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A Genetic Variant Associated with the Five Vascular Diseases Is a Distal Regulator of Endothelin-1 Gene Expression
Gupta et al. [Cell 2017; 170: 522 –533] have shown that a genetic variant associated with the 5 vascular diseases is a distal regulator of endothelin (ET)-1 gene (EDN1) expression. A common single nucleotide polymorphism (SNP) in the third intron of thePHACTR1 gene, rs9349379, is the potential variant responsible for increased risk of coronary artery disease, and lower risk of migraine, carotid dissection, fibromuscular dysplasia, and hypertension. Since ET-1 acts through ET type B receptors (ETBR) on endothelial cells to stimulate the production of nitric oxide and prostacyclin and induce vasodilation, this may cont...
Source: Nephron - February 15, 2018 Category: Urology & Nephrology Source Type: research

Low Cerebral Oxygenation Is Associated with Cognitive Impairment in Chronic Hemodialysis Patients
Conclusion: Decreased brain oxygenation is associated with weaker cognitive performance in patients undergoing chronic HD. Further understanding the causes of cerebral ischemia in HD patients could lead to the prevention of cognitive decline in this population.Nephron (Source: Nephron)
Source: Nephron - February 13, 2018 Category: Urology & Nephrology Source Type: research

< b > < i > Mannose-Binding Lectin2 < /i > < /b > Gene Polymorphism and IgG4 in Membranous Nephropathy
Conclusions: Our data suggests thatMBL2 polymorphisms may be associated with the activation of the lectin pathway by IgG4 subclass antibodies in MN.Nephron (Source: Nephron)
Source: Nephron - February 13, 2018 Category: Urology & Nephrology Source Type: research

Nephrocalcinosis in Amelogenesis Imperfecta Caused by the < b > < i > FAM20A < /i > < /b > Mutation
Conclusion: AI patients with similar clinical phenotypes andFAM20A mutations should be examined for nephropathy even if they lack pertinent symptoms. Nephrology referral is warranted for patients who have clinical phenotypes related to AI-gingival fibromatosis even if they are not symptomatic.Nephron (Source: Nephron)
Source: Nephron - February 13, 2018 Category: Urology & Nephrology Source Type: research

What We Do and Do Not Know about Women and Kidney Diseases; Questions Unanswered and Answers Unquestioned: Reflection on World Kidney Day and International Women ’s Day
Chronic kidney disease affects approximately 10% of the world ’s adult population: it is within the top 20 causes of death worldwide, and its impact on patients and their families can be devastating. World Kidney Day and International Women’s Day in 2018 coincide, thus offering an opportunity to reflect on the importance of women’s health and specificall y their kidney health, on the community and the next generations, as well as to strive to be more curious about the unique aspects of kidney disease in women so that we may apply those learnings more broadly. Girls and women, who make up approximately 50%...
Source: Nephron - February 13, 2018 Category: Urology & Nephrology Source Type: research

Assessing Extracellular Volume in Hemodialysis Patients Using Intradialytic Blood Pressure Slopes
Conclusions: In hypertensive hemodialysis patients, measurements of ECV excess are more strongly associated with IBPSs than with pre-dialysis, post-dialysis, or change in systolic BP. Among varying volume metrics, post-dialysis ECW/weight has the strongest association with these slopes. Determining IBPS is a novel method to optimize clinical assessment of ECV in hemodialysis patients.Nephron (Source: Nephron)
Source: Nephron - February 13, 2018 Category: Urology & Nephrology Source Type: research

< b > < i > Campylobacter rectus < /i > < /b > in the Oral Cavity Correlates with Proteinuria in Immunoglobulin A Nephropathy Patients
Conclusion: Our results suggest that harbouringC. rectus in the oral cavity could be associated with proteinuria in IgAN patients.Nephron (Source: Nephron)
Source: Nephron - February 9, 2018 Category: Urology & Nephrology Source Type: research

Serum Soluble Interleukin 2 Receptor Level as a Marker of Acute Rejection in Pediatric Kidney Transplant Recipients
Conclusion: Serum sIL2R level may serve as a noninvasive diagnostic indicator in pediatric kidney transplant recipients experiencing AR.Nephron (Source: Nephron)
Source: Nephron - February 8, 2018 Category: Urology & Nephrology Source Type: research

