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End Stage Renal Disease pediatric

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https://www.readbyqxmd.com/read/28926375/early-effects-of-renal-replacement-therapy-on-cardiovascular-comorbidity-in-children-with-end-stage-kidney-disease-findings-from-the-4c-t-study
#1
Bernhard M W Schmidt, Rizky I Sugianto, Daniela Thurn, Karolis Azukaitis, Aysun K Bayazit, Nur Canpolat, Ayse Guler Eroglu, Salim Caliskan, Anke Doyon, Ali Duzova, Tevfik Karagoz, Ali Anarat, Murat Deveci, Sevgi Mir, Bruno Ranchin, Rukshana Shroff, Esra Baskin, Mieczyslaw Litwin, Z Birsin Özcakar, Rainer Büscher, Oguz Soylemezoglu, Jiri Dusek, Markus Kemper, Maria C Matteucci, Sandra Habbig, Guido Laube, Elke Wühl, Uwe Querfeld, Anja Sander, Franz Schaefer, Anette Melk
BACKGROUND: The early impact of renal transplantation on subclinical cardiovascular measures in pediatric patients has not been widely investigated. This analysis is performed for pediatric patients participating in the prospective 4C study and focuses on the early effects of RRT modality on cardiovascular comorbidity in patients receiving a preemptive transplant or started on dialysis. METHODS: We compared measures indicating subclinical cardiovascular organ damage (aortal pulse wave velocity, carotid intima media thickness, left ventricular mass index) and evaluated cardiovascular risk factors in 166 pediatric patients before and 6 to 18 months after start of RRT (n=76 transplantation, n=90 dialysis)...
September 18, 2017: Transplantation
https://www.readbyqxmd.com/read/28921387/analysis-of-24-genes-reveals-a-monogenic-cause-in-11-1-of-cases-with-steroid-resistant-nephrotic-syndrome-at-a-single-center
#2
Weizhen Tan, Svjetlana Lovric, Shazia Ashraf, Jia Rao, David Schapiro, Merlin Airik, Shirlee Shril, Heon Yung Gee, Michelle Baum, Ghaleb Daouk, Michael A Ferguson, Nancy Rodig, Michael J G Somers, Deborah R Stein, Asaf Vivante, Jillian K Warejko, Eugen Widmeier, Friedhelm Hildebrandt
BACKGROUND: Steroid-resistant nephrotic syndrome (SRNS) is the second most frequent cause of end-stage renal disease (ESRD) among patients manifesting at under 25 years of age. We performed mutation analysis using a high-throughput PCR-based microfluidic technology in 24 single-gene causes of SRNS in a cohort of 72 families, who presented with SRNS before the age of 25 years. METHODS: Within an 18-month interval, we obtained DNA samples, pedigree information, and clinical information from 77 consecutive children with SRNS from 72 different families seen at Boston Children's Hospital (BCH)...
September 18, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28884036/severe-hepatopulmonary-syndrome-in-a-child-with-caroli-syndrome
#3
W De Jesus-Rojas, K McBeth, A Yadav, J M Stark, R A Mosquera, C Jon
Hepatopulmonary Syndrome (HPS) is a potential complication of chronic liver disease and is more commonly seen in the adult population. Caroli Syndrome is a rare inherited disorder characterized by intrahepatic ductal dilation and liver fibrosis that leads to portal hypertension. In children with liver disease, HPS should be considered in the differential diagnosis of prolonged, otherwise unexplained, hypoxemia. The presence of HPS can improve patient priority on the liver transplantation wait list, despite their Pediatric End-Stage Liver Disease (PELD) score...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28873129/association-of-serum-soluble-urokinase-receptor-levels-with-progression-of-kidney-disease-in-children
#4
Franz Schaefer, Howard Trachtman, Elke Wühl, Marietta Kirchner, Salim S Hayek, Ali Anarat, Ali Duzova, Sevgi Mir, Dusan Paripovic, Alev Yilmaz, Francesca Lugani, Klaus Arbeiter, Mieczyslaw Litwin, Jun Oh, Maria Chiara Matteucci, Jutta Gellermann, Simone Wygoda, Augustina Jankauskiene, Günter Klaus, Jiri Dusek, Sara Testa, Aleksandra Zurowska, Alberto Caldas Afonso, Melissa Tracy, Changli Wei, Sanja Sever, William Smoyer, Jochen Reiser
Importance: Conventional methods to diagnose and monitor chronic kidney disease (CKD) in children, such as creatinine level and cystatin C-derived estimated glomerular filtration rate (eGFR) and assessment of proteinuria in spot or timed urine samples, are of limited value in identifying patients at risk of progressive kidney function loss. Serum soluble urokinase receptor (suPAR) levels strongly predict incident CKD stage 3 in adults. Objective: To determine whether elevated suPAR levels are associated with renal disease progression in children with CKD...
