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Pediatric Nephrology: Journal of the International Pediatric Nephrology Association

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https://www.readbyqxmd.com/read/29243158/bilateral-native-nephrectomy-to-reduce-oxalate-stores-in-children-at-the-time-of-combined-liver-kidney-transplantation-for-primary-hyperoxaluria-type-1
#1
Eliza Lee, Gabriel Ramos-Gonzalez, Nancy Rodig, Scott Elisofon, Khashayar Vakili, Heung Bae Kim
OBJECTIVE: Primary hyperoxaluria type-1 (PH-1) is a rare genetic disorder in which normal hepatic metabolism of glyoxylate is disrupted resulting in diffuse oxalate deposition and end-stage renal disease (ESRD). While most centers agree that combined liver-kidney transplant (CLKT) is the appropriate treatment for PH-1, perioperative strategies for minimizing recurrent oxalate-related injury to the transplanted kidney remain unclear. We present our management of children with PH-1 and ESRD on hemodialysis (HD) who underwent CLKT at our institution from 2005 to 2015...
December 14, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29218437/d-lactic-acidosis-in-humans-systematic-literature-review
#2
Davide G A M Bianchetti, Giacomo S Amelio, Sebastiano A G Lava, Mario G Bianchetti, Giacomo D Simonetti, Carlo Agostoni, Emilio F Fossali, Gregorio P Milani
BACKGROUND: D-lactic acidosis is an uncommon and challenging form of metabolic acidosis that may develop in short bowel syndrome. It has been documented exclusively in case reports and small case series. METHODS: We performed a review of the literature in the National Library of Medicine and Excerpta Medica databases. RESULTS: We identified 84 original reports published between 1977 and 2017. D-lactic acidosis was observed in 98 individuals ranging in age from 7 months to 86 years with short bowel syndrome...
December 7, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29218436/peritoneal-dialysis-and-infants-further-insights-into-a-complicated-relationship
#3
EDITORIAL
Enrico Vidal
Peritoneal dialysis (PD) in infants represents one of the greatest challenges for pediatric nephrologists. Over recent years, positive outcome data described by several multicenter experiences and registry studies have increased the amount of information available to help determine whether to initiate a dialysis program in this high-risk patient population. There is no doubt that the rigorous implementation of strategies aimed at preventing infectious complications may have contributed to reducing the morbidity rate of these patients...
December 7, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29218435/educational-review-measurement-of-gfr-in-special-populations
#4
Guido Filler, Misan Lee
IMPORTANCE: Changes in kidney function are typically followed by the sequential estimation of glomerular filtration rate (eGFR). Formulae for eGFR work well on a population basis, but there are well-known conditions where they do not work. OBJECTIVE: The purpose of this review is to summarize the existing literature on special populations in the pediatric age range and provide recommendations on how to estimate GFR in these populations. FINDINGS: The reliability of creatinine depends on muscle mass, while cystatin C (not widely available) is limited by inflammation and changes in protein catabolism...
December 7, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29214443/contribution-of-symmetric-dimethylarginine-to-gfr-decline-in-pediatric-chronic-kidney-disease
#5
Ellen R Brooks, Shannon Haymond, Alfred Rademaker, Christopher Pierce, Irene Helenowski, Rod Passman, Faye Vicente, Bradley A Warady, Susan L Furth, Craig B Langman
BACKGROUND: In pediatric chronic kidney disease (pCKD), traditional factors (proteinuria, etiology, and race) do not fully explain disease progression. The levels of methylated arginine derivatives (MADs: asymmetric and symmetric dimethylarginine, respectively) rise in CKD and increase with CKD progression. The impact of MADs on glomerular filtration rate (GFR) decline has not been examined in pCKD. The aim of this study was to examine the additive impact of baseline (BL) levels of MADs on directly measured GFR (mGFR) decline per year (ml/min/1...
December 7, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29214442/shiga-toxin-triggers-endothelial-and-podocyte-injury-the-role-of-complement-activation
#6
REVIEW
Carlamaria Zoja, Simona Buelli, Marina Morigi
Shiga toxin (Stx)-producing Escherichia coli (STEC) is the offending agent in post-diarrhea-associated hemolytic uremic syndrome (HUS), a disorder characterized by thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney failure, with thrombi occluding the renal microvasculature. Endothelial dysfunction has been recognized as the trigger event in the development of microangiopathic processes. Glomerular endothelial cells are susceptible to the toxic effects of Stxs that, via nuclear factor kappa B (NF-κB) activation, induce the expression of genes encoding for adhesion molecules and chemokines, culminating in leukocyte adhesion and platelet thrombus formation on the activated endothelium...