Restless Legs Syndrome: An  Unresolved Uremic Disorder after Renal Transplantation
Conclusion: The prevalence of RLS after transplantation remains high (14.8%). This condition is twice more prevalent for females. Contribution of RAAS, graft function and phosphate overload requires further investigation.Nephron (Source: Nephron)
Source: Nephron - February 1, 2018 Category: Urology & Nephrology Source Type: research

Vascular Endothelial Growth Factor-A < sub > 165 < /sub > b Restores Normal Glomerular Water Permeability in a Diphtheria-Toxin Mouse Model of Glomerular Injury
Conclusion: Although only a mild model of podocyte injury, over-expression of the anti-permeability VEGF-A165b isoform in the podocytes of Pod-DTR mice had a protective effect. Therefore, this study further highlights the therapeutic potential of VEGF-A165b in glomerular disease.Nephron (Source: Nephron)
Source: Nephron - January 26, 2018 Category: Urology & Nephrology Source Type: research

Ultra-Low Contrast Volume for Patients with Advanced Chronic Kidney Disease Undergoing Coronary Procedures
Conclusions: In patients with advanced chronic kidney disease, the ultra-low contrast technique is feasible and effective and can be used safely without a significant deterioration in renal function. This technique may increase the utilization of PCI in high-risk coronary patients with chronic kidney disease.Nephron (Source: Nephron)
Source: Nephron - January 24, 2018 Category: Urology & Nephrology Source Type: research

Clinical Heterogeneity in Familial IgA Nephropathy
Conclusion: These data suggests familial aggregation of IgA nephropathy, confirm the clinical and histopathological heterogeneity and raise the possibility of monogenic inheritance.Nephron (Source: Nephron)
Source: Nephron - January 19, 2018 Category: Urology & Nephrology Source Type: research

Association of Ultrafiltration Rate with Mortality in Incident Hemodialysis Patients
Conclusions: Higher UFR is independently associated with higher all-cause and CV mortality in incident HD patients. Clinical trials are warranted to examine the effects of lowering UFR on outcomes.Nephron (Source: Nephron)
Source: Nephron - January 18, 2018 Category: Urology & Nephrology Source Type: research

The < b > < i > von Hippel-Lindau < /i > < /b > Gene Is Required to Maintain Renal Proximal Tubule and Glomerulus Integrity in Zebrafish Larvae
Conclusions: Our data indicates thatvhl is required to maintain pronephric tubule and glomerulus integrity during zebrafish development, and suggests a role for VHL in endocytic vesicle trafficking.Nephron (Source: Nephron)
Source: Nephron - January 17, 2018 Category: Urology & Nephrology Source Type: research

Unravelling Pathophysiology of Crystalline Nephropathy in Ceftriaxone-Associated Acute Kidney Injury: A Cellular Proteomic Approach
Conclusions: This study, for the first time, used the in vitro model of crystalline nephropathy to investigate the underlying pathophysiology of ceftriaxone-associated AKI, which should be investigated in vivo for potential clinical benefits in the future.Nephron (Source: Nephron)
Source: Nephron - January 16, 2018 Category: Urology & Nephrology Source Type: research

Erratum
Nephron 2018;138:88 (Source: Nephron)
Source: Nephron - January 11, 2018 Category: Urology & Nephrology Source Type: research

Hospital Readmission among New Dialysis Patients Associated with Young Age and Poor Functional Status
Conclusions: Overall, 30-day readmissions are common within the first year of dialysis start. The first 10-day period after discharge, young patients, and those with poor functional status represent key areas for targeted interventions to reduce readmissions.Nephron (Source: Nephron)
Source: Nephron - January 9, 2018 Category: Urology & Nephrology Source Type: research

Safety of Outpatient Kidney Biopsies
Conclusion: We found that kidney biopsy performed in an outpatient setting in select patients is only rarely associated with adverse events and is a safe procedure.Nephron (Source: Nephron)
Source: Nephron - January 4, 2018 Category: Urology & Nephrology Source Type: research

Associations of Fibroblast Growth Factor 23 with Markers of Inflammation and Leukocyte Transmigration in Chronic Kidney Disease
Conclusion: FGF23 levels are associated with various inflammatory markers such as pro-inflammatory cytokines and adhesion molecules on innate immune cells. However, further studies are warranted to define the direct role of FGF23 in modulation of the innate immune system in CKD.Nephron (Source: Nephron)
Source: Nephron - January 4, 2018 Category: Urology & Nephrology Source Type: research