September 5, 2017: JAMA Pediatrics
https://www.readbyqxmd.com/read/28860113/physiologically-based-pharmacokinetic-pbpk-model-of-the-cyp2d6-probe-atomoxetine-extrapolation-to-special-populations-and-drug-drug-interactions
#5
Weize Huang, Mariko Nakano, Jennifer E Sager, Isabelle Ragueneau-Majlessi, Nina Isoherranen
Physiologically based pharmacokinetic (PBPK) modeling of drug disposition and drug-drug interactions has become a key component of drug development. PBPK modeling has also been considered as an approach to predict drug disposition in special populations. However, whether models developed and validated in healthy populations can be extrapolated to special populations is not well established. The goal of this study was to determine whether a drug specific PBPK model validated using healthy populations could be used to predict drug disposition in specific populations and in organ impairment...
August 31, 2017: Drug Metabolism and Disposition: the Biological Fate of Chemicals
https://www.readbyqxmd.com/read/28858859/independent-risk-factors-and-2-year-outcomes-of-acute-kidney-injury-after-surgery-for-congenital-heart-disease
#6
Daishi Hirano, Akira Ito, Akifumi Yamada, Daisuke Kakegawa, Saori Miwa, Chisato Umeda, Kosuke Chiba, Yoichi Takemasa, Ai Tokunaga, Hiroyuki Ida
BACKGROUND: Data are limited regarding risk factors for acute kidney injury (AKI) following cardiac surgery in children with congenital heart disease (CHD). This observational study was performed to examine temporal trends in AKI incidence according to the Pediatric Risk, Injury, Failure, Loss, End-Stage Renal Disease (pRIFLE) criteria, identify independent risk factors for AKI after cardiac surgery, and examine associations between AKI and long-term mortality. METHODS: We retrospectively evaluated 418 patients (259 males, 159 females; median age, 5 months) who underwent cardiac surgery for CHD between April 2007 and August 2013...
September 1, 2017: American Journal of Nephrology
https://www.readbyqxmd.com/read/28856014/grayscale-ultrasound-characteristics-of-autosomal-dominant-polycystic-kidney-disease-severity-an-adult-and-pediatric-cohort-study
#7
Marcin Strzelczyk, Michał Podgórski, Susan Afshari, Marcin Tkaczyk, Monika Pawlak-Bratkowska, Piotr Grzelak
INTRODUCTION: The most common hereditary kidney condition is autosomal dominant polycystic kidney disease. It is the cause of 5-10% of end-stage renal disease. Its symptoms are generally late-onset, typically leading to development of hypertension and chronic kidney disease. Ultrasonography is the imaging modality of choice in its diagnosis and management. The aim of this study is to determine the diagnostic value of grayscale ultrasound imaging in evaluating disease severity. MATERIALS AND METHODS: The study group consisted of 81 patients diagnosed with autosomal dominant polycystic kidney disease, 35 adults and 46 children...
June 2017: Journal of Ultrasonography
https://www.readbyqxmd.com/read/28842757/successful-long-term-outcome-of-pediatric-liver-kidney-transplantation-a-single-center-study
#8
Jesús Quintero Bernabeu, Javier Juamperez, Marina Muñoz, Olalla Rodriguez, Ramon Vilalta, José A Molino, Marino Asensio, Itxarone Bilbao, Gema Ariceta, Carlos Rodrigo, Ramón Charco
INTRODUCTION: Liver-kidney transplantation is a rare procedure in children, with just ten to 30 cases performed annually worldwide. The main indications are autosomal recessive polycystic liver-kidney disease and primary hyperoxaluria. This study aimed to report outcomes of liver-kidney transplantation in a cohort of pediatric patients. METHODS: We retrospectively analyzed all pediatric liver-kidney transplantations performed in our center between September 2000 and August 2015...
August 25, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28828168/the-long-term-outcome-of-pediatric-kidney-transplantation-in-iran-results-of-a-25-year-single-center-cohort-study
#9
G Naderi, A Latif, S Karimi, F Tabassomi, S T Esfahani
BACKGROUND: Kidney transplantation is the optimal treatment for end-stage renal disease in children. However, long-term graft survival has not significantly improved among pediatric patients. OBJECTIVE: To investigate the determinants of long-term graft survival among Iranian pediatric recipients of kidney transplantation. METHODS: In a single-center cohort study, we studied 314 pediatric kidney transplantations performed from 1989 to 2013 at Dr...
