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https://www.readbyqxmd.com/read/28210838/urinary-ngal-deficiency-in-recurrent-urinary-tract-infections
#1
Catherine S Forster, Kathryn Johnson, Viral Patel, Rebecca Wax, Nancy Rodig, Jonathan Barasch, Richard Bachur, Richard S Lee
INTRODUCTION: Children with recurrent urinary tract infections (rUTI) often show no identifiable cause of their infections. Neutrophil gelatinase-associated lipocalin (NGAL) is known to be upregulated within the uroepithelium and kidney of patients with UTI and exhibits a localized bacteriostatic effect through iron chelation. We hypothesize that some patients with rUTI without an identifiable cause of their recurrent infections have locally deficient NGAL production. We therefore explored whether a lack of NGAL production may be a factor in the pathogenesis of rUTI...
February 16, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28202121/-oliguria-and-acute-renal-dysfunction-in-a-six-month-old-infant
#2
Ya-Jie Cui, Chun-Lan Song, Yi-Bing Cheng
The infant (a girl aged 6 months) was admitted to the hospital because of oliguria and acute renal dysfunction. The laboratory examination results showed serious metabolic acidosis and increased blood urea nitrogen and serum creatinine levels. The patient continued to be anuric after 10 days of treatment with continuous renal replacement therapy (CRRT). she died a day later. The family history showed that the patient's sister died of acute renal failure 6 months after birth. The genomic sequencing results showed AGXT mutation in the patient and confirmed the diagnosis of primary hyperoxaluria type 1 (PH1)...
February 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28202119/-acute-renal-failure-caused-by-rhabdomyolysis-in-children-a-clinical-analysis-of-26-cases
#3
Fan-Ying Meng, Xiao-Rong Liu, Qian Fu
OBJECTIVE: To investigate the clinical features and prognosis of acute renal failure (ARF) caused by rhabdomyolysis (RM) in children. METHODS: A retrospective analysis was performed for the clinical data, laboratory examination, and prognosis of 26 RM children with ARF. RESULTS: The causes for all 26 RM children with ARF were non-traumatic diseases, and the three most common causes were infection (69%), diabetes (12%), and metabolic disease (8%)...
February 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28199756/factors-associated-with-reduction-of-left-ventricular-mass-in-children-on-peritoneal-dialysis
#4
Jeong Jin Yu, Hyun Ok Jun, Eun Jung Shin, Jae Suk Baek, Joo Hoon Lee, Young-Hwue Kim, Young Seo Park, Jae-Kon Ko
AIM: This study aimed to investigate sensitive factors involved in left ventricular mass reduction in children with end-stage renal disease (ESRD) undergoing peritoneal dialysis. METHODS: Thirty-five subjects on peritoneal dialysis were enrolled. Two successive echocardiographic and clinical data for each subject were obtained. Blood pressure and left ventricular mass index (LVMI) were indexed through a division with the normal 95(th) percentile value. Differences in numeric data between two datasets were calculated...
February 15, 2017: Nephrology
https://www.readbyqxmd.com/read/28188683/functional-kras-mutations-and-a-potential-role-for-pi3k-akt-activation-in-wilms-tumors
#5
Dina Polosukhina, Harold D Love, Hernan Correa, Zengliu Su, Kimberly B Dahlman, William Pao, Harold L Moses, Carlos L Arteaga, Harold N Lovvorn, Roy Zent, Peter E Clark
Wilms tumor (WT) is the most common renal neoplasm of childhood and affects 1 in 10,000 children aged less than 15 years. These embryonal tumors are thought to arise from primitive nephrogenic rests that derive from the metanephric mesenchyme during kidney development and are characterized partly by increased Wnt/β-catenin signaling. We previously showed that coordinate activation of Ras and β-catenin accelerates the growth and metastatic progression of a murine WT model. Here, we show that activating KRAS mutations can be found in human WT...
