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End Stage Renal Disease pediatric

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https://www.readbyqxmd.com/read/28440913/piperacillin-tazobactam-versus-cefepime-incidence-of-acute-kidney-injury-in-combination-with-vancomycin-and-tobramycin-in-pediatric-cystic-fibrosis-patients
#1
Lisa K LeCleir, Rebecca S Pettit
BACKGROUND: Cystic fibrosis (CF) patients often receive prolonged courses of broad spectrum antibiotics, such as piperacillin-tazobactam or cefepime in combination with vancomycin and tobramycin. The objective of this study was to determine the difference in AKI for pediatric CF patients receiving piperacillin-tazobactam or cefepime in combination with vancomycin and tobramycin. METHODS: IRB approval from a single CF center was obtained for this retrospective cohort study...
April 25, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28409285/urinary-calprotectin-kidney-injury-molecule-1-and-neutrophil-gelatinase-associated-lipocalin-for-the-prediction-of-adverse-outcome-in-pediatric-acute-kidney-injury
#2
Jens H Westhoff, Felix S Seibert, Sina Waldherr, Frederic Bauer, Burkhard Tönshoff, Alexander Fichtner, Timm H Westhoff
Early identification of patients with acute kidney injury (AKI) being at high risk for adverse outcome can influence medical treatment. This study compares urinary calprotectin, kidney injury molecule-1 (KIM-1), and neutrophil gelatinase-associated lipocalin (NGAL) for their performance in predicting mortality and need for renal replacement therapy (RRT) in pediatric AKI patients. Urinary biomarker concentrations were assessed prospectively in 141 subjects aged 0-18 years including 55 patients with established AKI according to pediatric Risk, Injury, Failure, Loss, and End-stage kidney disease (pRIFLE) criteria, 27 patients without AKI, and 59 healthy children...
April 14, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28405841/focal-segmental-glomerulosclerosis-and-medullary-nephrocalcinosis-in-children-with-adck4-mutations
#3
Eujin Park, Hee Gyung Kang, Young Hun Choi, Kyoung Bun Lee, Kyung Chul Moon, Hyeon Joo Jeong, Michio Nagata, Hae Il Cheong
BACKGROUND: Mutations in the AarF domain containing kinase 4 gene (ADCK4), one of the novel genes causing steroid-resistant nephrotic syndrome (SRNS), usually manifest as isolated adolescent-onset focal segmental glomerulosclerosis (FSGS). ADCK4 interacts with components of the coenzyme Q10 (CoQ10) biosynthesis pathway. METHODS: The incidence and phenotypes of patients with ADCK4 mutations were investigated in a cohort of Korean pediatric patients with SRNS. RESULTS: Among the 53 patients enrolled in the study the incidence of ADCK4-associated FSGS was 7...
April 12, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28398236/genetics-of-congenital-anomalies-of-the-kidney-and-urinary-tract-the-current-state-of-play
#4
REVIEW
Valentina P Capone, William Morello, Francesca Taroni, Giovanni Montini
Congenital anomalies of the kidney and urinary tract (CAKUT) are the most frequent form of malformation at birth and represent the cause of 40-50% of pediatric and 7% of adult end-stage renal disease worldwide. The pathogenesis of CAKUT is based on the disturbance of normal nephrogenesis, secondary to environmental and genetic causes. Often CAKUT is the first clinical manifestation of a complex systemic disease, so an early molecular diagnosis can help the physician identify other subtle clinical manifestations, significantly affecting the management and prognosis of patients...
April 11, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28396942/frequent-hemodialysis-history-of-the-modality-and-assessment-of-outcomes
#5
Douglas M Silverstein
About 660,000 individuals have end-stage renal disease in the USA, the vast majority of whom are receiving standard, in-center, thrice-weekly hemodialysis (HD). The morbidity and mortality among patients receiving standard HD remain unacceptably high. Studies conducted over the past two decades have provided a substantial amount of information on the advantages and drawbacks of providing more frequent HD treatment, either in-center or at home. In this article I summarize these studies, focusing special attention on the randomized, cross-over study assessing outcomes in children who received either frequent, in-center HD or traditional, thrice-weekly, in-center HD performed by Laskin et al...
