Nephrology Commons^™

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) …

Diuretic, Natriuretic, And Vasodepressor Activity Of A Lipid Fraction Enhanced In Medium Of Cultured Mouse Medullary Interstitial Cells By A Selective Faah Inhibitor, Zdravka P. Daneva

Theses and Dissertations

The relationship between the endocannabinoid system in the renal medulla and the long-term regulation of blood pressure is not well understood. To investigate the possible role of the endocannabinoid system in renomedullary interstitial cells, mouse medullary interstitial cells (MMICs) were obtained, cultured and characterized for their responses to treatment with a selective inhibitor of fatty acid amide hydrolase (FAAH), PF-3845. Treatment of MMICs with PF-3845 increased cytoplasmic lipid granules detected by Sudan Black B staining and multilamellar bodies identified by transmission electron microscopy. HPLC analyses of lipid extracts of MMIC culture medium revealed a 205nm-absorbing peak that showed responsiveness to …

Clinical Characteristics And Treatment Patterns Of Children And Adults With Iga Nephropathy Or Iga Vasculitis: Findings From The Curegn Study., David T. Selewski, Josephine M. Ambruzs, Gerald B. Appel, Andrew S. Bomback, Raed Bou Matar, Yi Cai, Daniel C. Cattran, Aftab S. Chishti, Vivette D. D'Agati, Cynthia J. D'Alessandri-Silva, Rasheed A. Gbadegesin, Jonathan J. Hogan, Sandra Iragorri, J Charles Jennette, Bruce A. Julian, Myda Khalid, Richard A. Lafayette, Helen Liapis, Francesca Lugani, Sarah A. Mansfield, Sherene Mason, Patrick H. Nachman, Cynthia C. Nast, Carla M. Nester, Damien G. Noone, Jan Novak, Michelle M. O'Shaughnessy, Heather N. Reich, Michelle N. Rheault, Dana V. Rizk, Manish K. Saha, Neil S. Sanghani, C John Sperati, Rajasree Sreedharan, Tarak Srivastava, Agnieszka Swiatecka-Urban, Katherine Twombley, Tetyana L. Vasylyeva, Donald J. Weaver, Hong Yin, Jarcy Zee, Ronald J. Falk, Ali G. Gharavi, Brenda W. Gillespie, Debbie S. Gipson, Larry A. Greenbaum, Lawrence B. Holzman, Matthias Kretzler, Bruce M. Robinson, William E. Smoyer, Michael Flessner, Lisa M. Guay-Woodford, Krzysztof Kiryluk, Curegn Consortium

Manuscripts, Articles, Book Chapters and Other Papers

Introduction: The Cure Glomerulonephropathy Network (CureGN) is a 66-center longitudinal observational study of patients with biopsy-confirmed minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, or IgA nephropathy (IgAN), including IgA vasculitis (IgAV). This study describes the clinical characteristics and treatment patterns in the IgA cohort, including comparisons between IgAN versus IgAV and adult versus pediatric patients.

Methods: Patients with a diagnostic kidney biopsy within 5 years of screening were eligible to join CureGN. This is a descriptive analysis of clinical and treatment data collected at the time of enrollment.

Results: A total of 667 patients (506 IgAN, 161 IgAV) constitute …

Duet: A Phase 2 Study Evaluating The Efficacy And Safety Of Sparsentan In Patients With Fsgs., Howard Trachtman, Peter Nelson, Sharon Adler, Kirk N. Campbell, Abanti Chaudhuri, Vimal Kumar Derebail, Giovanni Gambaro, Loreto Gesualdo, Debbie S. Gipson, Jonathan Hogan, Kenneth Lieberman, Brad Marder, Kevin Edward Meyers, Esmat Mustafa, Jai Radhakrishnan, Tarak Srivastava, Miganush Stepanians, Vladimír Tesar, Olga Zhdanova, Radko Komers, Duet Study Group

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: We evaluated and compared the effects of sparsentan, a dual endothelin type A (ETA) and angiotensin II type 1 receptor antagonist, with those of the angiotensin II type 1 receptor antagonist irbesartan in patients with primary FSGS.

METHODS: In this phase 2, randomized, double-blind, active-control Efficacy and Safety of Sparsentan (RE-021), a Dual Endothelin Receptor and Angiotensin Receptor Blocker, in Patients with Focal Segmental Glomerulosclerosis (FSGS): A Randomized, Double-blind, Active-Control, Dose-Escalation Study (DUET), patients aged 8-75 years with biopsy-proven FSGS, eGFR>30 ml/min per 1.73 m2, and urinary protein-to-creatinine ratio (UP/C) ≥1.0 g/g received sparsentan (200, 400, or 800 …

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 …

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.