December 6, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29209823/autologous-arteriovenous-fistulas-for-hemodialysis-using-microsurgery-techniques-in-children-weighing-less-than-20%C3%A2-kg
#7
Vasiliki Karava, Pascal Jehanno, Theresa Kwon, Georges Deschênes, Marie-Alice Macher, Pierre Bourquelot
BACKGROUND: This study aimed to describe the efficiency and longevity of arteriovenous fistula (AVF) for hemodialysis (HD) in children weighing ≤20 kg. METHODS: We collected data of all AVFs created using microsurgery techniques between 1988 and 2015. Success was considered as the ability to use the AVF for HD. Primary and secondary patency rates were measured. RESULTS: Forty-eight AVFs (35 forearm, 13 upper arm) were created in 41 children with a median weight of 13...
December 5, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29197999/remote-ischemic-preconditioning-upregulates-microrna-21-to-protect-the-kidney-in-children-with-congenital-heart-disease-undergoing-cardiopulmonary-bypass
#8
Zhijuan Kang, Zhihui Li, Peng Huang, Jinwen Luo, Pingbo Liu, Ying Wang, Tuanhong Xia, Yuhang Zhou
BACKGROUND: Acute kidney injury (AKI) is one of the most common emergencies and severe diseases in the clinic. We sought to verify whether remote ischemic preconditioning (RIPC) has a protective effect on the kidney of child with congenital heart disease undergoing cardiopulmonary bypass (CPB) surgery. We hypothesized it may be related to the up-regulation of microRNA-21 (miR-21). METHODS: We performed a prospective randomized clinical study among children with congenital heart disease undergoing CPB surgery between January and December 2016...
December 2, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29196979/changes-in-arterial-pressure-and-markers-of-nitric-oxide-homeostasis-and-oxidative-stress-following-surgical-correction-of-hydronephrosis-in-children
#9
Ammar Al-Mashhadi, Antonio Checa, Nils Wåhlin, Tryggve Neveus, Magdalena Fossum, Craig E Wheelock, Birgitta Karanikas, Arne Stenberg, A Erik G Persson, Mattias Carlstrom
OBJECTIVE: Recent clinical studies have suggested an increased risk of elevated arterial pressure in patients with hydronephrosis. Animals with experimentally induced hydronephrosis develop hypertension, which is correlated to the degree of obstruction and increased oxidative stress. In this prospective study we investigated changes in arterial pressure, oxidative stress, and nitric oxide (NO) homeostasis following correction of hydronephrosis. METHODS: Ambulatory arterial pressure (24 h) was monitored in pediatric patients with hydronephrosis (n = 15) before and after surgical correction, and the measurements were compared with arterial pressure measurements in two control groups, i...
December 1, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29188361/disruptive-technological-advances-in-vascular-access-for-dialysis-an-overview
#10
REVIEW
Wee-Song Yeo, Qin Xiang Ng
End-stage kidney disease (ESKD), one of the most prevalent diseases in the world and with increasing incidence, is associated with significant morbidity and mortality. Current available modes of renal replacement therapy (RRT) include dialysis and renal transplantation. Though renal transplantation is the preferred and ideal mode of RRT, this modality may not be available to all patients with ESKD. Moreover, renal transplant recipients are constantly at risk of complications associated with immunosuppression and immunosuppressant use, and posttransplant lymphoproliferative disorder...
November 29, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29181713/genetic-testing-in-steroid-resistant-nephrotic-syndrome-why-who-when-and-how
#11
REVIEW
Rebecca Preston, Helen M Stuart, Rachel Lennon
Steroid-resistant nephrotic syndrome (SRNS) is a common cause of chronic kidney disease in childhood and has a significant risk of rapid progression to end-stage renal disease. The identification of over 50 monogenic causes of SRNS has revealed dysfunction in podocyte-associated proteins in the pathogenesis of proteinuria, highlighting their essential role in glomerular function. Recent technological advances in high-throughput sequencing have enabled indication-driven genetic panel testing for patients with SRNS...
November 27, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29181712/exosomes-and-microvesicles-in-normal-physiology-pathophysiology-and-renal-diseases
#12
REVIEW
Anne-Lie Ståhl, Karl Johansson, Maria Mossberg, Robin Kahn, Diana Karpman
Extracellular vesicles are cell-derived membrane particles ranging from 30 to 5,000 nm in size, including exosomes, microvesicles, and apoptotic bodies. They are released under physiological conditions, but also upon cellular activation, senescence, and apoptosis. They play an important role in intercellular communication. Their release may also maintain cellular integrity by ridding the cell of damaging substances. This review describes the biogenesis, uptake, and detection of extracellular vesicles in addition to the impact that they have on recipient cells, focusing on mechanisms important in the pathophysiology of kidney diseases, such as thrombosis, angiogenesis, tissue regeneration, immune modulation, and inflammation...