April 11, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28364132/challenges-in-establishing-genotype-phenotype-correlations-in-arpkd-case-report-on-a-toddler-with-two-severe-pkhd1-mutations
#6
Kathrin Ebner, Claudia Dafinger, Nadina Ortiz-Bruechle, Friederike Koerber, Bernhard Schermer, Thomas Benzing, Jörg Dötsch, Klaus Zerres, Lutz Thorsten Weber, Bodo B Beck, Max Christoph Liebau
BACKGROUND: Autosomal recessive polycystic kidney disease (ARPKD) constitutes an important cause of pediatric end stage renal disease and is characterized by a broad phenotypic variability. The disease is caused by mutations in a single gene, Polycystic Kidney and Hepatic Disease 1 (PKHD1), which encodes a large transmembrane protein of poorly understood function called fibrocystin. Based on current knowledge of genotype-phenotype correlations in ARPKD, two truncating mutations are considered to result in a severe phenotype with peri- or neonatal mortality...
March 31, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28261895/bilateral-native-nephrectomy-reduces-systemic-oxalate-level-after-combined-liver-kidney-transplant-a-case-report
#7
Vincenzo Villani, Neena Gupta, Nahel Elias, Parsia A Vagefi, James F Markmann, Elahna Paul, Avram Z Traum, Heidi Yeh
Primary hyperoxaluria type 1 (PH1) is a rare liver enzymatic defect that causes overproduction of plasma oxalate. Accumulation of oxalate in the kidney and subsequent renal failure are fatal to PH1 patients often in pediatric age. Combined liver and kidney transplantation is the therapy of choice for end-stage renal disease due to PH1. Levels of plasma oxalate remain elevated for several months after liver transplantation, as the residual body oxalate is slowly excreted. Patients with persistent hyperoxaluria after transplant often require hemodialysis, and accumulation of residual oxalate in the kidney can induce graft dysfunction...
May 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28260481/incidence-and-patient-outcomes-in-renal-replacement-therapy-after-orthotopic-liver-transplant
#8
Asude Ayhan, Zeynep Ersoy, Aydin Ulas, Pinar Zeyneloglu, Arash Pirat, Mehmet Haberal
OBJECTIVES: Our objective was to evaluate the incidence of renal replacement therapy after orthotopic liver transplant and to evaluate and analyze patient outcomes. MATERIALS AND METHODS: We performed a retrospective analysis of 177 consecutive patients at a tertiary care unit who underwent orthotopic liver transplant between January 2010 and June 2016. Patients who were admitted to the intensive care unit after orthotopic liver transplant and who required renal replacement therapy were included...
February 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28260445/renal-impairment-and-complication-after-kidney-transplant-at-queen-rania-abdulla-children-s-hospital
#9
Reham Issa Almardini, Ghazi Mohamad Salita, Mahdi Qasem Farah, Issa Ahmad Katatbeh, Katibh Al-Rabadi
OBJECTIVES: Kidney transplant is the treatment of choice for end-stage renal disease, but it is not without complications. We review the medical cause of significant renal impairment and complications that developed after kidney transplant in pediatric patients who required hospital admission and intervention and/or who were followed between 2007 and 2016. MATERIALS AND METHODS: A retrospective noninterventional chart review study was conducted in pediatric patients who received a kidney transplant and/or followed at the nephrology clinic at Queen Rania Abdulla Children's Hospital between 2007 and 2016...
February 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28254584/outcomes-and-risk-factors-for-graft-loss-lessons-learned-from-1-056-pediatric-kidney-transplants-at-the-university-of-minnesota
#10
Srinath Chinnakotla, Priya Verghese, Blanche Chavers, Michelle N Rheault, Varvara Kirchner, Ty Dunn, Clifford Kashtan, Thomas Nevins, Michael Mauer, Timothy Pruett
BACKGROUND: Advances in immunosuppression, surgical techniques, and management of infections in children receiving kidney transplants have impacted outcomes. STUDY DESIGN: We analyzed a prospectively maintained database of pediatric kidney transplants. RESULTS: From June 1963 through October 2016, we performed 1,056 pediatric kidney transplants. Of these 129 were < 2 years old. The most common indications for transplant were congenital anomalies (dysplastic kidneys), obstructive uropathy and congenital nephrotic syndrome...
January 11, 2017: Journal of the American College of Surgeons
https://www.readbyqxmd.com/read/28213931/successful-resumption-of-peritoneal-dialysis-following-living-donor-liver-transplantation-in-children-with-end-stage-renal-disease
#11
Hiroyuki Kanazawa, Akinari Fukuda, Mai Sato, Shingo Ishimori, Kengo Sasaki, Hajime Uchida, Takanobu Shigeta, Vidyadhar Padmakar Mali, Seisuke Sakamoto, Kenji Ishikura, Mureo Kasahara
Children with ESRD in need of RRT are commonly managed by PD due to difficulty with vascular access for HD and the relatively large extracorporeal blood volume required. Major abdominal surgery may result in injury to the peritoneum and consequent adhesion, thereby resulting in a reduction in the anatomical capacity and transport capability across the peritoneal membrane. Here, we report successful resumption of PD after LDLT in two pediatric patients. The causes of ESRD were PH1 and juvenile nephronophthisis, respectively...
