Nephrology Commons^™

Consensus Guidelines For Management Of Hyperammonaemia In Paediatric Patients Receiving Continuous Kidney Replacement Therapy., Rupesh Raina, Jirair K. Bedoyan, Uta Lichter-Konecki, Philippe Jouvet, Stefano Picca, Nicholas Ah Mew, Marcel C. Machado, Ronith Chakraborty, Meghana Vemuganti, Manpreet K. Grewal, Timothy Bunchman, Sidharth Kumar Sethi, Vinod Krishnappa, Mignon Mcculloch, Khalid Alhasan, Arvind Bagga, Rajit K. Basu, Franz Schaefer, Guido Filler, Bradley A. Warady

Manuscripts, Articles, Book Chapters and Other Papers

Hyperammonaemia in children can lead to grave consequences in the form of cerebral oedema, severe neurological impairment and even death. In infants and children, common causes of hyperammonaemia include urea cycle disorders or organic acidaemias. Few studies have assessed the role of extracorporeal therapies in the management of hyperammonaemia in neonates and children. Moreover, consensus guidelines are lacking for the use of non-kidney replacement therapy (NKRT) and kidney replacement therapies (KRTs, including peritoneal dialysis, continuous KRT, haemodialysis and hybrid therapy) to manage hyperammonaemia in neonates and children. Prompt treatment with KRT and/or NKRT, the choice of which depends on the …

Association Between Chronic Kidney Disease-Mineral Bone Disease (Ckd-Mbd) And Cognition In Children: Chronic Kidney Disease In Children (Ckid) Study., Jennifer S. Yokoyama, Mina Matsuda-Abedini, Michelle R. Denburg, Juhi Kumar, Bradley A. Warady, Susan L. Furth, Stephen R. Hooper, Anthony A. Portale, Farzana Perwad

Manuscripts, Articles, Book Chapters and Other Papers

Rationale & objective: Chronic kidney disease (CKD) in children is associated with cognitive dysfunction that affects school performance and quality of life. The relationship between CKD-mineral and bone disorder and cognitive function in children is unknown.

Study design: Observational study.

Participants: 702 children enrolled in the Chronic Kidney Disease in Children (CKiD) Study.

Predictors: Plasma fibroblast growth factor 23 (FGF-23), parathyroid hormone (PTH), calcium, phosphorus, 25 hydroxyvitamin D (25[OH]D), and 1,25 dihydroxyvitamin D (1,25[OH]2D).

Outcomes: Neurocognitive tests of intelligence, academic achievement, and executive functions.

Analytical approach: Linear regression models to analyze the cross-sectional associations between log2FGF-23, 25(OH)D, 1,25(OH)2D, PTH, calcium, …

Urinary Epidermal Growth Factor As A Marker Of Disease Progression In Children With Nephrotic Syndrome., Debbie S Gipson, Howard Trachtman, Anne Waldo, Keisha L Gibson, Sean Eddy, Katherine M Dell, Tarak Srivastava, Kevin V Lemley, Larry A Greenbaum, Sangeeta Hingorani, Kevin E Meyers, Frederick J Kaskel, Kimberly J Reidy, Christine B Sethna, Cheryl L Tran, Chia-Shi Wang, Katherine Tuttle, Gia Oh, Alicia M Neu, Elizabeth Brown, Jen-Jar Lin, Jennifer Lai Yee, Therese M Roth, Jonathan P Troost, Brenda W Gillespie, Matthew G Sampson, Matthias Kretzler, Wenjun Ju

Articles, Abstracts, and Reports

Introduction: Childhood-onset nephrotic syndrome has a variable clinical course. Improved predictive markers of long-term outcomes in children with nephrotic syndrome are needed. This study tests the association between baseline urinary epidermal growth factor (uEGF) excretion and longitudinal kidney function in children with nephrotic syndrome.

