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nephrotic syndrome, pediatric

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https://www.readbyqxmd.com/read/28943081/a-large-retrospective-review-of-persistent-proteinuria-in-children
#1
Chingying Chang-Chien, Gwo-Tsann Chuang, I-Jung Tsai, Bor-Luen Chiang, Yao-Hsu Yang
BACKGROUND: Proteinuria is a common finding in children. It may be due to a benign cause, but it can also represent early renal injury. Of children with persistent proteinuria noted in mass urine screening programs, 35% have a urine protein level greater than 100 mg/dl and many of them are associated with many underlying renal diseases. The aim of this study was to identify the etiology and prognosis of persistent proteinuria in children. METHODS: We collected data on urine protein from January 2011 to December 2016 in a tertiary medical center...
September 21, 2017: Journal of the Formosan Medical Association, Taiwan Yi Zhi
https://www.readbyqxmd.com/read/28937067/histopathological-patterns-of-renal-diseases-in-egyptian-children-a-single-center-experience
#2
Maher Ahmed Abdel-Hafez, Hend Abdel-Nabi, Mohamed El-Gamasy, Hossam Zayton, Ibrahim Nassar
The present cross-sectional, retrospective study was aimed to determine the histopathological spectrum of renal diseases in Egyptian children and to evaluate the indications, safety, and efficacy of percutaneous renal biopsy (PRB) in a large tertiary center in Egypt. PRBs performed at the Department of Pediatrics, Tanta University Hospital over a period of nine years (from January 2007 to December 2015) were included. Light microscopic (LM) examination was performed in all cases while immunofluorescence and electron microscopic examination were performed in selected cases...
September 2017: Saudi Journal of Kidney Diseases and 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
#3
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/28916546/approach-to-membranous-lupus-nephritis-a-survey-of-pediatric-nephrologists-and-pediatric-rheumatologists
#4
Alexis Boneparth, Suhas M Radhakrishna, Larry A Greenbaum, Eric Yen, Daryl M Okamura, Jennifer C Cooper, Sherene Mason, Deborah M Levy, Sangeeta D Sule, Paul T Jensen, Cagri Yildirim-Toruner, Stacy P Ardoin, Scott E Wenderfer
OBJECTIVE: To describe treatment practices for childhood pure membranous lupus nephritis (MLN). METHODS: Survey study of Childhood Arthritis and Rheumatology Research Alliance and American Society of Pediatric Nephrology members. RESULTS: There were 117 respondents who completed the survey (60 pediatric nephrologists, 57 pediatric rheumatologists). Steroids and nonsteroid immunosuppression (NSI) were routinely used by the majority for MLN...
September 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28915908/advances-in-paediatrics-in-2016-current-practices-and-challenges-in-allergy-autoimmune-diseases-cardiology-endocrinology-gastroenterology-infectious-diseases-neonatology-nephrology-neurology-nutrition-pulmonology
#5
REVIEW
Carlo Caffarelli, Francesca Santamaria, Dora Di Mauro, Carla Mastrorilli, Silvia Montella, Sergio Bernasconi
This review reports main progresses in various pediatric issues published in Italian Journal of Pediatrics and in international journals in 2016. New insights in clinical features or complications of several disorders may be useful for our better understanding. They comprise severe asthma, changing features of lupus erythematosus from birth to adolescence, celiac disease, functional gastrointestinal disorders, Moebius syndrome, recurrent pneumonia. Risk factors for congenital heart defects, Kawasaki disease have been widely investigated...
September 16, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/28884421/assessing-responsiveness-over-time-of-the-promis-%C3%A2-pediatric-symptom-and-function-measures-in-cancer-nephrotic-syndrome-and-sickle-cell-disease
#6
Bryce B Reeve, Lloyd J Edwards, Byron C Jaeger, Pamela S Hinds, Carlton Dampier, Debbie S Gipson, David T Selewski, Jonathan P Troost, David Thissen, Vaughn Barry, Heather E Gross, Darren A DeWalt
PURPOSE: Previous studies provided evidence for the validity of the PROMIS Pediatric measures in cross-sectional studies. This study evaluated the ability of the PROMIS Pediatric measures to detect change over time in children and adolescents with cancer, nephrotic syndrome (NS), or sickle cell disease (SCD). METHODS: Participants (8-17 years) completed measures of fatigue, pain interference, anger, anxiety, depressive symptoms, mobility, upper extremity, and peer relationships at three or four time points (T1-T4)...
