCONEPHROLOGY Source Type: research
Recurrent focal segmental glomerulosclerosis apparently resistant to plasmapheresis improves after surgical repair of arteriovenous fistula aneurysms
We present a case of recurrent FSGS 6 years after transplantation. It is instructive because proteinuria did not respond to intensive plasmapheresis—combined with rituximab—until the possibility of ineffective apheresis secondary to multiple aneurysms in the arteriovenous fistula (AVF) was considered. Proteinuria improved soon after alternative access for plasmapheresis was secured and AVF aneurysms were surgically repaired. (Source: CKJ: Clinical Kidney Journal)
Source: CKJ: Clinical Kidney Journal - May 30, 2016 Category: Urology & Nephrology Authors: Ding, Y., Francis, J., Kalish, J., Deshpande, A., Quillen, K. Tags: TRANSPLANTATION AND GLOMERULONEPHRITIS Source Type: research
Kidney transplant outcomes in familial C3 glomerulopathy
We describe the long-term outcomes following kidney transplantation in an Irish family with familial C3 glomerulopathy due to a hybrid CFHR3-1 gene. (Source: CKJ: Clinical Kidney Journal)
Source: CKJ: Clinical Kidney Journal - May 30, 2016 Category: Urology & Nephrology Authors: Wong, L., Moran, S., Lavin, P. J., Dorman, A. M., Conlon, P. J. Tags: TRANSPLANTATION AND GLOMERULONEPHRITIS Source Type: research
Revisiting post-infectious glomerulonephritis in the emerging era of C3 glomerulopathy
Conclusions
Although the identification of abnormalities of the alternative pathway of complement is characteristic of C3GN, testing is not widely available and the turnaround time often exceeds 1 month. Our study found that PIGN with either co-dominant or dominant C3 deposition in a cohort of young patients has excellent short-term outcomes. Close clinical observation for persistent abnormalities, such as hypocomplementemia, prolonged hematuria or proteinuria, is recommended to single out patients that may harbor intrinsic complement abnormalities. (Source: CKJ: Clinical Kidney Journal)
Source: CKJ: Clinical Kidney Journal - May 30, 2016 Category: Urology & Nephrology Authors: Khalighi, M. A., Wang, S., Henriksen, K. J., Bock, M., Keswani, M., Meehan, S. M., Chang, A. Tags: GLOMERULONEPHRITIS Source Type: research
Efficacy and safety of adrenocorticotropic hormone treatment in glomerular diseases: a systematic review and meta-analysis
Conclusions
ACTH is a well-tolerated therapy and is most promising when treating patients with MN. There may be a potential role for ACTH in patients with MCD and FSGS, but data are lacking. (Source: CKJ: Clinical Kidney Journal)
Source: CKJ: Clinical Kidney Journal - May 30, 2016 Category: Urology & Nephrology Authors: Kittanamongkolchai, W., Cheungpasitporn, W., Zand, L. Tags: GLOMERULONEPHRITIS Source Type: research
Therapeutic variability in adult minimal change disease and focal segmental glomerulosclerosis
Conclusions
We found variation from the guidelines in the duration of initial and tapered steroid therapy, in the medical criteria for classifying a steroid-resistant condition and in the chosen treatment for the second-line treatment. All nephrologists started with a daily dose of steroids as the first-line treatment. The most frequently used steroid-sparing drug was calcineurin inhibitors. Cyclophosphamide use was much lower than expected. (Source: CKJ: Clinical Kidney Journal)
Source: CKJ: Clinical Kidney Journal - May 30, 2016 Category: Urology & Nephrology Authors: Fernandez-Juarez, G., Villacorta, J., Ruiz-Roso, G., Panizo, N., Martinez-Marin, I., Marco, H., Arrizabalaga, P., Diaz, M., Perez-Gomez, V., Vaca, M., Rodriguez, E., Cobelo, C., Fernandez, L., Avila, A., Praga, M., Quereda, C., Ortiz, A. Tags: GLOMERULONEPHRITIS Source Type: research
Fat tissue and inflammation in patients undergoing peritoneal dialysis
Conclusions
Follow-up of weight and body mass index can underestimate the fat mass increase and miss lean mass loss. The increase in fat mass is associated with proinflammatory state and alteration in lipid profile. (Source: CKJ: Clinical Kidney Journal)
Source: CKJ: Clinical Kidney Journal - May 30, 2016 Category: Urology & Nephrology Authors: Rincon Bello, A., Bucalo, L., Abad Estebanez, S., Vega Martinez, A., Barraca Nunez, D., Yuste Lozano, C., Perez de Jose, A., Lopez-Gomez, J. M. Tags: FAT AND ADIPOKINES Source Type: research
Adipocytokines in renal transplant recipients
In the last two decades, perceptions about the role of body fat have changed. Adipocytes modulate endocrine and immune homeostasis by synthesizing hundreds of hormones, known as adipocytokines. Many studies have been investigating the influences and effects of these adipocytokines and suggest that they are modulated by the nutritional and immunologic milieu. Kidney transplant recipients (KTRs) are a unique and relevant population in which the function of adipocytokines can be examined, given their altered nutritional and immune status and subsequent dysregulation of adipocytokine metabolism. In this review, we summarize th...
