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https://www.readbyqxmd.com/read/29161808/renal-outcome-of-congenital-anomalies-of-the-kidney-and-urinary-tract-system-a-singe-center-retrospective-study
#1
Rahime Renda
BACKGROUND: Congenital anomalies of the kidney and urinary tract (CAKUT) have been defined in 20 to 50% of all fetal anomalies. The aim of this study was to determine the demographic and clinical characteristics of such patients, as well as to describe the outcome and risk factors that affect the prognosis. METHODS: The analysis investigated retrospective data from 303 patients diagnosed with CAKUT between January 2015 and April 2017. Demographic data, clinical history, diagnosis, investigations, disease outcomes, and treatment procedures were evaluated...
November 21, 2017: Minerva Urologica e Nefrologica, the Italian Journal of Urology and Nephrology
https://www.readbyqxmd.com/read/29154793/trends-in-urologic-indications-for-pediatric-renal-transplantation-over-a-27-year-period-unos-database
#2
Pankaj Dangle, Utsav Bansal, Rajeev Chaudhry, Glenn M Cannon, Francis X Schneck, Michael C Ost
OBJECTIVE: To elucidate the trends in urological causes for renal transplant in the pediatric population using a national database. Little is known about the specific pediatric urologic conditions and resultant trends that precede renal transplantation. MATERIAL AND METHODS: We reviewed the United Network for Organ Sharing (UNOS) database for pediatric patients (<18 years old) who underwent renal transplantation from January 1988- September 2015. We included those patients who received a renal transplant due to a urologic condition...
November 14, 2017: Urology
https://www.readbyqxmd.com/read/29151320/effects-of-whole-blood-viscosity-and-plasma-nox-on-cardiac-function-and-cerebral-blood-flow-in-children-with-chronic-kidney-disease
#3
Necla Buyan, Meltem Akçaboy, Tayfun Göktaş, Serdar Kula, Bijen Nazlıel, Nilgün Çakar, Nermin Uncu, Bülent Çelik, Deniz Erbaş
Background/aim: The aim of the study was to investigate the effects of whole blood viscosity and plasma nitric oxide on cerebral and cardiovascular risks associated with chronic kidney disease. Materials and methods: The study group consisted of 40 pediatric patients and 21 healthy control subjects. Hematologic and biochemical variables, viscosity and plasma nitric oxide levels, echocardiographic findings, and middle cerebral artery blood flow velocity were examined. Results: Viscosity values of patients were significantly lower than those of the control group...
November 13, 2017: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/29150712/unacylated-ghrelin-and-obestatin-promising-biomarkers-of-protein-energy-wasting-in-children-with-chronic-kidney-disease
#4
Alice Monzani, Michela Perrone, Flavia Prodam, Stefania Moia, Giulia Genoni, Sara Testa, Fabio Paglialonga, Anna Rapa, Gianni Bona, Giovanni Montini, Alberto Edefonti
BACKGROUND: Impairment in orexigenic/anorexigenic hormone balance may be key in the pathogenesis of protein energy wasting in children with chronic kidney disease (CKD). Measurement of ghrelin and obestatin concentrations in children with CKD would help assess the potential contribution of these hormones to uremic protein energy wasting. METHODS: This was a cross-sectional case-control study. Acylated and unacylated ghrelin and obestatin were measured in 42 children on conservative treatment (CT), 20 children on hemodialysis, 48 pediatric renal transplant (RTx) recipients and 43 controls (CTR) (mean age 11...
November 18, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29138824/genetic-mutational-testing-of-chinese-children-with-familial-hematuria-with-biopsy%C3%A2-proven-fsgs
#5
Yongzhen Li, Ying Wang, Qingnan He, Xiqiang Dang, Yan Cao, Xiaochuan Wu, Shuanghong Mo, Xiaoxie He, Zhuwen Yi
Focal segmental glomerulosclerosis (FSGS) is a pathological lesion rather than a disease, with a diverse etiology. FSGS may result from genetic and non‑genetic factors. FSGS is considered a podocyte disease due to the fact that in the majority of patients with proven‑FSGS, the lesion results from defects in the podocyte structure or function. However, FSGS does not result exclusively from podocyte‑associated genes, however also from other genes including collagen IV‑associated genes. Patients who carry the collagen type IVA3 chain (COL4A3) or COL4A4 mutations usually exhibit Alport Syndrome (AS), thin basement membrane neuropathy or familial hematuria (FH)...