February 11, 2017: Molecular Oncology
https://www.readbyqxmd.com/read/28188436/clinical-and-molecular-aspects-of-distal-renal-tubular-acidosis-in-children
#6
Martine T P Besouw, Marc Bienias, Patrick Walsh, Robert Kleta, William G Van't Hoff, Emma Ashton, Lucy Jenkins, Detlef Bockenhauer
BACKGROUND: Distal renal tubular acidosis (dRTA) is characterized by hyperchloraemic metabolic acidosis, hypokalaemia, hypercalciuria and nephrocalcinosis. It is due to reduced urinary acidification by the α-intercalated cells in the collecting duct and can be caused by mutations in genes that encode subunits of the vacuolar H(+)-ATPase (ATP6V1B1, ATP6V0A4) or the anion exchanger 1 (SLC4A1). Treatment with alkali is the mainstay of therapy. METHODS: This study is an analysis of clinical data from a long-term follow-up of 24 children with dRTA in a single centre, including a genetic analysis...
February 10, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28181337/deficiency-of-the-sphingosine-1-phosphate-lyase-sgpl1-is-associated-with-congenital-nephrotic-syndrome-and-congenital-adrenal-calcifications
#7
Andreas R Janecke, Ruijuan Xu, Elisabeth Steichen-Gersdorf, Siegfried Waldegger, Andreas Entenmann, Thomas Giner, Iris Krainer, Lukas A Huber, Michael W Hess, Yaacov Frishberg, Hila Barash, Shay Tzur, Nira Schreyer-Shafir, Rivka Sukenik-Halevy, Tania Zehavi, Annick Raas-Rothschild, Cungui Mao, Thomas Müller
We identified two unrelated consanguineous families with three children affected by the rare association of congenital nephrotic syndrome diagnosed in the first days of life, of hypogonadism, and of prenatally detected adrenal calcifications, associated with congenital adrenal insufficiency in one case. Using exome sequencing and targeted Sanger sequencing two homozygous truncating mutations, c.1513C>T (p.Arg505*) and c.934delC (p.Leu312Phefs*30), were identified in SGPL1 encoding sphingosine-1-phosphate lyase 1...
February 8, 2017: Human Mutation
https://www.readbyqxmd.com/read/28180952/endothelial-dysfunction-during-long-term-follow-up-in-children-with-stec-hemolytic-uremic-syndrome
#8
Martin Kreuzer, Laura Sollmann, Stephan Ruben, Maren Leifheit-Nestler, Dagmar-Christiane Fischer, Lars Pape, Dieter Haffner
BACKGROUND: Shiga-toxin-producing Escherichia coli (STEC)-associated hemolytic-uremic syndrome (HUS) is a major cause of acute kidney injury (AKI), especially in children. Its long-term outcome with respect to endothelial damage remains largely elusive. METHODS: This was a cross-sectional study in 26 children who had suffered from STEC-HUS in the past and achieved a complete recovery of renal function (eGFR >90 ml/min/1.73 m(2)). Skin microcirculation after local heating was assessed by laser Doppler fluximetry, carotid-femoral pulse wave velocity (PWV), carotid intima media thickness (cIMT), 24-h ambulatory blood pressure, and angiopoietin (Ang) 1 and 2 serum levels after a median follow-up period of 6...
February 8, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28177130/a-population-pharmacokinetic-model-of-at9283-in-adults-and-children-to-predict-the-maximum-tolerated-dose-in-children-with-leukaemia
#9
Janna K Duong, Melanie J Griffin, Darren Hargrave, Josef Vormoor, David Edwards, Alan V Boddy
AIMS: AT9283 is used to treat patients with solid tumors and patients with leukaemia. However, the maximum tolerated dose (MTD) for children with leukaemia remains unknown due to early termination of the Phase I trial. The aim of this study was to develop a population model of AT9283 to describe the pharmacokinetics in adults and children and to estimate the MTD in children with leukaemia. METHODS: Data from Phase I dose-escalation studies in adults and children were used to build a population pharmacokinetic model (NONMEM v7...
February 8, 2017: British Journal of Clinical Pharmacology
https://www.readbyqxmd.com/read/28169459/long-term-effectiveness-and-complication-rates-of-bladder-augmentation-in-patients-with-neurogenic-bladder-dysfunction-a-systematic-review
#10
Lisette 't Hoen, Hazel Ecclestone, Bertil F M Blok, Gilles Karsenty, Véronique Phé, Romain Bossier, Jan Groen, David Castro-Diaz, Bárbara Padilla Fernández, Giulio Del Popolo, Stefania Musco, Jürgen Pannek, Thomas M Kessler, Tobias Gross, Marc P Schneider, Rizwan Hamid
AIMS: To systematically evaluate effectiveness and safety of bladder augmentation for adult neuro-urological patients. METHODS: The Preferred Reporting Items for Systematic Review and Meta-analysis (PRISMA) statement was followed for review of publications. The Medline, Embase, and Cochrane controlled trial databases and clinicaltrial.gov were searched until January 2015. No limitations were placed on date or language. Non-original articles, conference abstracts, and publications involving children and animals were excluded...