2017: International Journal of Organ Transplantation Medicine
https://www.readbyqxmd.com/read/28799058/is-cd44-in-glomerular-parietal-epithelial-cells-a-pathological-marker-of-renal-function-deterioration-in-primary-focal-segmental-glomerulosclerosis
#10
Brunna Pinto Froes, Stanley de Almeida Araújo, Eduardo Alves Bambirra, Eduardo Araújo Oliveira, Ana Cristina Simões E Silva, Sérgio Veloso Brant Pinheiro
BACKGROUND: The search for risk factors for chronic kidney disease in children with focal segmental glomerulosclerosis (FSGS) is important in defining prognosis and individualized treatment. This study preliminarily investigated whether CD44 immunostaining in glomerular parietal epithelial cells (PECs) is a prognostic marker in pediatric FSGS. METHODS: In this retrospective study, 26 patients with FSGS, biopsied from 1985 to 2010, were evaluated. Immunohistochemistry for CD44 was performed in all cases...
August 10, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28766066/outcome-of-kidney-transplantation-in-pediatric-patients-with-anca-associated-glomerulonephritis-a-single-center-experience
#11
Damien Noone, Rae S M Yeung, Diane Hebert
OBJECTIVES: Data on kidney transplant outcomes for pediatric patients with end-stage renal disease (ESRD) secondary to anti-neutrophil cytoplasmic antibody glomerulonephritis (ANCA GN), particularly granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), is limited. We describe our experience of kidney transplantation in pediatric ANCA GN patients. METHODS: We performed a retrospective review of patients with ANCA GN who developed ESRD and were transplanted at a single center between the years 2000 and 2014...
August 1, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28750890/cost-analysis-of-substitutive-renal-therapies-in-children
#12
Maria Fernanda Carvalho de Camargo, Klenio de Souza Barbosa, Seiji Kumon Fetter, Ana Bastos, Luciana de Santis Feltran, Paulo Cesar Koch-Nogueira
OBJECTIVE: End-stage renal disease is a health problem that consumes public and private resources. This study aimed to identify the cost of hemodialysis (either daily or conventional hemodialysis) and transplantation in children and adolescents. METHODS: This was a retrospective cohort of pediatric patients with End-stage renal disease who underwent hemodialysis followed by kidney transplant. All costs incurred in the treatment were collected and the monthly total cost was calculated per patient and for each renal therapy...
July 24, 2017: Jornal de Pediatria
https://www.readbyqxmd.com/read/28735996/effect-of-hla-matching-on-pediatric-renal-transplant-graft-survival-in-china
#13
H Zhao, X Lai, J Lu, Y Lin, P Wang, L Zhu, L Wu, Z Xiao, Q Wang, J Tan
INTRODUCTION: Kidney transplantation (KTX) has become one of the most effective therapies of choice for pediatric patients with end-stage renal disease (ESRD). The extent to which HLA mismatching influences graft survival following KTX has not been clarified. PATIENTS AND METHODS: The effect of HLA amino acid residue matching on graft survival was assessed in 36 pediatric patients in whom complete donor-recipient HLA data were available. HLA-A, -B, -DR and HLA-A+B+DR loci residue mismatches were separately analyzed...
July 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28699323/intra-procedural-continuous-dialysis-to-facilitate-interventional-catheterization-in-pediatric-patients-with-severe-renal-failure
#14
Angeline D Opina, Athar M Qureshi, Eileen Brewer, Ewa Elenberg, Sarah Swartz, Mini Michael, Henri Justino
BACKGROUND: Interventional catheterization procedures may be needed for patients with severe renal failure who are dependent on dialysis. To avoid the risk of fluid overload and electrolyte derangement during complex procedures in this oliguric/anuric patient population, we performed intra-procedural dialysis, either continuous renal replacement therapy (CRRT) or continous cycling peritoneal dialysis (CCPD). METHODS: We performed a retrospective review of a cohort of pediatric patients, ages 0-18 years, with dialysis-dependent renal failure who received CRRT or CCPD during catheterization procedures from January 2013 to March 2016...
