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Clinical Journal of the American Society of Nephrology: CJASN

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https://www.readbyqxmd.com/read/29146704/prospective-evaluation-of-kidney-disease-in-joubert-syndrome
#1
Leah R Fleming, Daniel A Doherty, Melissa A Parisi, Ian A Glass, Joy Bryant, Roxanne Fischer, Baris Turkbey, Peter Choyke, Kailash Daryanani, Meghana Vemulapalli, James C Mullikin, May Christine Malicdan, Thierry Vilboux, John A Sayer, William A Gahl, Meral Gunay-Aygun
BACKGROUND AND OBJECTIVES: Joubert syndrome is a genetically heterogeneous ciliopathy associated with >30 genes. The characteristics of kidney disease and genotype-phenotype correlations have not been evaluated in a large cohort at a single center. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We evaluated 97 individuals with Joubert syndrome at the National Institutes of Health Clinical Center using abdominal ultrasonography, blood and urine chemistries, and DNA sequencing...
November 16, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29146703/a-perspective-on-inherited-kidney-disease-lessons-for-practicing-nephrologists
#2
EDITORIAL
Mathieu Lemaire, Rulan S Parekh
No abstract text is available yet for this article.
November 16, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29146702/prevalence-of-novel-maged2-mutations-in-antenatal-bartter-syndrome
#3
Anne Legrand, Cyrielle Treard, Isabelle Roncelin, Sophie Dreux, Aurélia Bertholet-Thomas, Françoise Broux, Daniele Bruno, Stéphane Decramer, Georges Deschenes, Djamal Djeddi, Vincent Guigonis, Nadine Jay, Tackwa Khalifeh, Brigitte Llanas, Denis Morin, Gilles Morin, François Nobili, Christine Pietrement, Amélie Ryckewaert, Rémi Salomon, Isabelle Vrillon, Anne Blanchard, Rosa Vargas-Poussou
BACKGROUND AND OBJECTIVES: Mutations in the MAGED2 gene, located on the X chromosome, have been recently detected in males with a transient form of antenatal Bartter syndrome or with idiopathic polyhydramnios. The aim of this study is to analyze the proportion of the population with mutations in this gene in a French cohort of patients with antenatal Bartter syndrome. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: The French cohort of patients with antenatal Bartter syndrome encompasses 171 families...
November 16, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29146701/mesangial-c4d-deposits-in-early-iga-nephropathy
#4
Alfons Segarra, Katheryne Romero, Irene Agraz, Natalia Ramos, Alvaro Madrid, Clara Carnicer, Elias Jatem, Ramón Vilalta, Luis Enrique Lara, Elena Ostos, Naiara Valtierra, Juliana Jaramillo, Karla V Arredondo, Gema Ariceta, Cristina Martinez
BACKGROUND AND OBJECTIVES: The prognostic value of mesangial C4d deposits in IgA nephropathy has been analyzed in patients with reduced GFR but has not been analyzed in those with normal kidney function. The main objective of the study was to analyze the prognostic value of C4d deposits and association with response to treatment in patients with IgA nephropathy and normal GFR. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: This retrospective cohort study included 190 patients with idiopathic IgA nephropathy diagnosed by kidney biopsy between 1988 and 2005...
November 16, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29146700/phenotypic-spectrum-of-children-with-nephronophthisis-and-related-ciliopathies
#5
Jens König, Birgitta Kranz, Sabine König, Karl Peter Schlingmann, Andrea Titieni, Burkhard Tönshoff, Sandra Habbig, Lars Pape, Karsten Häffner, Matthias Hansen, Anja Büscher, Martin Bald, Heiko Billing, Raphael Schild, Ulrike Walden, Tobias Hampel, Hagen Staude, Magdalena Riedl, Norbert Gretz, Martin Lablans, Carsten Bergmann, Friedhelm Hildebrandt, Heymut Omran, Martin Konrad
BACKGROUND AND OBJECTIVES: Genetic heterogeneity and phenotypic variability are major challenges in familial nephronophthisis and related ciliopathies. To date, mutations in 20 different genes (NPHP1 to -20) have been identified causing either isolated kidney disease or complex multiorgan disorders. In this study, we provide a comprehensive and detailed characterization of 152 children with a special focus on extrarenal organ involvement and the long-term development of ESRD. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We established an online-based registry (www...
