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Pediatric Nephrology: Journal of the International Pediatric Nephrology Association

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https://www.readbyqxmd.com/read/30109447/effect-of-different-rituximab-regimens-on-b-cell-depletion-and-time-to-relapse-in-children-with-steroid-dependent-nephrotic-syndrome
#1
Julien Hogan, Claire Dossier, Thérésa Kwon, Marie-Alice Macher, Anne Maisin, Anne Couderc, Olivier Niel, Véronique Baudouin, Georges Deschênes
BACKGROUND: Several studies have demonstrated that rituximab (RTX) improves relapse-free survival in patients with steroid-dependent nephrotic syndrome (SDNS). However, these studies used various RTX regimens and there are few data comparing these regimens in children with SDNS. In this retrospective study, we assessed the effect of three different initial RTX regimens on both time to B cell reconstitution and to first relapse. METHODS: Sixty-one SDNS patients receiving a first course of RTX were included...
August 14, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/30109446/writing-a-paper-for-publication
#2
EDITORIAL
Lesley Rees, Michel Baum
No abstract text is available yet for this article.
August 14, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/30109445/extra-renal-manifestations-of-atypical-hemolytic-uremic-syndrome
#3
Cassandra Formeck, Agnieszka Swiatecka-Urban
Atypical hemolytic uremic syndrome (aHUS) is a rare and complex disease resulting from abnormal alternative complement activation with a wide range of clinical presentations. Extra-renal manifestations of aHUS can involve many organ systems, including the peripheral and central nervous, gastrointestinal, cardiovascular, integumentary, pulmonary, as well as the eye. While some of these extra-renal manifestations occur in the acute phase of aHUS, some can also occur as long-term sequelae of unopposed complement activation...
August 14, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/30105415/time-averaged-hemoglobin-values-not-hemoglobin-cycling-have-an-impact-on-outcomes-in-pediatric-dialysis-patients
#4
Sevcan A Bakkaloğlu, Yaşar Kandur, Erkin Serdaroğlu, Aytül Noyan, Aysun Karabay Bayazıt, Mehmet Taşdemir, Sare Gülfem Özlü, Gül Özçelik, İsmail Dursun, Caner Alparslan, Meltem Akcaboy, Yeşim Özdemir Atikel, Gönül Parmaksız, Bahriye Atmış, Lale Sever
BACKGROUND: During erythropoietin-stimulating agent (ESA) treatment, hemoglobin (Hb) levels usually fluctuate; this phenomenon is known as "Hb cycling (HC)." In this study, we aimed to evaluate the predictors of HC and its impact on left ventricular hypertrophy (LVH) as a patient-important outcome parameter in pediatric dialysis patients. METHODS: Records of patients followed up in nine pediatric nephrology centers between 2008 and 2013 were reviewed. More than 1 g/dL decrease or increase in Hb level was considered as HC...
August 13, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/30105414/oxidative-stress-in-chronic-kidney-disease
#5
REVIEW
Kristien Daenen, Asmin Andries, Djalila Mekahli, Ann Van Schepdael, François Jouret, Bert Bammens
Oxidative stress (OS), defined as disturbances in the pro-/antioxidant balance, is harmful to cells due to the excessive generation of highly reactive oxygen (ROS) and nitrogen (RNS) species. When the balance is not disturbed, OS has a role in physiological adaptations and signal transduction. However, an excessive amount of ROS and RNS results in the oxidation of biological molecules such as lipids, proteins, and DNA. Oxidative stress has been reported in kidney disease, due to both antioxidant depletions as well as increased ROS production...
August 13, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/30105413/oxidative-stress-in-autosomal-dominant-polycystic-kidney-disease-player-and-or-early-predictor-for-disease-progression
#6
Asmin Andries, Kristien Daenen, François Jouret, Bert Bammens, Djalila Mekahli, Ann Van Schepdael
Autosomal dominant polycystic kidney disease (ADPKD), caused by mutations in PKD1 or PKD2 genes, is the most common hereditary renal disease. Renal manifestations of ADPKD are gradual cyst development and kidney enlargement ultimately leading to end-stage renal disease. ADPKD also causes extrarenal manifestations, including endothelial dysfunction and hypertension. Both of these complications are linked with reduced nitric oxide levels related to excessive oxidative stress (OS). OS, defined as disturbances in the prooxidant/antioxidant balance, is harmful to cells due to the excessive generation of highly reactive oxygen and nitrogen free radicals...