September 7, 2017: Quality of Life Research
https://www.readbyqxmd.com/read/28868299/tubuloreticular-inclusions-in-the-absence-of-systemic-lupus-erythematosus-and-hiv-infection-a-report-of-three-pediatric-cases
#7
Ayah Elmaghrabi, Elizabeth Brown, Ei Khin, Jared Hassler, Allen R Hendricks
Tubuloreticular inclusions (TRIs) are subcellular structures located within the cisternae of endoplasmic reticulum. Formation of TRIs has been linked to the exposure of excess interferon (IFN), either from endogenous or exogenous sources. In renal disease, TRIs have been most commonly associated with systemic lupus erythematosus (SLE), and human immunodeficiency virus-associated nephropathy (HIVAN). Case reports of patients with renal biopsies showing TRIs without underlying SLE or HIV are infrequent in adults, and to our knowledge none have been reported in children...
May 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/28864927/cost-analysis-on-the-use-of-rituximab-and-calcineurin-inhibitors-in-children-and-adolescents-with-steroid-dependent-nephrotic-syndrome
#8
Franca Iorember, Diego Aviles, Mahmoud Kallash, Oluwatoyin Bamgbola
BACKGROUND: Rituximab (RTX) is increasingly being used in place of calcineurin inhibitors (CNI) in pediatric patients with steroid-dependent nephrotic syndrome (SDNS). However, despite its favorable safety profile, its unit cost is prohibitive. We therefore compared the healthcare costs associated with the use of both agents in a retrospective cohort. METHODS: This study was a retrospective analysis of data retrieved from the medical charts and electronic databases of pediatric patients (age range 2-18 years) with SDNS who were treated with either CNI or RTX from January 2008 to December 2012 at Children's Hospital of New Orleans, Louisiana...
September 1, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28835233/responsiveness-of-the-promis%C3%A2-measures-to-changes-in-disease-status-among-pediatric-nephrotic-syndrome-patients-a-midwest-pediatric-nephrology-consortium-study
#9
David T Selewski, Jonathan P Troost, Danyelle Cummings, Susan F Massengill, Rasheed A Gbadegesin, Larry A Greenbaum, Ibrahim F Shatat, Yi Cai, Gaurav Kapur, Diane Hebert, Michael J Somers, Howard Trachtman, Priya Pais, Michael E Seifert, Jens Goebel, Christine B Sethna, John D Mahan, Heather E Gross, Emily Herreshoff, Yang Liu, Noelle E Carlozzi, Bryce B Reeve, Darren A DeWalt, Debbie S Gipson
BACKGROUND: Nephrotic syndrome represents a condition in pediatric nephrology typified by a relapsing and remitting course, proteinuria and the presence of edema. The PROMIS measures have previously been studied and validated in cross-sectional studies of children with nephrotic syndrome. This study was designed to longitudinally validate the PROMIS measures in pediatric nephrotic syndrome. METHODS: One hundred twenty seven children with nephrotic syndrome between the ages of 8 and 17 years participated in this prospective cohort study...
August 23, 2017: Health and Quality of Life Outcomes
https://www.readbyqxmd.com/read/28819780/preliminary-research-on-syndrome-types-of-chinese-medicine-in-children-with-primary-nephrotic-syndrome
#10
Wen Sun, Jian Yu, Gu-Lan Zeng, Bing-Feng Zhang
OBJECTIVE: To provide an objective reference for the syndrome types of Chinese medicine (CM) associated with pediatric primary nephrotic syndrome (PNS). METHODS: A cross-sectional study was performed. Data on clinical symptoms, CM syndrome types, biochemical indices, and medications used were collected from 98 children with PNS. Then, the correlation between CM syndromes and biochemical indices, as well as medications used, was analyzed. RESULTS: The four most common symptoms in children with PNS were brown urine, red tongue, excessive sweating, and swelling of the face and limbs...