Methods: The study evaluated 191 participants younger than 18 years enrolled in the Nephrotic Syndrome Study Network, including 118 with their first clinically indicated kidney biopsy (68 minimal change disease; 50 focal segmental glomerulosclerosis) and 73 with incident nephrotic syndrome without a biopsy. uEGF was measured at baseline for all participants and normalized by the urine …

Urinary Epidermal Growth Factor As A Marker Of Disease Progression In Children With Nephrotic Syndrome., Debbie S. Gipson, Howard Trachtman, Anne Waldo, Keisha L. Gibson, Sean Eddy, Katherine M. Dell, Tarak Srivastava, Kevin V. Lemley, Larry A. Greenbaum, Sangeeta Hingorani, Kevin E. Meyers, Frederick J. Kaskel, Kimberly J. Reidy, Christine B. Sethna, Cheryl L. Tran, Chia-Shi Wang, Katherine R. Tuttle, Gia Oh, Alicia M. Neu, Elizabeth Brown, Jen-Jar Lin, Jennifer Lai Yee, Therese M M. Roth, Jonathan P. Troost, Brenda W. Gillespie, Matthew G. Sampson, Matthias Kretzler, Wenjun Ju

Manuscripts, Articles, Book Chapters and Other Papers

Introduction: Childhood-onset nephrotic syndrome has a variable clinical course. Improved predictive markers of long-term outcomes in children with nephrotic syndrome are needed. This study tests the association between baseline urinary epidermal growth factor (uEGF) excretion and longitudinal kidney function in children with nephrotic syndrome.

Methods: The study evaluated 191 participants younger than 18 years enrolled in the Nephrotic Syndrome Study Network, including 118 with their first clinically indicated kidney biopsy (68 minimal change disease; 50 focal segmental glomerulosclerosis) and 73 with incident nephrotic syndrome without a biopsy. uEGF was measured at baseline for all participants and normalized by the urine …

Energy And Protein Requirements For Children With Ckd Stages 2-5 And On Dialysis-Clinical Practice Recommendations From The Pediatric Renal Nutrition Taskforce., Vanessa Shaw, Nonnie Polderman, José Renken-Terhaerdt, Fabio Paglialonga, Michiel Oosterveld, Jetta Tuokkola, Caroline Anderson, An Desloovere, Laurence Greenbaum, Dieter Haffner, Christina Nelms, Leila Qizalbash, Johan Vande Walle, Bradley A. Warady, Rukshana Shroff, Lesley Rees

Manuscripts, Articles, Book Chapters and Other Papers

Dietary management in pediatric chronic kidney disease (CKD) is an area fraught with uncertainties and wide variations in practice. Even in tertiary pediatric nephrology centers, expert dietetic input is often lacking. The Pediatric Renal Nutrition Taskforce (PRNT), an international team of pediatric renal dietitians and pediatric nephrologists, was established to develop clinical practice recommendations (CPRs) to address these challenges and to serve as a resource for nutritional care. We present CPRs for energy and protein requirements for children with CKD stages 2-5 and those on dialysis (CKD2-5D). We address energy requirements in the context of poor growth, obesity, and different …

The Dietary Management Of Calcium And Phosphate In Children With Ckd Stages 2-5 And On Dialysis-Clinical Practice Recommendation From The Pediatric Renal Nutrition Taskforce., Louise Mcalister, Pearl Pugh, Laurence Greenbaum, Dieter Haffner, Lesley Rees, Caroline Anderson, An Desloovere, Christina Nelms, Michiel Oosterveld, Fabio Paglialonga, Nonnie Polderman, Leila Qizalbash, José Renken-Terhaerdt, Jetta Tuokkola, Bradley A. Warady, Johan Vande Walle, Vanessa Shaw, Rukshana Shroff

Manuscripts, Articles, Book Chapters and Other Papers

In children with chronic kidney disease (CKD), optimal control of bone and mineral homeostasis is essential, not only for the prevention of debilitating skeletal complications and achieving adequate growth but also for preventing vascular calcification and cardiovascular disease. Complications of mineral bone disease (MBD) are common and contribute to the high morbidity and mortality seen in children with CKD. Although several studies describe the prevalence of abnormal calcium, phosphate, parathyroid hormone, and vitamin D levels as well as associated clinical and radiological complications and their medical management, little is known about the dietary requirements and management of calcium (Ca) and …

Remarks About The Study On Infection-Associated Relapses In Children With Nephrotic Syndrome: A Short-Term Outcome Study, Akbar Shoukat Ali, Raheel Allana, Rahim Jandani

Department of Medicine

No abstract provided.