November 10, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29138582/once-daily-intramuscular-amikacin-for-outpatient-treatment-of-lower-urinary-tract-infections-caused-by-extended-spectrum-%C3%AE-lactamase-producing-escherichia-coli-in-children
#6
Meltem Polat, Soner Sertan Kara
Background: The rise in community-acquired urinary tract infections (UTIs) with extended-spectrum β-lactamase (ESBL)-producing Escherichia coli strains raises the question of how to treat these infections effectively in pediatric outpatients. Amikacin has shown promising in vitro activity against ESBL-producing urinary isolates of E. coli; however, clinical data are limited. Objective: To investigate the clinical and microbiological outcomes of community-acquired lower UTIs caused by ESBL-producing E...
2017: Infection and Drug Resistance
https://www.readbyqxmd.com/read/29135146/-laparoscopic-heminephrureterectomy-for-duplex-kidney-in-children
#7
I M Kagantsov, V V Sizonov, V I Dubrov, S G Bon-Darenko, N R Akramov, O S Shmyrov, G I Kuzovleva, N V Markov, D E Sablin, R V Surov, A V Pirogov, A A Logval
INTRODUCTION: Duplication of the upper urinary tract is one of the most common congenital urological anomalies. In patients with critically decreased or lost function of one of the renal segments, heminephrureterectomy is usually the treatment of choice. Until recently, this was an open surgery; in cases of complete removal of the ureter, an additional incision in the iliac region was required. Currently, heminephrureterectomy is increasingly performed laparoscopically. We report the experience in laparoscopic heminephrureterectomy (LHNUE) in 10 clinics in Russia and Belarus...
October 2017: Urologii︠a︡
https://www.readbyqxmd.com/read/29134448/hyperammonemia-associated-with-distal-renal-tubular-acidosis-or-urinary-tract-infection-a-systematic-review
#8
Caterina M Clericetti, Gregorio P Milani, Sebastiano A G Lava, Mario G Bianchetti, Giacomo D Simonetti, Olivier Giannini
BACKGROUND: Hyperammonemia usually results from an inborn error of metabolism or from an advanced liver disease. Individual case reports suggest that both distal renal tubular acidosis and urinary tract infection may also result in hyperammonemia. METHODS: A systematic review of the literature on hyperammonemia secondary to distal renal tubular acidosis and urinary tract infection was conducted. RESULTS: We identified 39 reports on distal renal tubular acidosis or urinary tract infections in association with hyperammonemia published between 1980 and 2017...
November 13, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29132947/associations-between-weight-loss-kidney-function-decline-and-risk-of-esrd-in-the-chronic-kidney-disease-in-children-ckid-cohort-study
#9
Elaine Ku, Joel D Kopple, Charles E McCulloch, Bradley A Warady, Susan L Furth, Robert H Mak, Barbara A Grimes, Mark Mitsnefes
BACKGROUND: Anorexia and malnutrition are associated with poor outcomes in children with chronic kidney disease (CKD). STUDY DESIGN: Observational cohort study. SETTING & PARTICIPANTS: We assessed changes in body mass index (BMI) as kidney function declines and its association with risk for end-stage renal disease (ESRD) among 854 participants followed between 2005 to 2013 in the CKD in Children (CKiD) Study. PREDICTORS: Repeated measurements of estimated glomerular filtration rate (eGFR) by serum creatinine concentration in our trajectory analysis using mixed models; change in BMI z score (per year) after eGFR decreased to <35mL/min/1...
November 10, 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/29132458/-efficacy-of-analgesic-and-sedative-treatments-in-children-with-mechanical-ventilation-in-the-pediatric-intensive-care-unit
#10
Xiao-Fang Cai, Fu-Rong Zhang, Long Zhang, Ji-Min Sun, Wen-Bin Li
OBJECTIVE: To compare the efficacy and safety of different analgesic and sedative treatments in children with mechanical ventilation in the pediatric intensive care unit (PICU). METHODS: Eighty children with mechanical ventilation in the PICU who needed analgesic and sedative treatments were equally and randomly divided into midazolam group and remifentanil+midazolam group. The sedative and analgesic effects were assessed using the Ramsay Scale and the Face, Legs, Activity, Cry and Consolability (FLACC) Scale...