August 17, 2017: Chinese Journal of Integrative Medicine
https://www.readbyqxmd.com/read/28792718/mdr-1-and-cyp3a5-polymorphisms-in-pediatric-idiopathic-nephrotic-syndrome-impact-on-susceptibility-and-response-to-steroids-preliminary-results
#11
Amira Moussa, Sameh Mabrouk, Haithem Hamdouni, Maroua Ajmi, Miniar Tfifha, Asma Omezzine, Saoussen Abroug, Ali Bouslama
BACKGROUND: Oral steroid treatment is the first line of therapy for childhood nephrotic syndrome (NS). However, resistance to this treatment has been observed in some patients. Here, we investigated the association of two steroid metabolism-related genes with susceptibility to childhood NS and the steroid response. METHODS: We genotyped the single nucleotide polymorphisms (SNP) of MDR-1 [C1236T (rs1128503), G2677T/A (rs2032582), and C3435T (rs1045642)] and the CYP3A5 gene (A6986G) in 63 NS patients and 110 age and gender matched controls by PCR-RFLP...
July 1, 2017: Clinical Laboratory
https://www.readbyqxmd.com/read/28774288/the-impact-of-igm-deposits-on-the-outcome-of-nephrotic-syndrome-in-children
#12
Sandra Juozapaite, Rimante Cerkauskiene, Arvydas Laurinavicius, Augustina Jankauskiene
BACKGROUND: The significance of IgM deposits in glomerular mesangium has been controversial since they were first described due to the variations in the both the definitions used and described impact on clinical outcome. The aim of our study was to evaluate the significance of the IgM deposits in the glomerular mesangium for outcomes of nephrotic syndrome (NS) in children. METHODS: Forty-five children with NS who underwent renal biopsy at tertiary pediatric hospital from January 1st, 2000 to December 31st, 2015 and the pathology diagnosis of minimal change disease, focal segmental glomerulosclerosis and mesangial hypercellularity (MH) were retrospectively analyzed...
August 3, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28748888/childhood-idiopathic-steroid-resistant-nephrotic-syndrome-at-a-single-center-in-khartoum
#13
El-Tigani M A Ali, Hanna F K Makki, Mohamed B Abdelraheem, Salwa O Makke, Rashid A Allidir
Prevalence, clinicopathological features, and outcome of childhood idiopathic steroid-resistant nephrotic syndrome (ISRNS) vary in different countries. We report on these parameters in a single center in Khartoum. We retrospectively reviewed all the records of children with idiopathic nephrotic syndrome (INS) followed up in the pediatric renal unit, Soba Hospital, Khartoum between 2001 and 2012. ISRNS was defined as no remission within four weeks of daily prednisolone at a dose of 60 mg/m2. In 430 children with INS 130 (28%) had SRNS with a mean age of 7...
July 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28725977/the-effect-of-vitamin-d-and-calcium-supplementation-in-pediatric-steroid-sensitive-nephrotic-syndrome
#14
Sushmita Banerjee, Surupa Basu, Ananda Sen, Jayati Sengupta
BACKGROUND: Low serum levels of total 25-hydroxycholecalciferol (25(OH)D) occur in nephrotic syndrome (NS). We aimed to assess the effects of vitamin D3 and calcium supplementation on 25(OH)D levels, bone mineralization, and NS relapse rate in children with steroid-sensitive NS. METHODS: A randomized controlled trial (RCT) was performed in children with steroid-sensitive NS. The treatment group received vitamin D3 (60,000 IU orally, weekly for 4 weeks) and calcium supplements (500 to 1,000 mg/day for 3 months) after achieving NS remission...