Oxidant Stress And Renal Function Among Children With Chronic Kidney Disease: A Repeated Measures Study., Melanie H. Jacobson, Mengling Liu, Yinxiang Wu, Susan Furth, Bradley A. Warady, Howard Trachtman, Leonardo Trasande

Manuscripts, Articles, Book Chapters and Other Papers

It is hypothesized that chronic kidney disease (CKD) induces oxidant stress which contributes to the decline in kidney function. However, few studies have incorporated longitudinal designs and no studies have investigated this association among children. Using data from the Chronic Kidney Disease in Children (CKiD) study, we examined longitudinal associations between urinary biomarkers of oxidant stress, 8-OH deoxyguanosine (8-OHdG) and F2-isoprostane, and measures of renal function and blood pressure among children with CKD. Baseline levels of 8-OHdG were positively associated with estimated glomerular filtration rate (eGFR) over time and a log-unit increase in baseline 8-OHdG predicted a 5.68 ml/min/1.73 m2 …

Association Of Time-Varying Blood Pressure With Chronic Kidney Disease Progression In Children., Ben Christopher Reynolds, Jennifer Lynn Roem, Derek Kai Sing Ng, Mina Matsuda-Abedini, Joseph Thomas Flynn, Susan Lynn Furth, Bradley A. Warady, Rulan Savita Parekh

Manuscripts, Articles, Book Chapters and Other Papers

Importance: Optimal blood pressure (BP) management in children with chronic kidney disease (CKD) slows progression to end-stage renal disease. Studies often base progression risk on a single baseline BP measurement, which may underestimate risk.

Objective: To determine whether time-varying BP measurements are associated with a higher risk of progression of CKD than baseline BP measurements.

Design, Setting, and Participants: The ongoing longitudinal, prospective cohort study Chronic Kidney Disease in Children (CKID) recruited children from January 19, 2005, through March 19, 2014, from pediatric nephrology centers across North America, with data collected at annual study visits. Participants included children aged 1 …

Pediatric Immunization Practices In Nephrotic Syndrome: An Assessment Of Provider And Parental Knowledge, Cheryl L Tran, David T Selewski, Gia J Oh, Jonathan P Troost, Susan F Massengill, Samhar I Al-Akash, Shefali Mahesh, Rasheda Amin, Isa F Ashoor, Rahul Chanchlani, Mahmoud Kallash, Robert P Woroniecki, Debbie S Gipson

Faculty, Staff and Student Publications

Background: Children with nephrotic syndrome (NS) are at high risk for vaccine-preventable infections due to the immunological effects from the disease and concurrent treatment with immunosuppressive medications. Immunizations in these patients may be deferred due to their immunosuppressive treatment which may increase the risk for vaccine-preventable infections. Immunization practices in children with NS continue to vary among pediatric nephrologists. This raises the question of whether children with NS are receiving the recommended vaccinations at appropriate times. Therefore, it is critical to understand the practices and patient education provided by physicians to patients on the topic of vaccinations.

Methods: After informed …

Incidence Of Initial Renal Replacement Therapy Over The Course Of Kidney Disease In Children., Derek K. Ng, Matthew B. Matheson, Bradley A. Warady, Susan R. Mendley, Susan L. Furth, Alvaro Muñoz

Manuscripts, Articles, Book Chapters and Other Papers

The Chronic Kidney Disease in Children Study, a prospective cohort study with data collected from 2003 to 2018, provided the first opportunity to characterize the incidence of renal replacement therapy (RRT) initiation over the life course of pediatric kidney diseases. In the current analysis, parametric generalized gamma models were fitted and extrapolated for RRT overall and by specific treatment modality (dialysis or preemptive kidney transplant). Children were stratified by type of diagnosis: nonglomerular (mostly congenital; n = 650), glomerular-hemolytic uremic syndrome (HUS; n = 49), or glomerular-non-HUS (heterogeneous childhood onset; n = 216). Estimated durations of time to RRT after …

Improving End Stage Renal Disease Quality Incentive Payment Total Performance Scores, Shelly Guyer, Gina Gregg, Bradley Warady

Posters

The End Stage Rena Disease (ESRD) Quality Incentive Program (QIP) promotes high quality, transparent, cost-efficient care in outpatient dialysis facilities by establishing performance standards that are linked to payment.