August 13, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/30099615/endoplasmic-reticulum-stress-and-monogenic-kidney-diseases-in-precision-nephrology
#7
REVIEW
Sun-Ji Park, Yeawon Kim, Ying Maggie Chen
The advent of next-generation sequencing (NGS) in recent years has led to a rapid discovery of novel or rare genetic variants in human kidney cell genes, which is transforming the risk assessment, diagnosis, and treatment of kidney disease. Mutations may lead to protein misfolding, disruption of protein trafficking, and endoplasmic reticulum (ER) retention. An imbalance between the load of misfolded proteins and the folding capacity of the ER causes ER stress and unfolded protein response. Mutations in nephrin (NPHS1), podocin (NPHS2), laminin β2 (LAMB2), and α-actinin-4 (ACTN4) have been shown to induce ER stress in HEK293 cells and podocytes in hereditary nephrotic syndromes; various founder mutations in collagen IV α chains (COL4A) have been demonstrated to activate podocyte ER stress in collagen IV nephropathies; and mutations in uromodulin (UMOD) have been reported to trigger tubular ER stress in autosomal dominant tubulointerstitial kidney disease...
August 11, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/30091061/effect-of-atorvastatin-on-dyslipidemia-and-carotid-intima-media-thickness-in-children-with-refractory-nephrotic-syndrome-a-randomized-controlled-trial
#8
Pankaj Hari, Priyanka Khandelwal, Amit Satpathy, Smriti Hari, Ranjeet Thergaonkar, R Lakshmy, Aditi Sinha, Arvind Bagga
BACKGROUND: Dyslipidemia is an important cardiovascular risk factor in steroid-resistant nephrotic syndrome (SRNS). Efficacy of statins for treatment of hyperlipidemia in children with SRNS is unclear. METHODS: This prospective, randomized, double-blind, placebo-controlled, parallel-group clinical trial enrolled 30 patients with SRNS, aged 5-18 years, with serum low-density lipoprotein cholesterol (LDL-C) levels between 130 and 300 mg/dl, to receive a fixed dose of atorvastatin (n = 15, 10 mg/d) or placebo (n = 15) by block randomization in a 1:1 ratio...
August 8, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/30058048/drug-metabolizing-enzymes-cyp3a-as-a-link-between-tacrolimus-and-vitamin-d-in-renal-transplant-recipients-is-it-relevant-in-clinical-practice
#9
Agnieszka Prytuła, Karlien Cransberg, Ann Raes
CYP3A enzymes are involved in the metabolism of calcineurin inhibitor tacrolimus as well as vitamin D. In this review, we summarize the clinical aspects of CYP3A-mediated metabolism of tacrolimus and vitamin D with emphasis on the influence of single-nucleotide polymorphisms on tacrolimus disposition. We describe the utility of 4β hydroxycholesterol as a marker of CYP3A activity. Then, we discuss the possible interaction between calcineurin inhibitors and vitamin D in solid organ transplant recipients. Also, we review other mechanisms which may contribute to side effects of calcineurin inhibitors on bone...
July 30, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/30058047/jc-polyomavirus-replication-and-associated-disease-in-pediatric-renal-transplantation-an-international-certain-registry-study
#10
Britta Höcker, Julia Tabatabai, Lukas Schneble, Jun Oh, Florian Thiel, Lars Pape, Krisztina Rusai, Rezan Topaloglu, Birgitta Kranz, Günter Klaus, Nikoleta Printza, Onder Yavascan, Alexander Fichtner, Kai Krupka, Thomas Bruckner, Rüdiger Waldherr, Michael Pawlita, Paul Schnitzler, Hans H Hirsch, Burkhard Tönshoff
BACKGROUND: JC polyomavirus (JCPyV)-associated nephropathy (JCPyVAN) is a severe, but rare complication in adult renal transplant (RTx) recipients. Related data in pediatric patients are scarce. METHODS: Based on the CERTAIN Registry, we therefore performed a multi-center, retrospective study on the JCPyV antibody status, prevalence of JCPyV replication, and its associated disease in 139 pediatric RTx recipients (mean age, 8.5 ± 5.3 years). JCPyV DNA in plasma and/or urine was measured by quantitative PCR at a median time of 3...
July 30, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/30058046/eculizumab-in-the-treatment-of-shiga-toxin-haemolytic-uraemic-syndrome
#11
REVIEW
Patrick R Walsh, Sally Johnson
Haemolytic uraemic syndrome (HUS) remains a leading cause of paediatric acute kidney injury (AKI). Haemolytic uraemic syndrome is characterised by the triad of microangiopathic haemolytic anaemia, thrombocytopenia and AKI. In ~ 90% of cases, HUS is a consequence of infection with Shiga toxin-producing E. coli (STEC), most commonly serotype O157:H7. Acute mortality from STEC-HUS is now less than 5%; however, there is significant long-term renal morbidity in one third of survivors. Currently, no specific treatment exists for STEC-HUS...
July 30, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/30054737/a-child-with-arthritis-skin-rash-abdominal-pain-and-nephritis-searching-beyond-henoch-sch%C3%A3-nlein-purpura-answers
#12
William Chotas, Mohammad Ilyas, Asad Tolaymat
No abstract text is available yet for this article.