February 7, 2017: Neurourology and Urodynamics
https://www.readbyqxmd.com/read/28169124/-therapeutic-mega-ureter-primitive-before-one-year-of-life-retrospective-study-of-20years
#11
B Picart, M Pons, A Line, C François, M-L Poli Merol
INTRODUCTION: What is the proper way to manage complicated primary mega-ureter in infants under the age of one. This has already been discussed in the literature but the controversy remains. OBJECTIVE: Evaluate the long-term results of the management of mega-ureter based support under the age of one. MATERIAL AND METHODS: Single-center retrospective study from 1990 to 2010. All children under one year found were evaluated including clinical examination, ultrasound, scintigraphy and cystography...
February 3, 2017: Progrès en Urologie
https://www.readbyqxmd.com/read/28168731/statistical-methodology-for-estimating-the-mean-difference-in-a-meta-analysis-without-study-specific-variance-information
#12
Patarawan Sangnawakij, Dankmar Böhning, Stephen Adams, Michael Stanton, Heinz Holling
Statistical inference for analyzing the results from several independent studies on the same quantity of interest has been investigated frequently in recent decades. Typically, any meta-analytic inference requires that the quantity of interest is available from each study together with an estimate of its variability. The current work is motivated by a meta-analysis on comparing two treatments (thoracoscopic and open) of congenital lung malformations in young children. Quantities of interest include continuous end-points such as length of operation or number of chest tube days...
February 6, 2017: Statistics in Medicine
https://www.readbyqxmd.com/read/28164508/urinary-neutrophil-gelatinase-associated-lipocalin-does-not-distinguish-acute-rejection-from-other-causes-of-acute-kidney-injury-in-pediatric-renal-transplant-recipients
#13
Tomas Seeman, Karel Vondrak, Jiri Dusek, Nadezda Simankova, Jakub Zieg, Jaromir Hacek, Maria Chadimova, Bruno Sopko, Magdalena Fortova
BACKGROUND: The aim of this prospective single center study was to investigate the ability of urinary neutrophil gelatinase-associated lipocalin (NGAL) to distinguish acute rejection from other causes of acute kidney injury (AKI) in children after renal transplantation. METHODS: Fifteen children fulfilled the inclusion criteria (acute kidney injury (AKI) with allograft biopsy, at least 21 days after renal transplantation, no sepsis) during 2013 - 2014 in our pediatric transplantation center...
January 1, 2017: Clinical Laboratory
https://www.readbyqxmd.com/read/28161229/-secondary-megaureter-a-rare-complication-of-deflux-%C3%A2-endoscopic-management-of-vesicoureteral-reflux-in-children
#14
N Boudaoud, A Line, M Pons, F Lefebvre, M A Bouche Pillon, C Francois, M L Poli Merol
Endoscopic management is the gold standard for symptomatic low-grade vesicoureteral reflux (VUR) in children. Deflux(®) (hyaluronic acid/dextranomer) injection is highly effective and has very few complications. We report on two cases of secondary megaureter after Deflux(®) injections. In the first case, a boy presented with Grade 4 VUR. He received a bilateral Deflux(®) injection with a total of three syringes. The postoperative ultrasound was normal. However, a check-up ultrasound 3 years later showed a significant ureteropyelocalyceal dilatation, with stasis and decreased renal function on scintigraphy, the reason why antireflux surgery (Cohen procedure) was performed...