The development of a subject matter expert and the sharing of ESRD QIP knowledge resulted in buy-in from dialysis staff and physicians leading to a higher total performance score and the avoidance of payment reduction.

Text Messaging For Disease Monitoring In Childhood Nephrotic Syndrome., Chia-Shi Wang, Jonathan P. Troost, Larry A. Greenbaum, Tarak Srivastava, Kimberly Reidy, Keisha Gibson, Howard Trachtman, John D. Piette, Christine B. Sethna, Kevin Meyers, Katherine M. Dell, Cheryl L. Tran, Suzanne Vento, Krishna Kallem, Emily Herreshoff, Sangeeta Hingorani, Kevin Lemley, Gia Oh, Elizabeth Brown, Jen-Jar Lin, Frederick Kaskel, Debbie S. Gipson

Manuscripts, Articles, Book Chapters and Other Papers

Introduction: There is limited information on effective disease monitoring for prompt interventions in childhood nephrotic syndrome. We examined the feasibility and effectiveness of a novel text messaging system (SMS) for disease monitoring in a multicenter, prospective study.

Methods: A total of 127 patientsresults, symptoms, and medication adherence were sent to a designated caregiver (n = 116) or adolescent patient (n = 3). Participants responded by texting. Feasibility of SMS was assessed by SMS adoption, retention, and engagement, and concordance between participant-reported results and laboratory/clinician assessments. The number of disease relapses and time-to-remission data captured by SMS were compared …

Effects Of The 2017 Clinical Practice Guidelines On Hypertension In Children And Adolescents: A Commentary, Bonita Falkner

Department of Medicine Faculty Papers

No abstract provided.

The Effect Of Antihypertensive Medications Dosing On Blood Pressure Control And Left Ventricular Hypertrophy In Children With Chronic Kidney Disease, Benjamin A. Matta

Research Days

No abstract provided.

Predictive Performance Of Existing Population Pharmacokinetic Models Of Tacrolimus In Pediatric Kidney Transplant Recipients, Alenka Chapron

Research Days

No abstract provided.

The Physiology And Hormonal Control Of Calcium, Phosphate And Vitamin D, Uri Alon

Presentations

Describes calcium, phophate, and vitamin D in the context of pediatric rickets.

The Effect Of Antihypertensive Dosing On Hypertension In Children With Chronic Kidney Disease, Benjamin A. Matta, Uri S. Alon, Tarak Srivastava, Bradley A. Warady, Darcy Weidemann

Posters

This study's objective was to determine the effect of antihypertensive dose on hypertension status in children with chronic kidney disease. This was the first quantitative analysis of antihypertensive dose expressed as a newly developed measure, cDDI, and is relationship with hypertension status in children with CKD.

The Effect Of Antihypertensive Dose On Blood Pressure Control In Children With Chronic Kidney Disease, Benjamin A. Matta

Presentations

This presentation describes a study to determine the effect of antihypertensive dosing on blood pressure control in children with chronic kidney disease. The original version of this presentation is a prezi show at https://prezi.com/view/4qluDL1gMKFELOf1nLVA/

The Effect Of Antihypertensive Dosing On Hypertension In Children With Chronic Kidney Disease, Benjamin A. Matta, Uri S. Alon, Bradley Warady Md, Tarak Srivastava, Darcy Weidemann

Posters

Background

Hypertension (HTN) is a highly prevalent and major risk factor for poor cardiovascular and renal outcomes in chronic kidney disease (CKD). Previous research suggests that HTN is underdiagnosed and undertreated in children with CKD. To our knowledge no studies have investigated the effect of antihypertensive (antiHTN) dose on blood pressure (BP) control in this population.

Objective

To determine the effect of antiHTN dose on HTN status in children with CKD.

Methods

Study population: 255 participants studied in the Chronic Kidney Disease in Children (CKiD) study at their 3rd visit, taking at least 1 antiHTN drug.