Vanishing Immunoglobulins: The Formation of Pauci-Immune Lesions in Myeloperoxidase-Antineutrophil Cytoplasmic Antibody-Associated Vasculitis
Nephron (Source: Nephron)
Source: Nephron - December 21, 2017 Category: Urology & Nephrology Source Type: research

Renal PGC1 α May Be Associated with Recovery after Delayed Graft Function
Conclusions: These data suggest that higher PGC1 α expression is associated with faster and more complete recovery from DGF. Mitochondrial biogenesis may be a therapeutic target for DGF. Larger studies are needed to validate these findings.Nephron (Source: Nephron)
Source: Nephron - December 21, 2017 Category: Urology & Nephrology Source Type: research

Factors Associated with Chronic Kidney Disease and Their Clinical Utility in Primary Care Clinics in a Multi-Ethnic Southeast Asian Population
Conclusions: CKD prevalence in our Southeast Asian population is high and under-documented even in high-risk patients. Our findings highlight factors associated with CKD, and the predictive value of hypertension, diabetes, and advancing age as EHR-based screening targets for CKD. Our results also suggest that complementary educational efforts will be needed to increase physician detection and optimize the management of CKD, especially in high risk and marginalized groups across all clinics in Singapore, and possibly in the region.Nephron (Source: Nephron)
Source: Nephron - December 15, 2017 Category: Urology & Nephrology Source Type: research

Erratum
Nephron (Source: Nephron)
Source: Nephron - December 14, 2017 Category: Urology & Nephrology Source Type: research

Atypical Hemolytic Uremic Syndrome Associated with Complement Factor H Mutation and IgA Nephropathy: A Case Report Successfully Treated with Eculizumab
We present a rare case of IgA nephropathy in a patient who developed atypical hemolytic uremic syndrome (aHUS) associated with a complement factor H (CFH) gene mutation, and who was successfully treated with eculizmab. A 76-year-old man was admitted as the patients had thrombotic microangiopathies findings. The patient was treated with plasma exchange, hemodialysis and methylprednisolone. A disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 level was not decreased. Light microscopy findings were consistent with hemolytic uremic syndrome and immunofluorescence analysis revealed IgA and C3 were d...
Source: Nephron - December 14, 2017 Category: Urology & Nephrology Source Type: research

Peritoneal Dialysis Catheter Revision and Replacement by Nephrologist for Peritoneal Dialysis Catheter Malfunction
Conclusion: PD catheter revision and replacement by nephrologist has an acceptable catheter survival and a reasonable complication rate. Given that prompt intervention is an important consideration, catheter revision and replacement by nephrologist is a suitable method for the management of catheter malfunction.Nephron (Source: Nephron)
Source: Nephron - December 14, 2017 Category: Urology & Nephrology Source Type: research

Impact of Kidney Transplantation on Reproductive Hormone Levels in Males: A Longitudinal Study
Conclusion: Sertoli cell function, based on AMH and inhibin B levels, does not improve to the same extent or as fast as Leydig cell function after kidney transplantation, as determined by testosterone and LH levels.Nephron (Source: Nephron)
Source: Nephron - December 14, 2017 Category: Urology & Nephrology Source Type: research

Renoprotective Effects of Metformin
Conclusion: Based on experimental evidence and some relevant clinical observations, metformin seems to be a promising drug in the treatment of progressive renal damage. RCT studies are the next essential step.Nephron (Source: Nephron)
Source: Nephron - December 14, 2017 Category: Urology & Nephrology Source Type: research

Vaccination Practices in Pediatric Dialysis Patients Across Europe. A European Pediatric Dialysis Working Group and European Society for Pediatric Nephrology Dialysis Working Group Study
Conclusion: There are variations in vaccination practice across Europe. Children with CKD, those undergoing dialysis, and transplant candidates should receive age-appropriate vaccinations before RTx as well as before the transition to adult nephrology clinics and antibody levels should be monitored to evaluate the immunization status before and after RTx.Nephron (Source: Nephron)
Source: Nephron - December 12, 2017 Category: Urology & Nephrology Source Type: research

miR302a-3p May Modulate Renal Epithelial-Mesenchymal Transition in Diabetic Kidney Disease by Targeting ZEB1
Conclusions: These findings suggest that miR-302a-3p may play a protective role by targeting ZEB1 in renal EMT in DKD. In view of these findings, it is conceivable that miR-302a-3p may serve as a potential novel target in pre-EMT states for the amelioration renal fibrosis seen in DKD.Nephron (Source: Nephron)
Source: Nephron - December 8, 2017 Category: Urology & Nephrology Source Type: research