November 16, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29146699/association-between-urine-ammonium-and-urine-tgf-%C3%AE-1-in-ckd
#6
Kalani L Raphael, Sarah Gilligan, Thomas H Hostetter, Tom Greene, Srinivasan Beddhu
BACKGROUND AND OBJECTIVES: Urinary ammonium excretion increases in response to nonvolatile acids to maintain normal systemic bicarbonate and pH. However, enhanced ammonia production promotes tubulointerstitial fibrosis in animal models. Therefore, a subset of individuals with CKD and normal bicarbonate may have acid-mediated kidney fibrosis that might be better linked with ammonium excretion than bicarbonate. We hypothesized that urine TGF-β1, as an indicator of kidney fibrosis, would be more tightly linked with urine ammonium excretion than serum bicarbonate and other acid-base indicators...
November 16, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29127259/whole-exome-sequencing-of-patients-with-steroid-resistant-nephrotic-syndrome
#7
Jillian K Warejko, Weizhen Tan, Ankana Daga, David Schapiro, Jennifer A Lawson, Shirlee Shril, Svjetlana Lovric, Shazia Ashraf, Jia Rao, Tobias Hermle, Tilman Jobst-Schwan, Eugen Widmeier, Amar J Majmundar, Ronen Schneider, Heon Yung Gee, J Magdalena Schmidt, Asaf Vivante, Amelie T van der Ven, Hadas Ityel, Jing Chen, Carolin E Sadowski, Stefan Kohl, Werner L Pabst, Makiko Nakayama, Michael J G Somers, Nancy M Rodig, Ghaleb Daouk, Michelle Baum, Deborah R Stein, Michael A Ferguson, Avram Z Traum, Neveen A Soliman, Jameela A Kari, Sherif El Desoky, Hanan Fathy, Martin Zenker, Sevcan A Bakkaloglu, Dominik Müller, Aytul Noyan, Fatih Ozaltin, Melissa A Cadnapaphornchai, Seema Hashmi, Jeffrey Hopcian, Jeffrey B Kopp, Nadine Benador, Detlef Bockenhauer, Radovan Bogdanovic, Nataša Stajić, Gil Chernin, Robert Ettenger, Henry Fehrenbach, Markus Kemper, Reyner Loza Munarriz, Ludmila Podracka, Rainer Büscher, Erkin Serdaroglu, Velibor Tasic, Shrikant Mane, Richard P Lifton, Daniela A Braun, Friedhelm Hildebrandt
BACKGROUND AND OBJECTIVES: Steroid-resistant nephrotic syndrome overwhelmingly progresses to ESRD. More than 30 monogenic genes have been identified to cause steroid-resistant nephrotic syndrome. We previously detected causative mutations using targeted panel sequencing in 30% of patients with steroid-resistant nephrotic syndrome. Panel sequencing has a number of limitations when compared with whole exome sequencing. We employed whole exome sequencing to detect monogenic causes of steroid-resistant nephrotic syndrome in an international cohort of 300 families...
November 10, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29122911/prognosis-of-patients-with-cirrhosis-and-aki-who-initiate-rrt
#8
Andrew S Allegretti, Xavier Vela Parada, Nwamaka D Eneanya, Hannah Gilligan, Dihua Xu, Sophia Zhao, Jules L Dienstag, Raymond T Chung, Ravi I Thadhani
BACKGROUND AND OBJECTIVES: Literature on the prognosis of patients with cirrhosis who require RRT for AKI is sparse and is confounded by liver transplant eligibility. An update on outcomes in the nonlisted subgroup is needed. Our objective was to compare outcomes in this group between those diagnosed with hepatorenal syndrome and acute tubular necrosis, stratifying by liver transplant listing status. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Retrospective cohort study of patients with cirrhosis acutely initiated on hemodialysis or continuous RRT at five hospitals, including one liver transplant center...
November 9, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29114007/effective-treatment-of-pd-peritonitis
#9
EDITORIAL
Beth Piraino
No abstract text is available yet for this article.
November 7, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29114006/hyperkalemia-across-the-continuum-of-kidney-function
#10
Biff F Palmer, Deborah J Clegg
No abstract text is available yet for this article.
November 7, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29114005/residual-kidney-function-and-peritoneal-dialysis-associated-peritonitis-treatment-outcomes
#11
Rachel Whitty, Joanne M Bargman, Alex Kiss, Linda Dresser, Philip Lui
BACKGROUND AND OBJECTIVES: Residual kidney function contributes to the clearance of antibiotics excreted by the kidneys, lowering the antibiotic concentration, which may adversely affect the treatment of peritoneal dialysis-associated peritonitis. The objective of our study was to examine the relationship between residual kidneyfunction and peritonitis treatment outcomes. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Our study included 181 participants who experienced 339 episodes of Gram-positive, Gram-negative, and culture-negative peritoneal dialysis-associated peritonitis at a single centerfrom 2003 to 2010...
