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Chronic Kidney Disease in Children

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https://www.readbyqxmd.com/read/29146214/-treatments-of-steroid-dependent-nephrotic-syndrome-in-children
#1
A Couderc, E Bérard, V Guigonis, I Vrillon, J Hogan, V Audard, V Baudouin, C Dossier, O Boyer
Primary nephrotic syndrome (NS) is the most common glomerular disease in children. It is characterized by massive proteinuria and hypoalbuminemia. It typically has a sudden onset and more than 70% of patients will experience at least one relapse. An immunological origin has long been postulated, although the precise molecular mechanisms underlying the disease remain debated. Steroids are the first-line therapy with cumulative dose and duration of initial treatment varying among countries. Steroid-sparing agents may be indicated in case of steroid-dependency or frequent relapses...
November 13, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/29142939/negative-staining-for-col4a5-correlates-with-worse-prognosis-and-more-severe-ultrastructural-alterations-in-males-with-alport-syndrome
#2
Samar M Said, Mary E Fidler, Anthony M Valeri, Brooke McCann, Wade Fiedler, Lynn D Cornell, Mariam Priya Alexander, Ahmed M Alkhunaizi, Anne Sullivan, Carl H Cramer, Marie C Hogan, Samih H Nasr
Introduction: Alport syndrome (AS) is a genetic disorder characterized by progressive hematuric nephropathy with or without sensorineural hearing loss and ocular lesions. Previous studies on AS included mostly children. Methods: To determine the prognostic value of loss of staining for collagen type IV alpha 5 (COL4A5) and its relationship with the ultrastructural glomerular basement membrane alterations, we performed direct immunofluorescence using a mixture of fluorescein isothiocyanate-conjugated and Texas-red conjugated antibodies against COL4A5 and COL4A2, respectively, on renal biopsies of 25 males with AS (including 16 who were diagnosed in adulthood)...
January 2017: KI Reports
https://www.readbyqxmd.com/read/29138824/genetic-mutational-testing-of-chinese-children-with-familial-hematuria-with-biopsy%C3%A2-proven-fsgs
#3
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/29136192/assessing-the-hydration-status-of-children-with-chronic-kidney-disease-and-on-dialysis-a-comparison-of-techniques
#4
Caroline S Y Eng, Devina Bhowruth, Mark Mayes, Lynsey Stronach, Michelle Blaauw, Amy Barber, Lesley Rees, Rukshana C Shroff
Background: Fluid balance is pivotal in the management of children with chronic kidney disease (CKD) and on dialysis. Although many techniques are available to assess fluid status, there are only a few studies for children, of which none have been comparable against cardiovascular outcome measures. Methods: We performed a longitudinal study in 30 children with CKD5-5D and 13 age-matched healthy controls (71 measurements) to determine a correlation between optimal weight by bioimpedance spectroscopy (Wt-BIS) and clinical assessment (Wt-CA)...
November 9, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/29134449/iohexol-plasma-clearance-in-children-validation-of-multiple-formulas-and-single-point-sampling-times
#5
Camilla Tøndel, Cathrin Lytomt Salvador, Karl Ove Hufthammer, Bjørn Bolann, Damien Brackman, Anna Bjerre, Einar Svarstad, Atle Brun
BACKGROUND: The non-ionic agent iohexol is increasingly used as the marker of choice for glomerular filtration rate (GFR) measurement. Estimates of GFR in children have low accuracy and limiting the number of blood-draws in this patient population is especially relevant. We have performed a study to evaluate different formulas for calculating measured GFR based on plasma iohexol clearance with blood sampling at only one time point (GFR1p) and to determine the optimal sampling time point...
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
#6
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/29101428/composition-of-urinary-calculi-in-infants-a-report-from-an-endemic-country
#7
Mirza Naqi Zafar, Salma Ayub, Hafsa Tanwri, Syed Ali Anwar Naqvi, Syed Adibul Hasan Rizvi
Pediatric urolithiasis remains endemic in low-resource countries where infants constitute 17-40% of all children with urolithiasis. This study reports socio-economic factors, medical history and chemical composition of urinary stones in 976 infants of up to 2 years of age. Between 1992 and 2016, 976 infants presented to our institute with 1038 stones. Chemical composition of stones was analyzed by Fourier transformation infrared spectroscopy (FTIR). The mean age of infants was 19.5 ± 5.74 months with a M:F ratio of 5...