Predicting Adverse Outcomes In End Stage Renal Disease: Machine Learning Applied To The United States Renal Data System, Zeid Khitan, Alexis D. Jacob, Courtney Balentine, Adam N. Jacob, Juan R. Sanabria, Joseph I. Shapiro

Marshall Journal of Medicine

We examined machine learning methods to predict death within six months using data derived from the United States Renal Data System (USRDS). We specifically evaluated a generalized linear model, a support vector machine, a decision tree and a random forest evaluated within the context of K-10 fold validation using the CARET package available within the open source architecture R program. We compared these models with the feed forward neural network strategy that we previously reported on with this data set.

Thrombocytopenia In End-Stage Renal Disease And Chronic Viral Hepatitis B Or C, Olga Hilda Orasan, Laura Urian, George Ciulei, Iulia Breaban, Andreea Maria Stefan, Sorina Cezara Secara, Adela Sitar Taut, Simina Tarmure Sarlea, Vasile Negrean, Dorel Sampelean, Ioan Mihai Patiu, Remus Aurel Orasan, Angela Cozma

Journal of Mind and Medical Sciences

Objectives. We evaluated platelet counts in end-stage renal disease and chronic viral hepatitis.

Materials and Methods. We studied 70 patients with end-stage renal disease and chronic viral hepatitis and compared them to a control group of 45 patients without hepatitis.

Results. The presence of viral hepatitis was associated with a higher prevalence of thrombocytopenia. Correlations between age, C-reactive protein, liver stiffness measurement, and platelet count were observed. C-reactive protein levels > 10 mg/dl were associated with a lower risk of thrombocytopenia in patients with end-stage renal disease and chronic viral hepatitis, yet age > 60 years, dialysis vintage > 10 years, aspartate and …

A Brief Review Of The Literature On The Malignant Ureteral Obstruction, Sorin Lupu, Adrian Brînză, Bogdan Socea, Dragos Marcu, Ileana Peride, Ana Maria Alexandra Stanescu, Tiberiu P. Neagu, Laurian Maxim

Journal of Mind and Medical Sciences

Malignant ureteral obstruction (MUO) caused by a primarily urological tumor or secondary to a late-stage malignancy can be difficult for the urologist to manage. Due to a lack of clinical data on the management of MUO, every case is particular and should be aborted individually.

Lack of specific treatment, either palliative or definitive, can severely damage renal function and lifetime expectancy in patients, causing even more damage that could otherwise be avoided.

Prompt management directed at the recovery of renal function is the main goal in such cases. Even after urinary flow is restored, life threatening post-obstructive diuresis needs to …

Young Man With Severe Metabolic Acidosis After Transformer Oil Ingestion: A Case Report, Farah Gul Khan, Syed Muhammad Zubair

Section of Nephrology

Background: Transformer oil is used in oil-filled transformers for its insulating as well as coolant properties. Transformer oil ingestion for attempted suicide is seldom heard of. Our patient’s case presented us with a major diagnostic as well as treatment challenge because we encountered such a case for the first time and were totally unaware of the fact that methanol might make up the main component of an aged transformer oil.

Case presentation: A 19-year-old Pakistani/Asian man was brought to our hospital with altered sensorium. He was found to have elevated anion gap acidosis, increased osmolal gap, and acute kidney injury. …

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 …

Hyperfiltration-Mediated Injury In The Remaining Kidney Of A Transplant Donor., Tarak Srivastava, Sundaram Hariharan, Uri S. Alon, Ellen T. Mccarthy, Ram Sharma, Ashraf El-Meanawy, Virginia J. Savin, Mukut Sharma

Manuscripts, Articles, Book Chapters and Other Papers

Kidney donors face a small but definite risk of end-stage renal disease 15 to 30 years postdonation. The development of proteinuria, hypertension with gradual decrease in kidney function in the donor after surgical resection of 1 kidney, has been attributed to hyperfiltration. Genetic variations, physiological adaptations, and comorbidities exacerbate the hyperfiltration-induced loss of kidney function in the years after donation. A focus on glomerular hemodynamics and capillary pressure has led to the development of drugs that target the renin-angiotensin-aldosterone system (RAAS), but these agents yield mixed results in transplant recipients and donors. Recent work on glomerular biomechanical forces highlights the …

Frequency Of Bloodstream Infection In Febrile Neutropenic Patients, Experience From A Developing Country, Basit Siddiqui, Rabeea Azmat, Shiyam Sunder Tikmani, Shumaila Rafi, Beenish Syed, Muhammad Tahir Khan, Hareem Rehman, Saleemullah Paracha

Section of Nephrology

Introduction: About 20% of febrile neutropenic patients are presented with bloodstream infection which is a leading cause of mortality among these patients. Awareness of the locally prevalent pathogens and their susceptibility pattern is important for proper treatment of infection which improves survival in these patients. The objective of this study was to determine the frequency of bloodstream infection in patients with febrile neutropenia admitted in Abbasi Shaheed Hospital.
Methods; This cross-sectional study was conducted in the Medical wards in Abbasi Shaheed Hospital, Karachi from 21-2-2016 to 20-8-2017. Patients of over 15 years of age of either gender with severe neutropenia …