November 7, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29114004/dysproteinemias-and-glomerular-disease
#12
Nelson Leung, Maria E Drosou, Samih H Nasr
Dysproteinemia is characterized by the overproduction of an Ig by clonal expansion of cells from the B cell lineage. The resultant monoclonal protein can be composed of the entire Ig or its components. Monoclonal proteins are increasingly recognized as a contributor to kidney disease. They can cause injury in all areas of the kidney, including the glomerular, tubular, and vascular compartments. In the glomerulus, the major mechanism of injury is deposition. Examples of this include Ig amyloidosis, monoclonal Ig deposition disease, immunotactoid glomerulopathy, and cryoglobulinemic GN specifically from types 1 and 2 cryoglobulins...
November 7, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29102960/new-frontiers-in-treating-uremic-metabolic-acidosis
#13
EDITORIAL
Denver Brown, Michal L Melamed
No abstract text is available yet for this article.
November 4, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29102959/randomized-controlled-trial-of-trc101-to-increase-serum-bicarbonate-in-patients-with-ckd
#14
David A Bushinsky, Thomas Hostetter, Gerrit Klaerner, Yuri Stasiv, Claire Lockey, Sarah McNulty, Angela Lee, Dawn Parsell, Vandana Mathur, Elizabeth Li, Jerry Buysse, Robert Alpern
BACKGROUND AND OBJECTIVES: Metabolic acidosis is common in patients with CKD and has significant adverse effects on kidney, muscle, and bone. We tested the efficacy and safety of TRC101, a novel, sodium-free, nonabsorbed hydrochloric acid binder, to increase serum bicarbonate in patients with CKD and metabolic acidosis. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: One hundred thirty-five patients were enrolled in this randomized, double-blind, placebo-controlled, multicenter, in-unit study (designated the TRCA-101 Study)...
November 4, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29101187/consequences-of-overinterpreting-serum-creatinine-increases-when-achieving-bp-reduction-balancing-risks-and-benefits-of-bp-reduction-in-hypertension
#15
EDITORIAL
Hala Yamout, George L Bakris
No abstract text is available yet for this article.
November 3, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29101186/bp-reduction-kidney-function-decline-and-cardiovascular-events-in-patients-without-ckd
#16
Rita Magriço, Miguel Bigotte Vieira, Catarina Viegas Dias, Lia Leitão, João Sérgio Neves
BACKGROUND AND OBJECTIVES: In the Systolic Blood Pressure Intervention Trial (SPRINT), intensive systolic BP treatment (target <120 mm Hg) was associated with fewer cardiovascular events and higher incidence of kidney function decline compared with standard treatment (target <140 mm Hg). We evaluated the association between mean arterial pressure reduction, kidney function decline, and cardiovascular events in patients without CKD. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We categorized patients in the intensive treatment group of the SPRINT according to mean arterial pressure reduction throughout follow-up: <20, 20 to <40, and ≥40 mm Hg...
November 3, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29097482/urine-anion-gap-to-predict-urine-ammonium-and-related-outcomes-in-kidney-disease
#17
Kalani L Raphael, Sarah Gilligan, Joachim H Ix
BACKGROUND AND OBJECTIVES: Low urine ammonium excretion is associated with ESRD in CKD. Few laboratories measure urine ammonium, limiting clinical application. We determined correlations between urine ammonium, the standard urine anion gap, and a modified urine anion gap that includes sulfate and phosphate and compared risks of ESRD or death between these ammonium estimates and directly measured ammonium. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We measured ammonium, sodium, potassium, chloride, phosphate, and sulfate from baseline 24-hour urine collections in 1044 African-American Study of Kidney Disease and Hypertension participants...
November 2, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29097481/efficacy-and-long-term-safety-of-c-e-r-a-maintenance-in-pediatric-hemodialysis-patients-with-anemia-of-ckd
#18
Michel Fischbach, Elke Wühl, Sylvie C Meyer Reigner, Zoe Morgan, Franz Schaefer
BACKGROUND AND OBJECTIVES: The study was conducted to identify a conversion factor for switching from previous erythropoiesis-stimulating agents (ESAs) to continuous erythropoietin receptor activator-methoxy polyethylene glycol-epoetin beta (C.E.R.A.) and to document the efficacy and long-term safety of C.E.R.A. in pediatric patients with anemia of CKD undergoing hemodialysis. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: In this open-label, multicenter study, patients aged 6-17 years, with stable chronic anemia of CKD, undergoing hemodialysis received C...
November 2, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29092892/coaching-nephrology-trainees-who-struggle-with-clinical-performance
#19
Karen M Warburton, John D Mahan
No abstract text is available yet for this article.
November 1, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29084721/the-journey-to-full-health-care-responsibility-for-one-esco-provider
#20
Franklin W Maddux, Terry L Ketchersid
No abstract text is available yet for this article.
October 30, 2017: Clinical Journal of the American Society of Nephrology: CJASN
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