November 3, 2017: Urolithiasis
https://www.readbyqxmd.com/read/29100091/mutations-in-greb1l-cause-bilateral-kidney-agenesis-in-humans-and-mice
#8
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
#9
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/29077646/high-incidence-of-proteinuria-in-children-with-chronic-intestinal-failure-under-long-term-parenteral-nutrition
#10
Heiko Billing, Anna Traunspurger, Ekkehard Sturm, Andreas Busch
Long-term home parenteral nutrition (LTPN) in children with chronic intestinal failure (CIF) is associated with renal complications such as fluid and electrolyte imbalances, nephrocalcinosis, nephrolithiasis and chronic kidney disease (CKD). The etiology of CIF-associated nephropathy is multifactorial. The aim of this study was to evaluate renal involvement under LTPN. In this study 50 patients with CIF, median age 4.2 years (1.4 - 9.3, 23 female) were included. Kidney involvement was a frequent finding in this study...
October 27, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29068584/hydronephrosis-in-the-course-of-ureteropelvic-junction-obstruction-an-underestimated-problem-current-opinions-on-the-pathogenesis-diagnosis-and-treatment
#11
REVIEW
Wojciech Krajewski, Joanna Wojciechowska, Janusz Dembowski, Romuald Zdrojowy, Tomasz Szydełko
Ureteropelvic junction obstruction (UPJO) causes a reduction in the urine flow from the renal pelvis into the ureter. Untreated UPJO may cause hydronephrosis, chronic infection or urolithiasis and will often result in progressive deterioration of renal function. Most cases of UPJO are congenital; however, the disease can be clinically silent until adulthood. Other causes, both intrinsic and extrinsic, are acquired and include urolithiasis, post-operative/inflammatory/ischemic stricture, fibroepithelial polyps, adhesions and malignancy...
August 2017: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
https://www.readbyqxmd.com/read/29063524/on-the-relationship-between-proteinuria-and-plasma-phosphate
#12
Sophie de Seigneux, Alexandra Wilhelm-Bals, Marie Courbebaisse
Albuminuria is strongly associated with renal and cardiovascular outcomes independently of renal function level. However, the pathophysiology of these associations is debated. In chronic kidney disease (CKD), phosphate retention participates in cardiovascular events and increased cardiovascular mortality. We hypothesised that albuminuria may modulate tubular phosphate handling by the kidney. To verify this hypothesis, we first studied the association between phosphataemia and albuminuria in children with nephrotic syndrome and in adults with CKD...
October 24, 2017: Swiss Medical Weekly
https://www.readbyqxmd.com/read/29062906/elevated-cystatin-c-is-it-a-reflection-for-kidney-or-liver-impairment-in-hepatic-children
#13
Behairy El-Sayed, Hanaa El-Araby, Nermin Adawy, Mona Hassona, Naglaa El-Nady, Haidy Zakaria, Mohammed Khedr
AIM OF THE STUDY: To assess if elevated serum cystatin C (Cyst-C) is an indicator for renal or hepatic dysfunction in presence of liver fibrosis. MATERIAL AND METHODS: Data of 50 children with chronic liver diseases (CLDs), out of which 25 were without renal impairment, and 25 with renal impairment were analyzed. Twenty healthy children served as a healthy control group. Routine investigations, creatinine clearance, hepatitis viral markers, abdominal ultrasonography, and liver biopsy were performed for patients with CLDs...
September 2017: Clin Exp Hepatol
https://www.readbyqxmd.com/read/29061539/impact-of-adjuvant-urinary-diversion-versus-valve-ablation-alone-on-progression-from-chronic-to-end-stage-renal-disease-in-posterior-urethral-valves-a-single-institution-15-year-time-to-event-analysis
#14
Michael E Chua, Jessica M Ming, Simon Carter, Yaser El Hout, Martin A Koyle, Damien Noone, Walid A Farhat, Armando J Lorenzo, Darius J Bägli
PURPOSE: Long-term progression to end-stage renal disease (ESRD) of valve ablation alone versus ablation followed by additional urinary diversion were compared among children with chronic kidney disease stage 3 (CKD3) due to posterior urethral valves (PUV). MATERIALS AND METHODS: A retrospective study of children with PUV and CKD3 managed at a single institution between 1986 and 2011. Three treatment groups were classified: Group1 (valve ablation alone), group2 (ablation plus subsequent vesicostomy), and group3 (ablation followed by ureterostomies and/or pyelostomies)...