November 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/29129242/intestinal-failure-as-a-significant-risk-factor-for-renal-impairment-in-children
#11
Elisa Ylinen, Laura Merras-Salmio, Riikka Gunnar, Timo Jahnukainen, Mikko P Pakarinen
OBJECTIVE: Although impaired renal function has been a frequent finding among adults with intestinal failure (IF), the data on children is scarce. The aim of this study was to assess renal function in pediatric-onset IF. METHODS: Medical records of 70 patients (38 boys) with pediatric-onset IF due to either short bowel syndrome (n = 59) or primary motility disorder (n = 11) and a history of parenteral nutrition (PN) dependency for ≥1 mo were evaluated. Renal function at the most recent follow-up was studied using plasma creatinine, cystatin C, and urea concentrations and estimated glomerular filtration rate (eGFR)...
January 2018: Nutrition
https://www.readbyqxmd.com/read/29125523/the-national-spina-bifida-patient-registry-past-present-and-future
#12
Judy Thibadeau
"The National Spina Bifida Patient Registry: Past, Present, and Future" was presented at the Spina Bifida World Congress, March 17, 2017, San Diego, California. This commentary provides a summary of registry activities including the reason for development, a description of the clinic participants and their patients who are participating, analytic works and publications. Two specific efforts that are related to the work of the registry, a urologic protocol to preserve renal function for newborns and young children, and a skin breakdown prevention bundle developed and implemented in registry clinics, are highlighted...
October 20, 2017: Journal of Pediatric Rehabilitation Medicine
https://www.readbyqxmd.com/read/29115052/dose-selection-based-on-modeling-and-simulation-for-rivipansel-in-pediatric-patients-aged-6-to-11-years-with-sickle-cell-disease
#13
Brinda K Tammara, Lutz O Harnisch
This modeling and simulation exercise aimed to provide dosing recommendations for rivipansel phase 3 studies in children aged 6 to 11 years with sickle cell disease (SCD). Pharmacokinetic data from 109 patients aged 12 to 51 years who received rivipansel (2-40 mg/kg) in previous studies (3 phase 1, one phase 2) were integrated to build a 3-compartmental simulation model. Renal clearance simulations across the age range accounted for renal function development and postulated hyperfiltration in SCD. Simulated demographic distributions for the pediatric SCD population were used to predict concentration-time profiles from 3 dosing regimens, which were then compared against efficacious average steady-state concentrations observed in phase 2...
November 8, 2017: CPT: Pharmacometrics & Systems Pharmacology
https://www.readbyqxmd.com/read/29114374/prevention-of-chemotherapy-induced-nephrotoxicity-in-children-with-cancer
#14
REVIEW
Fatemeh Ghane Sharbaf, Hamid Farhangi, Farahnak Assadi
Children with cancer treated with cytotoxic drugs are frequently at risk of developing renal dysfunction. The cytotoxic drugs that are widely used for cancer treatment in children are cisplatin (CPL), ifosfamide (IFO), carboplatin, and methotrexate (MTX). Mechanisms of anticancer drug-induced renal disorders are different and include acute kidney injury (AKI), tubulointerstitial disease, vascular damage, hemolytic uremic syndrome (HUS), and intrarenal obstruction. CPL nephrotoxicity is dose-related and is often demonstrated with hypomagnesemia, hypokalemia, and impaired renal function with rising serum creatinine and blood urea nitrogen levels...
2017: International Journal of Preventive Medicine
https://www.readbyqxmd.com/read/29111613/management-of-shiga-toxin-producing-escherichia-coli-infected-children-a-multi-national-multi-specialty-survey
#15
Stephen B Freedman, Silviu Grisaru, Jianling Xie, Susan Samuel, Andrew Dixon, Amy C Plint, David Schnadower
AIM: Research has highlighted the potential role that hydration status may play in predicting outcomes in Shiga toxin-producing Escherichia coli (STEC)-infected children. Because little is known about the management of STEC-infected children in the pre-haemolytic uremic syndrome phase, we compared paediatric emergency medicine and nephrologist-stated management approaches to STEC-infected children. METHODS: Members of the Pediatric Emergency Research Canada (PERC; n = 228), the Pediatric Emergency Medicine Collaborative Research Committee (PEM CRC; n = 221) and the Canadian Association of Pediatric Nephrologists (CAPN; n = 66) were surveyed...