February 1, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28159657/dose-optimisation-of-voriconazole-with-therapeutic-drug-monitoring-in-children-a-single-centre-experience-in-china
#15
Liang Liu, Xing Zhou, Tingting Wu, Hongliang Jiang, Sitao Yang, Yang Zhang
The pharmacokinetic profile of voriconazole is highly variable, rendering inconsistent and/or inadequate dosing, especially in children <2 years old. A retrospective analysis was performed in children receiving voriconazole with at least one plasma trough level (Ctrough) monitored. Statistical analyses were performed to examine the dose-exposure relationship as well as other factors potentially affecting voriconazole Ctrough in different age children. A total of 107 paediatric patients were included, of whom 75 were <2 years old...
January 31, 2017: International Journal of Antimicrobial Agents
https://www.readbyqxmd.com/read/28133953/successful-renal-transplantation-in-small-children-with-a-completely-thrombosed-inferior-vena-cava
#16
P Verghese, E Minja, V Kirchner, B Chavers, A Matas, S Chinnakotla
In small children with end-stage renal disease, an adult-size kidney transplant is the best option. However, in the face of a completely thrombosed inferior vena cava (IVC), such transplants can be challenging, given the difficulty of achieving adequate renal venous outflow and the risk of graft thrombosis. Using a new technique to anastomose the renal vein to the right hepatic vein/IVC junction, we successfully implanted an adult-size graft in 2 small children (9.8 kg and 14 kg) who had end-stage renal disease and a completely thrombosed IVC...
January 30, 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/28130969/the-oxford-classification-predictors-of-chronic-kidney-disease-in-pediatric-patients-with-iga-nephropathy
#17
Rafaela C G Fabiano, Stanley A Araújo, Eduardo A Bambirra, Eduardo A Oliveira, Ana Cristina Simõese 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 25, 2017: Jornal de Pediatria
https://www.readbyqxmd.com/read/28127175/management-of-bilateral-wilms-tumor-our-experience
#18
Raghunath Bv, Vinay Jadhav, Gowri Shankar, Narendrababu M, Ramesh S
Management of bilateral Wilms' tumor is particularly challenging, considering the chances of recurrence and long-term renal function for affected patients. Aggressive surgical resection to prevent recurrence must be balanced with the desire to preserve renal function. We evaluated our experience in the management of bilateral Wilms' tumor stressing the challenges encountered in decision making and the role of nephron sparing surgery. We had four children presenting with bilateral Wilms' tumor. All of them were appropriately staged and given standard chemotherapy as per NWTS-5 guidelines...
March 2017: Indian Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28124539/one-staged-reconstruction-of-bladder-exstrophy-in-male-patients-long-term-follow-up-outcomes
#19
Amilcar Martins Giron, Marcos Figueiredo Mello, Paulo Afonso Carvalho, Paulo Renato Marcelo Moscardi, Roberto Iglesias Lopes, Miguel Srougi
INTRODUCTION: The surgical correction of bladder exstrophy remains challenging. In our institution, the repair has evolved from a staged repair to one-stage reconstruction. The one-stage reconstruction includes; bladder closure, Cantwell-Ransley neourethroplasty and abdominoplasty using groin flaps, without the need of pelvic ostheotomies. Repair of urinary continence (UC) and vesicoureteral reflux (VUR) is done after development of the infant. OBJECTIVE: To present our experience of our modified one-stage reconstruction of bladder exstrophy in male patients...
January 2017: International Braz J Urol: Official Journal of the Brazilian Society of Urology
https://www.readbyqxmd.com/read/28121970/resorbable-mesh-cranioplasty-repair-of-bilateral-cerebrospinal-fluid-leaks-following-pediatric-simultaneous-bilateral-auditory-brainstem-implant-surgery
#20
Giacomo Colletti, Marco Mandalà, Vittorio Colletti, Alberto Deganello, Fabiana Allevi, Liliana Colletti
OBJECTIVE: To present a child with cochlear nerve deficiency (CND) who received simultaneous bilateral simultaneous auditory brainstem implants (BS-ABI) and subsequently presented with bilateral cerebrospinal fluid (CSF) leaks unresponsive to standard treatments. To propose a novel rigid retrosigmoid cranioplasty for treating and preventing CSF leaks in children at high risk for this complication. PATIENT: A 3.5-year-old child with CND, vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities, coloboma, heart defect, atresia choanae, retarded growth and development, genital abnormality, and ear abnormality, Arnold Chiari malformation, previous treated tracheo-esophageal fistula underwent BS-ABI...
January 24, 2017: Otology & Neurotology
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