July 27, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/30054736/a-child-with-arthritis-skin-rash-abdominal-pain-and-nephritis-searching-beyond-henoch-sch%C3%A3-nlein-purpura-questions
#13
William Chotas, Mohammad Ilyas, Asad Tolaymat
No abstract text is available yet for this article.
July 27, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/30043116/does-idiopathic-hypercalciuria-affect-bone-metabolism-during-childhood-a-prospective-case-control-study
#14
Maria Pavlou, Vasileios Giapros, Anna Challa, Nikolaos Chaliasos, Ekaterini Siomou
BACKGROUND: A limited number of studies have evaluated biochemical bone metabolism markers in children with idiopathic hypercalciuria, which in adults has been linked with osteopenia. Our aim was to investigate in children with idiopathic hypercalciuria biochemical markers of bone formation and resorption and the osteoprotegerin (OPG) and soluble receptor activator of nuclear factor kB ligand (sRANKL) system which is involved in the osteoclastogenesis process. METHODS: A prospective study was conducted on 50 children with idiopathic hypercalciuria and 50 healthy age-, sex-, and Tanner stage-matched control subjects...
July 25, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/30039433/management-of-chronic-renal-allograft-dysfunction-and-when-to-re-transplant
#15
Richard J Baker, Stephen D Marks
Despite the advances in renal transplantation over the last decades, chronic allograft dysfunction remains the largest concern for patients, their families, clinicians and other members of the multi-disciplinary team. Although we have made progress in improving patient and renal allograft survival within the first year after transplantation, the rate of transplant failure with requirement for commencement of dialysis or re-transplantation has essentially remained unchanged. It is important that paediatric and adult nephrologists and transplant surgeons, not only manage their patients and their renal transplants but provide the best chronic kidney disease management during the time of decline of renal allograft function...
July 23, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/30032326/blood-borne-viral-infections-in-pediatric-hemodialysis
#16
Shina Menon, Raj Munshi
Hemodialysis patients are at increased risk for development of blood-borne viral infections. Human immunodeficiency virus (HIV), a once fatal infection, has become treatable, but continues to be associated with increased mortality. Hepatitis B and C viral infections can lead to acute and chronic hepatitis, cirrhosis, or hepatocellular carcinoma. Young children and immunocompromised patients are more likely to develop chronic disease leading to increased morbidity and mortality, as compared to the healthy population...
July 21, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/30030607/response-to-erythropoietin-in-pediatric-patients-with-chronic-kidney-disease-insights-from-an-in-vitro-bioassay
#17
Rachel Gavish, Salmas Watad, Nathalie Ben-Califa, Ori Jacob Goldberg, Orly Haskin, Miriam Davidovits, Gili Koren, Yafa Falush, Drorit Neumann, Irit Krause
BACKGROUND: Decreased production of erythropoietin (EPO) is a major cause of anemia associated with chronic kidney disease (CKD). Treatment with recombinant human EPO (rHuEPO) improves patients' quality of life and survival; however, there is a marked variability in response to rHuEPO. At present, no available laboratory test is capable of evaluating responsiveness to EPO treatment. The aim of the present study was to use an in vitro bioassay to estimate the effect of uremic environment on EPO-dependent erythroid cell proliferation...
July 20, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/30027358/an-infant-with-hypercalcemia-and-hyperammonia-inborn-error-of-metabolism-or-not-answers
#18
Arife Uslu Gökceoğlu, Medine Ayşin Taşar, Zahide Yalaki, Abdullah Güneş, Abdullatif Bakır
No abstract text is available yet for this article.
July 19, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/30027357/an-infant-with-hypercalcemia-and-hyperammonia-inborn-error-of-metabolism-or-not-questions
#19
Arife Uslu Gökceoğlu, Medine Ayşin Taşar, Zahide Yalaki, Abdullah Güneş, Abdullatif Bakır
No abstract text is available yet for this article.
July 19, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/30022312/pre-operative-level-of-fgf23-predicts-severe-acute-kidney-injury-after-heart-surgery-in-children
#20
Oded Volovelsky, Tara C Terrell, Hayley Swain, Michael R Bennett, David S Cooper, Stuart L Goldstein
BACKGROUND: Early detection of acute kidney injury (AKI) after cardiac surgery has improved recently with the discovery and validation of novel urinary biomarkers. However, objective tools to predict the risk of AKI before the insult are still missing. We tested the hypothesis that pre-operative serum fibroblast growth factor 23 (FGF23) concentrations would be elevated in children who develop AKI after heart surgery with cardiopulmonary bypass (CPB). We also compared post-operative FGF23 concentrations to other biomarkers for early detection of AKI...
July 19, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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