November 27, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29177628/mycophenolate-mofetil-following-glucocorticoid-treatment-in-henoch-sch%C3%A3-nlein-purpura-nephritis-the-role-of-early-initiation-and-therapeutic-drug-monitoring
#13
Agnes Hackl, Jan U Becker, Lisa M Körner, Rasmus Ehren, Sandra Habbig, Eva Nüsken, Kai-Dietrich Nüsken, Kathrin Ebner, Max C Liebau, Carsten Müller, Martin Pohl, Lutz T Weber
BACKGROUND: Henoch-Schönlein purpura (HSP) is the most common vasculitis in childhood and traditionally considered as a self-limiting disease. However, renal involvement can unfavorably determine long-term prognosis. The reported regimens to treat HSP nephritis (HSPN) are diverse, indicating that the most effective treatment remains controversial. METHODS: This retrospective, single-center study involved 18 patients presenting with HSPN and nephrotic-range proteinuria...
November 25, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29159474/two-rare-cases-of-abdominal-tumor-in-children-answers
#14
Haojie Tang, Mengtian Lu, Shan Jiang, Ying Ren
No abstract text is available yet for this article.
November 20, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29159473/two-rare-cases-of-abdominal-tumor-in-children-questions
#15
Haojie Tang, Mengtian Lu, Shan Jiang, Ying Ren
No abstract text is available yet for this article.
November 20, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29159472/current-strategies-to-predict-and-manage-sequelae-of-posterior-urethral-valves-in-children
#16
Aniruddh V Deshpande
Posterior urethral valves (PUV) constitute a significant urological cause of chronic kidney disease (CKD) in children. The condition is characterised by the unique pathophysiology of the evolution of bladder dysfunction after relief of obstruction, which contributes to CKD. Improvements in prenatal diagnosis followed by selective foetal intervention have not yet produced improvement in long-term renal outcomes, although better patient selection may alter this in the future. Proactive management with surveillance, pharmacotherapy, timed voiding, double voiding, and/or assisted bladder-emptying, is being increasingly offered to those with severe bladder dysfunction and has the potential of reducing the burden of renal disease...
November 20, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29150712/unacylated-ghrelin-and-obestatin-promising-biomarkers-of-protein-energy-wasting-in-children-with-chronic-kidney-disease
#17
Alice Monzani, Michela Perrone, Flavia Prodam, Stefania Moia, Giulia Genoni, Sara Testa, Fabio Paglialonga, Anna Rapa, Gianni Bona, Giovanni Montini, Alberto Edefonti
BACKGROUND: Impairment in orexigenic/anorexigenic hormone balance may be key in the pathogenesis of protein energy wasting in children with chronic kidney disease (CKD). Measurement of ghrelin and obestatin concentrations in children with CKD would help assess the potential contribution of these hormones to uremic protein energy wasting. METHODS: This was a cross-sectional case-control study. Acylated and unacylated ghrelin and obestatin were measured in 42 children on conservative treatment (CT), 20 children on hemodialysis, 48 pediatric renal transplant (RTx) recipients and 43 controls (CTR) (mean age 11...
November 18, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29150711/epidemiology-of-peritonitis-following-maintenance-peritoneal-dialysis-catheter-placement-during-infancy-a-report-of-the-scope-collaborative
#18
Joshua Jacob Zaritsky, Coral Hanevold, Raymond Quigley, Troy Richardson, Cynthia Wong, Jennifer Ehrlich, John Lawlor, Jonathan Rodean, Alicia Neu, Bradley A Warady
BACKGROUND: Maintenance peritoneal dialysis (PD) is the dialysis modality of choice for infants and young children. However, there are limited outcome data for those who undergo PD catheter insertion and initiate maintenance PD within the first year of life. METHODS: Using data from the Children's Hospital Association's Standardizing Care to Improve Outcomes in Pediatric End Stage Renal Disease (ESRD) Collaborative (SCOPE), we examined peritonitis rates and patient survival in 156 infants from 29 North American pediatric dialysis centers who had a chronic PD catheter placed prior to their first birthday...
November 17, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29147862/eosinophilia-in-a-peritoneal-dialysis-patient-answers
#19
Shimrit Tzvi-Behr, Yaacov Frishberg, Efrat Ben-Shalom, Choni Rinat, Rachel Becker-Cohen
Icodextrin is a starch-derived glucose polymer used in peritoneal dialysis dialysate to treat volume overload by increasing ultrafiltration in patients with end-stage renal disease. Reported adverse reactions to icodextrin are mild and rare and mainly consist of skin rash that resolves spontaneously after discontinuation of treatment. We describe a young patient with extreme eosinophilia that appeared with the use of icodextrin, disappeared after its discontinuation, and reappeared after a rechallenge with the drug...
November 16, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29147861/eosinophilia-in-a-peritoneal-dialysis-patient-questions
#20
Shimrit Tzvi-Behr, Yaacov Frishberg, Efrat Ben-Shalom, Choni Rinat, Rachel Becker-Cohen
No abstract text is available yet for this article.
November 16, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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