Cumulative Drug Dose Index …

Peritoneal Dialysis Vintage And Glucose Exposure But Not Peritonitis Episodes Drive Peritoneal Membrane Transformation During The First Years Of Pd., Maria Bartosova, Betti Schaefer, Karel Vondrak, Peter Sallay, Christina Taylan, Rimante Cerkauskiene, Maria Dzierzega, Gordana Milosevski-Lomic, Rainer Büscher, Ariane Zaloszyc, Philipp Romero, Felix Lasitschka, Bradley A. Warady, Franz Schaefer, Akos Ujszaszi, Claus Peter Schmitt

Manuscripts, Articles, Book Chapters and Other Papers

The impact of peritoneal dialysis (PD) associated peritonitis on peritoneal membrane integrity is incompletely understood. Children are particularly suited to address this question, since they are largely devoid of preexisting tissue damage and life-style related alterations. Within the International Peritoneal Biobank, 85 standardized parietal peritoneal tissue samples were obtained from 82 children on neutral pH PD fluids with low glucose degradation product (GDP) content. 37 patients had a history of peritonitis and 16 of the 37 had two or more episodes. Time interval between tissue sampling and the last peritonitis episode was 9 (4, 36) weeks. Tissue specimen underwent digital …

Dr. Warady Wins 2019 Karl D. Nolph Lifetime Achievement Award In Dialysis, Children's Mercy Hospital

Our Story Continues

Bradley Warady, MD, Division Director-Pediatric Nephrology and Director-Dialysis and Transplantation, recently was presented with the 2019 Karl D. Nolph Lifetime Achievement Award in Dialysis at the 39th Annual Dialysis Conference in Dallas.

The Nolph award recognizes individuals whose academic achievements exemplify the academic life of Dr. Nolph and have contributed to unique and broader understanding and application of renal replacement therapies, including peritoneal dialysis.

Global Variation Of Nutritional Status In Children Undergoing Chronic Peritoneal Dialysis: A Longitudinal Study Of The International Pediatric Peritoneal Dialysis Network., Franz Schaefer, Laura Benner, Dagmara Borzych-Dużałka, Joshua Zaritsky, Hong Xu, Lesley Rees, Zenaida L Antonio, Erkin Serdaroglu, Nakysa Hooman, Hiren Patel, Lale Sever, Karel Vondrak, Joseph Flynn, Anabella Rébori, William Wong, Tuula Hölttä, Zeynep Yuruk Yildirim, Bruno Ranchin, Ryszard Grenda, Sara Testa, Dorota Drożdz, Attila J. Szabo, Loai Eid, Biswanath Basu, Renata Vitkevic, Cynthia Wong, Stephen J. Pottoore, Dominik Müller, Ruhan Dusunsel, Claudia Gonzalez Celedon, Marc Fila, Lisa Sartz, Anja Sander, Bradley A. Warady, International Pediatric Peritoneal Dialysis Network (Ippn) Registry

Manuscripts, Articles, Book Chapters and Other Papers

While children approaching end-stage kidney disease (ESKD) are considered at risk of uremic anorexia and underweight they are also exposed to the global obesity epidemic. We sought to investigate the variation of nutritional status in children undergoing chronic peritoneal dialysis (CPD) around the globe. The distribution and course of body mass index (BMI) standard deviation score over time was examined prospectively in 1001 children and adolescents from 35 countries starting CPD who were followed in the International Pediatric PD Network (IPPN) Registry. The overall prevalence of underweight, and overweight/obesity at start of CPD was 8.9% and 19.7%, respectively. Underweight was …

Maternal And Gestational Influences On Childhood Blood Pressure., Bonita Falkner

Department of Medicine Faculty Papers

Exposures that contribute to a sub-optimal intrauterine environment can have an effect on the developing fetus. Impaired fetal growth that results in low birth weight is an established risk factor for cardio-metabolic disorders later in life. Recent epidemiologic and prospective cohort studies that include the maternal and gestational period have identified maternal and gestational conditions that confer increased risk for subsequent cardio-metabolic disorders in the absence of low birth weight. Maternal pre-conception health status, including chronic obesity and type 2 diabetes, increase risk for childhood obesity and obesity-related higher blood pressure (BP) in child offspring. Maternal gestational exposures, including gestational …