May 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28133935/post-transplant-immune-complex-nephritis-in-a-patient-with-systemic-lupus-erythematosus-associated-with-anca-vasculitis
#12
Carlos Sanchez, Alejandra Rebolledo, Junior Gahona, Mauricio Rojas, Raquel Jiménez, Aurora Bojórquez
Nearly 20% of SLE corresponds to the pediatric population, and 75% of them have kidney involvement representing an important etiology of chronic kidney disease. A correlation between SLE and ANCA-associated vasculitis has been identified as an overlapping syndrome. Kidney allograft recurrence is rare in SLE when disease control is achieved and with nowadays immunosuppression treatment. Histologic transformation is unusual, especially when there are negative serologic markers and no immune complex deposition reported in native kidneys...
May 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28133685/impact-of-acute-kidney-injury-on-long-term-mortality-and-progression-to-chronic-kidney-disease-among-critically-ill-children
#13
Najlaa G Al-Otaibi, Maryam Zeinelabdin, Mohamed A Shalaby, Norah Khathlan, Ghadi D Mashat, Amal A Zahrani, Sundus Mw NoorSaeed, Nora M Shalabi, Khalid A Alhasan, Sara N Sharief, Amr S Albanna, Jameela A Kari
To determine the 2-year outcome of acute kidney injury (AKI) following admission to pediatric critical care units (PICU). Methods: A retrospective cohort study was conducted between January 2012 and December 2013. We followed 131 children admitted to PICU, King Abdulaziz University Hospital, Jeddah, Kingdom of Saudi Arabia with a diagnosis of AKI, based on pRIFLE (pediatric risk, injury, failure, loss, and end-stage renal disease), for 2 years. During the study period, 46 children died and 38 of survivors completed the follow-up...
February 2017: Saudi Medical Journal
https://www.readbyqxmd.com/read/28130969/the-oxford-classification-predictors-of-chronic-kidney-disease-in-pediatric-patients-with-iga-nephropathy
#14
Rafaela C G Fabiano, Stanley A Araújo, Eduardo A Bambirra, Eduardo A Oliveira, Ana Cristina Simões E Silva, Sérgio V B Pinheiro
OBJECTIVE: The Oxford Classification for Immunoglobulin A nephropathy (IgAN) identifies pathological variables that may predict the decline of renal function. This study aimed to evaluate the Oxford Classification variables as predictors of renal dysfunction in a cohort of Brazilian children and adolescents with IgAN. METHODS: A total of 54 patients with IgAN biopsied from 1982 to 2010 were assessed. Biopsies were re-evaluated and classified according to the Oxford Classification...
January 27, 2017: Jornal de Pediatria
https://www.readbyqxmd.com/read/28124483/mutations-in-ttc21b-cause-different-phenotypes-in-two-childhood-cases-in-china
#15
Hongwen Zhang, Baige Su, Xiaoyu Liu, Huijie Xiao, Jie Ding, Yong Yao
AIM: The TTC21B gene is now known as causative of nephronophthisis-related ciliopathies (NPHP-RC). We reported two Chinese pediatric cases with end-stage renal disease and other phenotypes caused by the TTC21B gene mutations. METHODS: The clinical features of Chinese pediatric cases with NPHP-RC were summarized. Mutation analysis of the TTC21B gene was performed using next-generation sequencing. RESULTS: The two cases both had nephrotic proteinuria, renal failure, hypertension and abnormal liver function (or hepatic fibrosis)...
January 26, 2017: Nephrology
https://www.readbyqxmd.com/read/28117080/genomic-and-clinical-profiling-of-a-national-nephrotic-syndrome-cohort-advocates-a-precision-medicine-approach-to-disease-management
#16
Agnieszka Bierzynska, Hugh J McCarthy, Katrina Soderquest, Ethan S Sen, Elizabeth Colby, Wen Y Ding, Marwa M Nabhan, Larissa Kerecuk, Shivram Hegde, David Hughes, Stephen Marks, Sally Feather, Caroline Jones, Nicholas J A Webb, Milos Ognjanovic, Martin Christian, Rodney D Gilbert, Manish D Sinha, Graham M Lord, Michael Simpson, Ania B Koziell, Gavin I Welsh, Moin A Saleem
Steroid Resistant Nephrotic Syndrome (SRNS) in children and young adults has differing etiologies with monogenic disease accounting for 2.9-30% in selected series. Using whole exome sequencing we sought to stratify a national population of children with SRNS into monogenic and non-monogenic forms, and further define those groups by detailed phenotypic analysis. Pediatric patients with SRNS were identified via a national United Kingdom Renal Registry. Whole exome sequencing was performed on 187 patients, of which 12% have a positive family history with a focus on the 53 genes currently known to be associated with nephrotic syndrome...