Real or Perceived: Hyperkalemia Is a Major Deterrent for Renin-Angiotensin Aldosterone System Inhibition in Heart Failure
Nephron (Source: Nephron)
Source: Nephron - December 5, 2017 Category: Urology & Nephrology Source Type: research

Acknowledgement to Reviewers
Nephron 2017;137:310-312 (Source: Nephron)
Source: Nephron - November 30, 2017 Category: Urology & Nephrology Source Type: research

Contents Vol. 137, 2017
Nephron 2017;137:I-VI (Source: Nephron)
Source: Nephron - November 30, 2017 Category: Urology & Nephrology Source Type: research

Focal Segmental Glomerulosclerosis Associated with Chronic Progressive External Ophthalmoplegia and Mitochondrial DNA A3243G Mutation
Focal segmental glomerulosclerosis (FSGS) is caused by various etiologies, with mitochondrial dysfunction being one of the causes. FSGS is known to be associated with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS), which is a subclass of mitochondrial disease. However, it has rarely been reported in other mitochondrial disease subclasses. Here, we reported a 20-year-old man diagnosed with FSGS associated with chronic progressive external ophthalmoplegia (CPEO) due to mitochondrial DNA (mtDNA) 3243A#x3e;G mutation. He presented with left ptosis, short stature, mild sensorineural deafness,...
Source: Nephron - November 30, 2017 Category: Urology & Nephrology Source Type: research

Hyperkalemia Associated with Renin-Angiotensin-Aldosterone System Inhibition in Patients with Heart Failure; Underexplored and Overrated?
Nephron (Source: Nephron)
Source: Nephron - November 24, 2017 Category: Urology & Nephrology Source Type: research

An Unexpected Lesson in Global Nephrology
Nephron (Source: Nephron)
Source: Nephron - November 23, 2017 Category: Urology & Nephrology Source Type: research

Digoxin and Hypermagnesuria
In a recent issue of Nephron, Abu-Amer et al.[1] reported the presence of hypermagnesuria in patients following acute intravenous administration of digoxin and suggested that the Na+/K+-ATPase γ-subunit, which is the pharmacological target of digoxin, can play a role in this process. Hypermagnesuria induced by digoxin may have important clinical consequences, particularly in the presence of inherited and acquired conditions associated with hypermagnesuria and hypomagnesemia. Moreover, th e co-administration of digoxin with other drugs that reduce gastrointestinal absorption (i.e., proton pump inhibitors) or increase ...
Source: Nephron - November 23, 2017 Category: Urology & Nephrology Source Type: research

Acute Kidney Injury in Elderly Population: A Prospective Observational Study
Conclusions: The severity of AKI and the need for RRT were risk factors for mortality and dependence on dialysis. Antecedents of CKD seem to be associated with a poor renal outcome following an AKI episode. Starting RRT had an impact on the clinical decision to enroll these patients into palliative care.Nephron (Source: Nephron)
Source: Nephron - November 23, 2017 Category: Urology & Nephrology Source Type: research

Care of the Acute Kidney Injury Survivor
While the short-term implications of acute kidney injury (AKI) have been known for many years, far less attention has been paid to the health of AKI survivors. This was perhaps fueled by a prevailing wisdom that if a patient was fortunate enough to survive the primary illness associated with AKI, the prognosis for future health was auspicious. More recently, this dogma was challenged by data suggesting that after an episode of AKI, patients remain at risk of experiencing multiple adverse health outcomes. Greater recognition and appreciation of the risks faced by AKI survivors have led to the development of quality improvem...
Source: Nephron - November 13, 2017 Category: Urology & Nephrology Source Type: research

An Introduction to Contrast-Enhanced Ultrasound for Nephrologists
In conclusion, CEUS is an emerging imaging technique that may have clinically useful applications for detecting malignant kidney lesions, specifically in patients with CKD. However, most of the current data come from small, single-center studies, and larger, multicenter studies are needed.Nephron (Source: Nephron)
Source: Nephron - November 9, 2017 Category: Urology & Nephrology Source Type: research