Bilateral Psoas Abscess After Extracorporeal Shock Wave Lithotripsy: Reminder Of A Rare Complication, Sidra Zafar, Syed Usman Bin Mahmood, Arsal Tharwani, Noreen Nasir

Section of Nephrology

Extracorporeal shock wave lithotripsy (ESWL) is among the most widely used modalities for treatment of urolithiasis since its introduction in the 1980s. The non-invasive technique reduces the risk of post-procedure complications and produces excellent stone free rates. However, complications may still follow and albeit rare, there are reports in literature of abscess formation post ESWL. Herein, we report the case of a young, immunocompetent gentleman who developed bilateral psoas abscess after undergoing this procedure. He was successfully managed at our institute with intravenous antibiotics, percutaneous abscess drainage and was discharged on day 5 post-intervention in a stable condition. There have …

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 …

Cardiovascular Outcomes Reported In Hemodialysis Trials, Emma O'Lone, Andrea K. Viecelli, Jonathan C. Craig, Allison Tong, Benedicte Sautenet, David Roy, William G. Herrington, Charles A. Herzog, Tazeen Jafar, Meg Jardine

Section of Nephrology

Patients on long-term hemodialysis are at very high risk for cardiovascular disease but are usually excluded from clinical trials conducted in the general population or in at-risk populations. There are no universally agreed cardiovascular outcomes for trials conducted specifically in the hemodialysis population. In this review, we highlight that trials reporting cardiovascular outcomes in hemodialysis patients are usually of short duration (median 3 to 6 months) and are small (59% of trials have <100 participants). Overall, the cardiovascular outcomes are very heterogeneous and may not reflect outcomes that are meaningful to patients and clinicians in supporting decision making, as they are often surrogates of uncertain clinical importance. Composite outcomes used in different trials rarely share the same components. In a field in which a single trial is often insufficiently powered to fully assess the clinical and economic impact of interventions, differences in outcome reporting across trials make the task of meta-analysis and interpretation of all the available evidence challenging. Core outcome sets are now being established across many specialties in health care to prevent these problems. Through the global Standardized Outcomes in Nephrology-Hemodialysis initiative, cardiovascular disease was identified as a critically important core domain to be reported in all trials in hemodialysis. Informed by the current state of reporting of cardiovascular outcomes, a core outcome measure for cardiovascular disease is currently being established with involvement of patients, caregivers, and health professionals. Consistent reporting of cardiovascular outcomes that are critically important to hemodialysis patients and clinicians will strengthen the evidence base to inform care in this very high-risk population.

Urinothorax Caused By Xanthogranulomatous Pyelonephritis, Waiel Abusnina, Hazim Bukamur, Zeynep C. Koc

Internal Medicine

Xanthogranulomatous pyelonephritis is a rare form of chronic pyelonephritis that generally afflicts middle-aged women with a history of recurrent urinary tract infections. Its pathogenesis generally involves calculus obstructive uropathy and its histopathology is characterized by replacement of the renal parenchyma with lipid filled macrophages. This often manifests as an enlarged, nonfunctioning kidney that may be complicated by abscess or fistula. This case details the first reported case of xanthogranulomatous pyelonephritis complicated by urinothorax, which resolved on follow-up chest X-ray after robot-assisted nephrectomy.

Pelvic Kidney: A Review Of The Literature., Seif Eid, Joe Iwanaga, Marios Loukas, Rod J Oskouian, R Shane Tubbs

Articles, Abstracts, and Reports

Kidney development is a complex process that begins during the sixth to eighth weeks of life. Failure of ascent of the kidney will cause the kidney to remain in the pelvis i.e., pelvic kidney. Here, we review this entity in detail and illustrate such embryological derailment. In most cases, a pelvic kidney is an incidental finding and is usually asymptomatic. Anatomic variations of the renal vasculature have been reported in cases of pelvic kidneys and these are highlighted in this review. Clinicians who treat patients for renal or pelvic disease or interpret images of the pelvis should be well informed …

Improving Transitions Of Care For Inpatients With An Egfr < 20ml/Min, Brianna Shinn, Md, Brandon Menachem, Md, Robert Park, Md, Sarah Houtmann, Md, Zachary Lee, Md, Thomas Holden, Md, Tomoyuki Hongo, Md, Vincent Yeung, Md, Goni Katz-Greenberg, Md, Peter Burke, Do, Mba

House Staff Quality Improvement and Patient Safety Conference (2016-2019)

In this study we conducted an analysis of 22 inpatients with an eGFR<20 that revealed there was a greater prevalence of patients without permanent AV access for HD initiation when compared to national data. These findings demonstrated the need for improved transitions of care and timely creation of AV access for this patient population, thus we sought to improve this. This is the first inpatient initiative that our group is aware with the aim of improving timely AV access and transitions of care for an inpatient population (Figure 1).