April 2017: Kidney International
https://www.readbyqxmd.com/read/28110812/effect-of-omega-3-fatty-acids-on-serum-lipid-profile-and-oxidative-stress-in-pediatric-patients-on-regular-hemodialysis-a-randomized-placebo-controlled-study
#17
Areej Mohamed Ateya, Nagwa Ali Sabri, Ihab El Hakim, Sara M Shaheen
OBJECTIVE: We sought to evaluate the effects of omega-3 fatty acids supplementation on serum lipid profile and oxidative stress markers in pediatric patients with end-stage renal disease on regular hemodialysis (HD). DESIGN: This study was a double-blinded, randomized, placebo-controlled trial conducted on 49 pediatric patients on regular HD for at least 6 months. INTERVENTION: Patients were randomly divided into either omega-3 group (n = 25) who received 1-g oral omega-3 capsule once daily for 16 weeks or placebo group (n = 24) who received 1-g matching oral placebo capsule once daily for 16 weeks...
May 2017: Journal of Renal Nutrition
https://www.readbyqxmd.com/read/28087478/renal-involvement-in-lysinuric-protein-intolerance-contribution-of-pathology-to-assessment-of-heterogeneity-of-renal-lesions
#18
Emmanuel Estève, Pauline Krug, Aurélie Hummel, Jean-Baptiste Arnoux, Olivia Boyer, Anais Brassier, Pascale de Lonlay, Vincent Vuiblet, Stéphanie Gobin, Rémi Salomon, Christine Piètrement, Jean-Paul Bonnefont, Aude Servais, Louise Galmiche
Lysinuric protein intolerance (LPI) is a rare autosomal recessive disease caused by mutations in the SLC7A7 gene encoding the light subunit of a cationic amino acid transporter. Symptoms mimic primary urea cycle defects but dysimmune symptoms are also described. Renal involvement in LPI was first described in the 1980s. In 2007, it appeared that it could concern as much as 75% of LPI patients and could lead to end-stage renal disease. The most common feature is proximal tubular dysfunction and nephrocalcinosis but glomerular lesions are also reported...
April 2017: Human Pathology
https://www.readbyqxmd.com/read/28060690/-peritoneal-dialysis-in-the-first-two-years-of-life-experience-of-a-nephrology-and-renal-transplantation-pediatric-unit
#19
Sofia Deuchande, Tânia Mano, Cristina Novais, Rute Machado, Rosário Stone, Margarida Almeida
INTRODUCTION: Peritoneal dialysis is the dialytic method of choice in chronic end-stage renal disease in children. This study main purpose was to characterize the long-term survival of a pediatric population who began peritoneal dialysis within the first two years of life. MATERIAL AND METHODS: A descriptive and retrospective study was performed in a portuguese nephrology and renal transplantation pediatric unit, between January 1991 and August 2014. End-stage renal disease etiology, mortality, comorbidities and complications of peritoneal dialysis and end-stage renal disease, growth and psychomotor development were evaluated...
September 2016: Acta Médica Portuguesa
https://www.readbyqxmd.com/read/28035470/the-clinical-and-laboratory-features-of-chinese-han-anti-factor-h-autoantibody-associated-hemolytic-uremic-syndrome
#20
Di Song, Xiao-Rong Liu, Zhi Chen, Hui-Jie Xiao, Jie Ding, Shu-Zhen Sun, Hong-Yan Liu, Wei-Yi Guo, Su-Xia Wang, Feng Yu, Ming-Hui Zhao
BACKGROUND: Anti-complement factor H (CFH) autoantibody-associated hemolytic uremic syndrome (HUS) is a severe sub-type of HUS. METHODS: We assessed the clinical and renal pathological features, circulating complement levels, and genetic background of Chinese pediatric patients with this sub-type of HUS. Thirty-three consecutive patients with acute kidney injury who tested positive for serum anti-CFH autoantibodies were enrolled in this study. RESULTS: All of the eight patients who underwent renal biopsies presented with changes typical of thrombotic microangiopathy, especially changes in chronic characteristics...
December 29, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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