November 7, 2017: Journal of Paediatrics and Child Health
https://www.readbyqxmd.com/read/29100091/mutations-in-greb1l-cause-bilateral-kidney-agenesis-in-humans-and-mice
#16
Lara De Tomasi, Pierre David, Camille Humbert, Flora Silbermann, Christelle Arrondel, Frédéric Tores, Stéphane Fouquet, Audrey Desgrange, Olivier Niel, Christine Bole-Feysot, Patrick Nitschké, Joëlle Roume, Marie-Pierre Cordier, Christine Pietrement, Bertrand Isidor, Philippe Khau Van Kien, Marie Gonzales, Marie-Hélène Saint-Frison, Jelena Martinovic, Robert Novo, Juliette Piard, Christelle Cabrol, Ishwar C Verma, Ratna Puri, Hubert Journel, Jacqueline Aziza, Laurent Gavard, Marie-Hélène Said-Menthon, Laurence Heidet, Sophie Saunier, Cécile Jeanpierre
Congenital anomalies of the kidney and urinary tract (CAKUT) constitute a major cause of chronic kidney disease in children and 20% of prenatally detected anomalies. CAKUT encompass a spectrum of developmental kidney defects, including renal agenesis, hypoplasia, and cystic and non-cystic dysplasia. More than 50 genes have been reported as mutated in CAKUT-affected case subjects. However, the pathophysiological mechanisms leading to bilateral kidney agenesis (BKA) remain largely elusive. Whole-exome or targeted exome sequencing of 183 unrelated familial and/or severe CAKUT-affected case subjects, including 54 fetuses with BKA, led to the identification of 16 heterozygous variants in GREB1L (growth regulation by estrogen in breast cancer 1-like), a gene reported as a target of retinoic acid signaling...
November 2, 2017: American Journal of Human Genetics
https://www.readbyqxmd.com/read/29098007/primary-renal-lymphoma-presenting-as-end-stage-renal-disease
#17
Lavjay Butani, Jonathan Ducore
Primary renal lymphoma is a rare entity, even more so in children. Children with primary renal lymphoma present with variable clinical features such as constitutional signs and symptoms, acute kidney injury, palpable abdominal masses, and gross hematuria. Herein we report a child who presented with seemingly advanced chronic kidney disease and was eventually diagnosed with primary lymphoma. He responded well to intensive chemotherapy and recovered renal function, although he was left with some functional limitations as a consequence of his treatment regimen...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/29097267/spontaneous-neonatal-renal-vein-thromboses-should-we-treat-them-all-a-report-of-five-cases-and-a-literature-review
#18
François Niada, René Tabin, Simon Kayemba-Kay's
Renal vein thrombosis (RVT) is a rare but well-known neonatal entity for which several therapeutic modalities are reported in the literature because of the lack of consensus management guidelines. POPULATION AND METHODS: A retrospective study of the medical records of children managed between January 1990 and December 2013, and whose final diagnosis was RVT. The diagnosis was initially clinical and subsequently confirmed by the abdominal ultrasonography (AUS) and Doppler imaging if necessary...
October 9, 2017: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/29094445/kidney-transplantation-in-a-child-with-pierson-syndrome
#19
Sanem Guler, Sertac Cimen, Phillip Acott, Kathy Whelan, Michele Molinari
Congenital nephrotic syndrome is commonly associated with mutations in genes that encode podocyte and slit diaphragm proteins or the structural and regulatory proteins of the GBM. These mutations lead to the formation of dysfunctional proteins, which account for the resistance of the renal manifestations to conventional treatment methods. Consequently, patients become renal replacement therapy dependent. Mutation of the LAMB2 gene is associated with Pierson syndrome, which is an autosomal recessive disorder characterized by congenital nephrotic syndrome and ocular abnormalities...
November 1, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/29092060/evidence-for-bone-and-mineral-metabolism-alterations-in-children-with-autosomal-dominant-polycystic-kidney-disease
#20
Stéphanie De Rechter, Justine Bacchetta, Nathalie Godefroid, Laurence Dubourg, Pierre Cochat, Julie Maquet, Ann Raes, Jean De Schepper, Pieter Vermeersch, Maria Van Dyck, Elena Levtchenko, Patrick D'Haese, Pieter Evenepoel, Djalila Mekahli
Context: Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease. Hypophosphatemia was demonstrated in adult patients with preserved renal function, together with high fibroblast growth factor 23 (FGF23) and low soluble Klotho levels. The latter explained the relative FGF23 hyporesponsiveness in this cohort. Objective: Evaluating phosphate and bone mineral metabolism in children with ADPKD compared with what is known in adult ADPKD patients...
November 1, 2017: Journal of Clinical Endocrinology and Metabolism
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