July 12, 2017: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/28673276/worldwide-view-of-nephropathic-cystinosis-results-from-a-survey-from-30-countries
#15
Aurélia Bertholet-Thomas, Julien Berthiller, Velibor Tasic, Behrouz Kassai, Hasan Otukesh, Marcella Greco, Jochen Ehrich, Rejane de Paula Bernardes, Georges Deschênes, Sally-Ann Hulton, Michel Fischbach, Kenza Soulami, Bassam Saeed, Ehsan Valavi, Carlos Jose Cobenas, Bülent Hacihamdioglu, Gabrielle Weiler, Pierre Cochat, Justine Bacchetta
BACKGROUND: Nephropathic cystinosis is a rare inherited metabolic disorder leading to progressive renal failure and extra-renal comorbidity. The prognosis relies on early adherence to cysteamine treatment and symptomatic therapies. Developing nations [DiN] experience many challenges for management of cystinosis. The aim of this study was to assess the management characteristics in DiN compared with developed nations [DeN]. METHODS: A questionnaire was sent between April 2010 and May 2011 to 87 members of the International Pediatric Nephrology Association, in 50 countries...
July 3, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28631039/graft-nephrectomy-in-children
#16
Benedict L Phillips, Chris J Callaghan
Kidney transplantation is recognised as the gold standard treatment of end-stage renal disease in most children, with excellent graft survival rates. When graft failure occurs, renal transplant recipients (RTRs) have the option of removal of the transplant (graft nephrectomy [GN]), or leaving the failed transplant in situ. The aims of this review are to discuss the indications for GN, surgical techniques, outcomes after GN (including risks of allosensitisation and the impact on subsequent transplants), and the possible role of routine GN in the asymptomatic RTR with a failed renal allograft...
June 19, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28612957/the-impact-of-end-stage-renal-disease-in-children-on-their-parents
#17
Hee Sun Baek, Ki-Soo Park, Il Soo Ha, Hee Gyung Kang, Hae Il Cheong, Young Seo Park, Joo Hoon Lee, Hee Yeon Cho, Min Hyun Cho
AIM: This study was designed to investigate the impact of pediatric end-stage renal disease (ESRD) on parents, based on the PedsQL™ Family Impact Module (FIM), and the relationship to the quality of life (QOL) of pediatric ESRD patients measured by PedsQL(TM) 3.0 ESRD module. METHODS: We performed a cross-sectional study using Korean translations of the PedsQL(TM) FIM and the PedsQL(TM) 3.0 ESRD module. In all, 79 patients were enrolled, including 47 children receiving dialysis and 32 children who underwent renal transplant...
June 14, 2017: Nephrology
https://www.readbyqxmd.com/read/28604513/severe-sarcopenia-and-increased-fat-stores-in-pediatric-patients-with-liver-kidney-or-intestine-failure
#18
Richard S Mangus, Weston J Bush, Chandrashekhar A Kubal, Christina Miller
OBJECTIVES: Malnutrition and wasting predict clinical outcomes in children with severe chronic illness. Objectively calculated malnutrition in children with end-stage organ failure has not been well studied. This analysis compares children with kidney, liver or intestine failure to healthy controls to quantitate the disparity in muscle and fat stores. METHODS: Children younger than age 19 with end stage liver, kidney or intestine failure and with pre-transplant computed tomography (CT) imaging were selected from the transplant database...
June 9, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28597060/-early-bilateral-nephrectomy-in-neonatal-autosomal-recessive-polycystic-kidney-disease-improved-prognosis-or-unnecessary-effort
#19
REVIEW
S Riechardt, M Koch, J Oh, M Fisch
BACKGROUND: Neonatal autosomal recessive polycystic kidney disease (ARPKD) is associated with giant kidneys, lung hypoplasia, pulmonal hypertension, and end-stage renal failure. Depending on the study, mortality is reported to range between 20 and 80%. OBJECTIVES: Does bilateral nephrectomy improve survival? PATIENTS AND METHODS: Between 2010 and 2016, we treated 7 children with prenatally diagnosed ARPKD. All had a planned delivery by cesarean section...
July 2017: Der Urologe. Ausg. A
https://www.readbyqxmd.com/read/28573315/pediatric-renal-transplant-biopsy-with-ultrasound-guidance-the-core-essentials
#20
REVIEW
Aris Oates, Saveen Ahuja, Marsha M Lee, Andrew S Phelps, John D Mackenzie, Jesse L Courtier
This review provides a comprehensive and practical approach to pediatric percutaneous renal transplant biopsies, highlighting techniques and strategies to optimize adequate sample yield and ensure patient safety. In children with end-stage renal disease, transplantation is the preferred choice of therapy, providing for overall lower long-term morbidity and mortality compared with dialysis. In the ongoing management of renal transplant patients, core tissue sampling via a percutaneous renal biopsy remains the gold standard when transplant dysfunction is suspected...
June 1, 2017: Pediatric Radiology
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