The Copy Number Variation Landscape Of Congenital Anomalies Of The Kidney And Urinary Tract., Miguel Verbitsky, Rik Westland, Alejandra Perez, Krzysztof Kiryluk, Qingxue Liu, Priya Krithivasan, Adele Mitrotti, David A. Fasel, Ekaterina Batourina, Matthew G. Sampson, Monica Bodria, Max Werth, Charlly Kao, Jeremiah Martino, Valentina P. Capone, Asaf Vivante, Shirlee Shril, Byum Hee Kil, Maddalena Marasà, Jun Y. Zhang, Young-Ji Na, Tze Y. Lim, Dina Ahram, Patricia L. Weng, Erin L. Heinzen, Alba Carrea, Giorgio Piaggio, Loreto Gesualdo, Valeria Manca, Giuseppe Masnata, Maddalena Gigante, Daniele Cusi, Claudia Izzi, Francesco Scolari, Joanna A E Van Wijk, Marijan Saraga, Domenico Santoro, Giovanni Conti, Pasquale Zamboli, Hope White, Dorota Drozdz, Katarzyna Zachwieja, Monika Miklaszewska, Marcin Tkaczyk, Daria Tomczyk, Anna Krakowska, Przemyslaw Sikora, Tomasz Jarmoliński, Maria K. Borszewska-Kornacka, Robert Pawluch, Maria Szczepanska, Piotr Adamczyk, Malgorzata Mizerska-Wasiak, Grazyna Krzemien, Agnieszka Szmigielska, Marcin Zaniew, Mark G. Dobson, John M. Darlow, Prem Puri, David E. Barton, Susan L. Furth, Bradley A. Warady, Zoran Gucev, Vladimir J. Lozanovski, Velibor Tasic, Isabella Pisani, Landino Allegri, Lida M. Rodas, Josep M. Campistol, Cécile Jeanpierre, Shumyle Alam, Pasquale Casale, Craig S. Wong, Fangming Lin, Débora M. Miranda, Eduardo A. Oliveira, Ana Cristina Simões-E-Silva, Jonathan M. Barasch, Brynn Levy, Nan Wu, Friedhelm Hildebrandt, Gian Marco Ghiggeri, Anna Latos-Bielenska, Anna Materna-Kiryluk, Feng Zhang, Hakon Hakonarson, Virginia E. Papaioannou, Cathy L. Mendelsohn, Ali G. Gharavi, Simone Sanna-Cherchi

Manuscripts, Articles, Book Chapters and Other Papers

Congenital anomalies of the kidney and urinary tract (CAKUT) are a major cause of pediatric kidney failure. We performed a genome-wide analysis of copy number variants (CNVs) in 2,824 cases and 21,498 controls. Affected individuals carried a significant burden of rare exonic (that is, affecting coding regions) CNVs and were enriched for known genomic disorders (GD). Kidney anomaly (KA) cases were most enriched for exonic CNVs, encompassing GD-CNVs and novel deletions; obstructive uropathy (OU) had a lower CNV burden and an intermediate prevalence of GD-CNVs; and vesicoureteral reflux (VUR) had the fewest GD-CNVs but was enriched for novel exonic CNVs, …

An Open-Label, Single-Dose Study To Evaluate The Safety, Tolerability, Pharmacokinetics, And Pharmacodynamics Of Cinacalcet In Pediatric Subjects Aged 28 Days To < 6 Years With Chronic Kidney Disease Receiving Dialysis., Winnie Y. Sohn, Anthony A. Portale, Isidro B. Salusky, Hao Zhang, Lucy L. Yan, Bella Ertik, Shahnaz Shahinfar, Edward Lee, Bastian Dehmel, Bradley A. Warady

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Calcimimetics, shown to control biochemical parameters of secondary hyperparathyroidism (SHPT), have well-established safety and pharmacokinetic profiles in adult end-stage renal disease subjects treated with dialysis; however, such studies are limited in pediatric subjects.

METHODS: In this study, the safety, tolerability, pharmacokinetics (PK), and pharmacodynamics (PD) of cinacalcet were evaluated in children with chronic kidney disease (CKD) and SHPT receiving dialysis. Twelve subjects received a single dose of cinacalcet (0.25 mg/kg) orally or by nasogastric or gastric tube. Subjects were randomized to one of two parathyroid hormone (PTH) and serum calcium sampling sequences: [(1) 2, 8, 48 h; or (2) …

Childhood Kidney Disease: A Troubling Prognosis?, Darcy K. Weidemann, Bradley A. Warady, Susan L. Furth

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.