October 20, 2017: Journal of Urology
https://www.readbyqxmd.com/read/29055396/role-of-gut-microbiota-in-idiopathic-nephrotic-syndrome-in-children
#15
Kazunari Kaneko, Shoji Tsuji, Takahisa Kimata
Nephrotic syndrome characterized by heavy proteinuria and edema is the most common chronic kidney disease in children. It is classified into three categories, of which the idiopathic type accounts for the vast majority of cases. As indicated by the name, the etiology of idiopathic nephrotic syndrome remains unknown though it has been suggested that impaired T cell function is involved. Recently, evidence has mounted to suggest that dysfunction in regulatory T cells plays an important role in the development of allergic disease, a recognized comorbid condition for children with idiopathic nephrotic syndrome...
October 2017: Medical Hypotheses
https://www.readbyqxmd.com/read/29048948/the-spectrum-of-sickle-hemoglobin-related-nephropathy-from-sickle-cell-disease-to-sickle-trait
#16
Rakhi P Naik, Vimal K Derebail
Renal dysfunction is among the most common complication of sickle cell disease (SCD), from hyposthenuria in children to progression to overt chronic kidney disease (CKD) in young adults. Emerging evidence now suggests that sickle hemoglobin-related nephropathy extends to individuals with sickle cell trait (SCT). Areas covered: This review will highlight the pathophysiology, epidemiology, and management recommendations for sickle nephropathy in both SCD and SCT. In addition, it will focus on the major demographic and genetic modifiers of renal disease in sickling hemoglobinopathies...
October 19, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/29036228/factors-associated-to-acceptable-treatment-adherence-among-children-with-chronic-kidney-disease-in-guatemala
#17
Brooke M Ramay, Alejandro Cerón, Luis Pablo Méndez-Alburez, Randall Lou-Meda
Pediatric patients with Chronic Kidney Disease face several barriers to medication adherence that, if addressed, may improve clinical care outcomes. A cross sectional questionnaire was administered in the Foundation for Children with Kidney Disease (FUNDANIER, Guatemala City) from September of 2015 to April of 2016 to identify the predisposing factors, enabling factors and need factors related to medication adherence. Sample size was calculated using simple random sampling with a confidence level of 95%, confidence interval of 0...
2017: PloS One
https://www.readbyqxmd.com/read/29029121/clinical-heterogeneity-and-phenotypic-expansion-of-napi-iia-associated-disease
#18
Korcan Demir, Melek Yildiz, Hilla Bahat, Michael Goldman, Nisreen Hassan, Shay Tzur, Ayala Ofir, Daniella Magen
Context: NaPi-IIa, encoded by SLC34A1 is a key phosphate transporter in the mammalian proximal tubule, and plays a cardinal role in renal phosphate handling. NaPi-IIa impairment has been linked to various overlapping clinical syndromes, including hypophosphatemic nephrolithiasis with osteoporosis, renal Fanconi's syndrome with chronic kidney disease, and most recently, with idiopathic infantile hypercalcemia and nephrocalcinosis. Objectives: We studied the molecular basis of idiopathic infantile hypercalcemia with partial proximal tubulopathy in two apparently unrelated patients of Israeli and Turkish descent...
September 29, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29027595/carnosinase-diabetes-mellitus-and-the-potential-relevance-of-carnosinase-deficiency
#19
REVIEW
Verena Peters, Johannes Zschocke, Claus P Schmitt
Carnosinase (CN1) is a dipeptidase, encoded by the CNDP1 gene, that degrades histidine-containing dipeptides, such as carnosine, anserine and homocarnosine. Loss of CN1 function (also called carnosinase deficiency or aminoacyl-histidine dipeptidase deficiency) has been reported in a small number of patients with highly elevated blood carnosine concentrations, denoted carnosinaemia; it is unclear whether the variety of clinical symptoms in these individuals is causally related to carnosinase deficiency. Reduced CN1 function should increase serum carnosine concentrations but the genetic basis of carnosinaemia has not been formally confirmed to be due to CNDP1 mutations...
October 13, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/29025789/fgf23-and-left-ventricular-hypertrophy-in-children-with-ckd
#20
Mark M Mitsnefes, Aisha Betoko, Michael F Schneider, Isidro B Salusky, Myles Selig Wolf, Harald Jüppner, Bradley A Warady, Susan L Furth, Anthony A Portale
BACKGROUND AND OBJECTIVES: High plasma concentration of fibroblast growth factor 23 (FGF23) is a risk factor for left ventricular hypertrophy (LVH) in adults with CKD, and induces myocardial hypertrophy in experimental CKD. We hypothesized that high FGF23 levels associate with a higher prevalence of LVH in children with CKD. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We performed echocardiograms and measured plasma C-terminal FGF23 concentrations in 587 children with mild-to-moderate CKD enrolled in the Chronic Kidney Disease in Children (CKiD) study...
October 12, 2017: Clinical Journal of the American Society of Nephrology: CJASN
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