Spurious Hyperchloremia in the Presence of Elevated Plasma Salicylate: A Cohort Study
Conclusion: Circulating levels of salicylate can interfere with chloride measured by using routine techniques, resulting in spurious hyperchloremia outcomes and erroneous AG values. In patients with acute metabolic acidosis and abnormally reduced or negative AG, salicylate interference with chloride measurement should be suspected.Nephron (Source: Nephron)
Source: Nephron - November 9, 2017 Category: Urology & Nephrology Source Type: research

Value of Intraoperative Parathyroid Hormone Assay during Parathyroidectomy in Dialysis and Renal Transplant Patients with Secondary and Tertiary Hyperparathyroidism
Conclusions: The 20-ioPTH is a good indicator of long-term PTH levels in dialysis and renal transplant patients. Hypocalcemia is a common complication, particularly in dialysis patients, and it is the main reason for readmission after PTX. Hypoparathyroidism is a potential concern after PTX in dialysis patients.Nephron (Source: Nephron)
Source: Nephron - November 9, 2017 Category: Urology & Nephrology Source Type: research

Heritability of End-Stage Renal Disease: A Swedish Adoption Study
Conclusion: The family history of ESRD in a biological parent is an important risk factor for ESRD. The high heritability indicates that genetic factors play an important role in understanding the etiology of ESRD.Nephron (Source: Nephron)
Source: Nephron - November 9, 2017 Category: Urology & Nephrology Source Type: research

Chloride Intracellular Channel 4 Overexpression in the Proximal Tubules of Kidneys from the Spontaneously Hypertensive Rat: Insight from Proteomic Analysis
Conclusions: Our studies provide evidence that major changes occur in the renal parenchyma from early stages of the development of hypertension. The overexpression of CLIC4 suggests that alterations in the proximal tubular compartment during hypertension should be further examined and that CLIC4 may be a useful early marker of renal tubular alterations due to elevated blood pressure.Nephron (Source: Nephron)
Source: Nephron - November 6, 2017 Category: Urology & Nephrology Source Type: research

Detection of a Splice Site Variant in a Patient with Glomerulopathy and Fibronectin Deposits
Conclusion: Intronic variants can cause GFND. Minigene analysis is useful for determining the pathogenicity of the intronic variants and could be used for all inherited kidney diseases.Nephron (Source: Nephron)
Source: Nephron - November 3, 2017 Category: Urology & Nephrology Source Type: research

Changing Paradigms in Acute Kidney Injury: From Mechanisms to Management
Nephron 2017;137:251-252 (Source: Nephron)
Source: Nephron - November 3, 2017 Category: Urology & Nephrology Source Type: research

Points of Concern in Post Acute Kidney Injury Management
The incidence of acute kidney injury (AKI) will in the future remain high, partly due to an increase in comorbidities and other AKI favoring factors such as the rise in high-risk diagnostic and therapeutic interventions. AKI has emerged as a major public health concern with high human and financial costs. It has recently been demonstrated that patients surviving an AKI episode show increased all-cause mortality, chronic kidney disease (CKD), ESRD, cardiovascular events, and reduced quality of life. Although it is important to acknowledge that, after an AKI episode, the risk of dying by far exceeds the risk of developing in...
Source: Nephron - November 2, 2017 Category: Urology & Nephrology Source Type: research

Erratum
Nephron 2017;137:250 (Source: Nephron)
Source: Nephron - October 31, 2017 Category: Urology & Nephrology Source Type: research

Hypermagnesuria in Humans Following Acute Intravenous Administration of Digoxin
Conclusions: The hypermagnesuric effect of acute Digoxin treatment is reminiscent of the effect of the missense mutation in FXYD2, which assumes that FXYD2 is a positive regulator of Na,K-ATPase in the distal convoluted tubule (DCT). The borderline calciuric effect of Digoxin may point to an additional site of action, more proximal to the DCT, that is, the thick ascending limb.Nephron (Source: Nephron)
Source: Nephron - October 30, 2017 Category: Urology & Nephrology Source Type: research

UK Renal Registry 19th Annual Report: Chapter 1 UK RRT Adult Incidence in 2015: National and Centre-specific Analyses
Nephron 2017;137(suppl1):11-44 (Source: Nephron)
Source: Nephron - October 25, 2017 Category: Urology & Nephrology Source Type: research

UK Renal Registry 19th Annual Report: Introduction
Nephron 2017;137(suppl1):1-10 (Source: Nephron)
Source: Nephron - October 25, 2017 Category: Urology & Nephrology Source Type: research