Pharmacokinetics Of Ferric Pyrophosphate Citrate Administered Via Dialysate And Intravenously To Pediatric Patients On Chronic Hemodialysis., Raymond D. Pratt, Sarah Grimberg, Joshua J. Zaritsky, Bradley A. Warady

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Iron deficiency is a common cause of anemia in pediatric patients with hemodialysis-dependent chronic kidney disease (CKD-5HD). Ferric pyrophosphate citrate (FPC, Triferic®) donates iron directly to transferrin, bypassing the reticuloendothelial system and avoiding iron sequestration. Administration of FPC via dialysate or intravenously (IV) may provide a suitable therapeutic option to current IV iron preparations for these patients.

METHODS: The pharmacokinetics and safety of FPC administered via dialysate and IV to patients aged < 6 years (n = 3), 6 to < 12 years (n = 4), and 12 to < 18 years (n = 15) were investigated in a multicenter, open-label, two-period, single-dose study. FPC (0.07 mg iron/kg) was infused IV into the venous blood return line during hemodialysis session no. 1. FPC iron was added to bicarbonate concentrate to deliver 2 μM (110 μg/L) iron via dialysate during hemodialysis session no. 2.

RESULTS: Mean serum total iron concentrations peaked 3 to 4 h after administration via dialysate and 2 to 4 h after IV administration and returned to baseline …

Recurrence Of Nephrotic Syndrome Following Kidney Transplantation Is Associated With Initial Native Kidney Biopsy Findings., Jonathan H. Pelletier, Karan R. Kumar, Rachel Engen, Adam Bensimhon, Jennifer D. Varner, Michelle N. Rheault, Tarak Srivastava, Caroline Straatmann, Cynthia Silva, T Keefe Davis, Scott E. Wenderfer, Keisha Gibson, David Selewski, John Barcia, Patricia Weng, Christoph Licht, Natasha Jawa, Mahmoud Kallash, John W. Foreman, Delbert R. Wigfall, Annabelle N. Chua, Eileen Chambers, Christoph P. Hornik, Eileen D. Brewer, Shashi K. Nagaraj, Larry A. Greenbaum, Rasheed A. Gbadegesin

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND AND OBJECTIVES: Steroid-resistant nephrotic syndrome (SRNS) due to focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD) is a leading cause of end-stage kidney disease in children. Recurrence of primary disease following transplantation is a major cause of allograft loss. The clinical determinants of disease recurrence are not completely known. Our objectives were to determine risk factors for recurrence of FSGS/MCD following kidney transplantation and factors that predict response to immunosuppression following recurrence.

METHODS: Multicenter study of pediatric patients with kidney transplants performed for ESKD due to SRNS between 1/2006 and 12/2015. Demographics, clinical course, and biopsy data were …

An Eqtl Landscape Of Kidney Tissue In Human Nephrotic Syndrome., Christopher E. Gillies, Rosemary Putler, Rajasree Menon, Edgar Otto, Kalyn Yasutake, Viji Nair, Paul Hoover, David Lieb, Shuqiang Li, Sean Eddy, Damian Fermin, Michelle T. Mcnulty, Nephrotic Syndrome Study Network (Neptune), Nir Hacohen, Krzysztof Kiryluk, Matthias Kretzler, Xiaoquan Wen, Matthew G. Sampson, Tarak Srivastava

Manuscripts, Articles, Book Chapters and Other Papers

Expression quantitative trait loci (eQTL) studies illuminate the genetics of gene expression and, in disease research, can be particularly illuminating when using the tissues directly impacted by the condition. In nephrology, there is a paucity of eQTL studies of human kidney. Here, we used whole-genome sequencing (WGS) and microdissected glomerular (GLOM) and tubulointerstitial (TI) transcriptomes from 187 individuals with nephrotic syndrome (NS) to describe the eQTL landscape in these functionally distinct kidney structures. Using MatrixEQTL, we performed cis-eQTL analysis on GLOM (n = 136) and TI (n = 166). We used the Bayesian "Deterministic Approximation of Posteriors" (DAP) to fine-map …