Source: CKJ: Clinical Kidney Journal - May 30, 2016 Category: Urology & Nephrology Authors: Nagy, K., Nagaraju, S. P., Rhee, C. M., Mathe, Z., Molnar, M. Z. Tags: FAT AND ADIPOKINES Source Type: research
The cell cycle biomarkers: promising research, but do not oversell them
This review focuses on the most recent scientific and clinical information on the development and clinical applicability of the cell cycle biomarkers TIMP-2 and IGFBP-7 in the diagnosis and prognosis of patients at risk for and suffering from acute kidney injury (AKI). A number of evaluation studies have demonstrated that compared with existing biomarkers, urinary excretion of the product of both biomarkers, [TIMP-2]•[IGFBP-7], improved diagnostic performance in assessing the risk for AKI, predicting the need for renal replacement therapy, AKI-related complications and short- and long-term prognoses. The reference int...
Source: CKJ: Clinical Kidney Journal - May 30, 2016 Category: Urology & Nephrology Authors: Lameire, N., Vanmassenhove, J., Van Biesen, W., Vanholder, R. Tags: TRANSLATIONAL NEPHROLOGY Source Type: research
Omics databases on kidney disease: where they can be found and how to benefit from them
In the recent decades, the evolution of omics technologies has led to advances in all biological fields, creating a demand for effective storage, management and exchange of rapidly generated data and research discoveries. To address this need, the development of databases of experimental outputs has become a common part of scientific practice in order to serve as knowledge sources and data-sharing platforms, providing information about genes, transcripts, proteins or metabolites. In this review, we present omics databases available currently, with a special focus on their application in kidney research and possibly in clin...
Source: CKJ: Clinical Kidney Journal - May 30, 2016 Category: Urology & Nephrology Authors: Papadopoulos, T., Krochmal, M., Cisek, K., Fernandes, M., Husi, H., Stevens, R., Bascands, J.-L., Schanstra, J. P., Klein, J. Tags: TRANSLATIONAL NEPHROLOGY Source Type: research
Feasibility of a hemodialysis safety checklist for nurses and patients: a quality improvement study
Conclusions
The Hemo Pause safety checklist was acceptable to both nurses and patients over 3 months. Our next step is to spread this checklist locally and conduct a mixed methods study to determine mechanisms by which its use may improve safety culture and reduce adverse events. (Source: CKJ: Clinical Kidney Journal)
Source: CKJ: Clinical Kidney Journal - May 30, 2016 Category: Urology & Nephrology Authors: Thomas, A., Silver, S. A., Rathe, A., Robinson, P., Wald, R., Bell, C. M., Harel, Z. Tags: PATIENT SAFETY Source Type: research
Announcements
(Source: CKJ: Clinical Kidney Journal)
Source: CKJ: Clinical Kidney Journal - March 15, 2016 Category: Urology & Nephrology Tags: ANNOUNCEMENTS Source Type: research