July 19, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28724397/partial-uniparental-isodisomy-of-chromosome-16-unmasks-a-deleterious-biallelic-mutation-in-ift140-that-causes-mainzer-saldino-syndrome
#15
Benjamin M Helm, Jason R Willer, Azita Sadeghpour, Christelle Golzio, Eric Crouch, Samantha Schrier Vergano, Nicholas Katsanis, Erica E Davis
BACKGROUND: The ciliopathies represent an umbrella group of >50 clinical entities that share both clinical features and molecular etiology underscored by structural and functional defects of the primary cilium. Despite the advances in gene discovery, this group of entities continues to pose a diagnostic challenge, in part due to significant genetic and phenotypic heterogeneity and variability. We consulted a pediatric case from asymptomatic, non-consanguineous parents who presented as a suspected ciliopathy due to a constellation of retinal, renal, and skeletal findings...
July 19, 2017: Human Genomics
https://www.readbyqxmd.com/read/28717937/clinical-presentation-and-outcomes-of-childhood-onset-membranous-lupus-nephritis
#16
Maria Pereira, Eyal Muscal, Karen Eldin, M John Hicks, Anna Carmela P Sagcal-Gironella, Marietta DeGuzman, Scott E Wenderfer
BACKGROUND: Best practices for managing childhood-onset membranous lupus nephritis (MLN) are not yet established. Most studies involve primarily or exclusively adult cohorts or pediatric cohorts with combinations of pure or mixed membranous and proliferative nephritis. METHODS: We performed a single-center cohort study of consecutively diagnosed children with pure MLN from 1990 and 2016. Patients received care in Houston, Texas, one of the most diverse metropolitan areas in North America...
July 17, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28699616/levamisole-in-frequently-relapsing-and-steroid-dependent-nephrotic-syndrome
#17
Evangeline Mary Kiruba Samuel, Sriram Krishnamurthy, Singanamalla Bhanudeep, Sravani Muske
OBJECTIVE: To evaluate the efficacy of levamisole in children with frequently relapsing nephrotic syndrome (FRNS) and steroid dependent nephrotic syndrome (SDNS) when administered on an alternate day ('initial therapy' in all cases) or daily basis ('rescue therapy' in whom alternate day therapy failed). METHODS: The records of 95 children (age 1-18y) with FRNS and SDNS, who were treated at the Pediatric nephrology clinic, and received levamisole therapy (maximum 2 years duration, between 2010-2013) with a follow-up period of minimum1 year, were included...
July 11, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/28699031/effectiveness-and-nephrotoxicity-of-a-2-year-medium-dose-of-cyclosporine-in-pediatric-patients-with-steroid-dependent-nephrotic-syndrome-determination-of-the-need-for-follow-up-kidney-biopsy
#18
Yoshiyuki Kuroyanagi, Yoshimitsu Gotoh, Katsuaki Kasahara, China Nagano, Naoya Fujita, Satoshi Yamakawa, Masaki Yamamoto, Asami Takeda, Osamu Uemura
BACKGROUND: High dose of cyclosporine (CyA) for ≥2 years in children with steroid-dependent nephrotic syndrome (SDNS) increases the risk for nephropathy. Considering this, risk can be lowered with lower doses of CyA; we evaluated the effects of a medium dose of CyA, with target serum level, C2, of 450 ng/ml, over a 2-year period of observation, to determine the need for follow-up kidney biopsy. METHODS: We retrospectively evaluated C2 levels in 38 patients (17 males, 5...
July 11, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/28613279/nox-mediated-impairment-of-pdgf-induced-dna-synthesis-in-peripheral-blood-lymphocytes-of-children-with-idiopathic-nephrotic-syndrome
#19
Amal Al-Eisa, Gursev S Dhaunsi
BackgroundCellular oxidative stress, inflammatory responses, and immunogenic events are involved in pathogenesis of idiopathic nephrotic syndrome (INS); however, the exact mechanism remains unknown. We examined NADPH oxidase (NOX) activity and platelet-derived growth factor (PDGF)-induced DNA synthesis in peripheral blood lymphocytes (PBL) of patients with INS.MethodsPBL from 15 patients with INS and 15 age- and gender-matched controls were isolated, and enzyme activities of NOX, catalase, and superoxide dismutase (SOD) were measured along with the assay of malondialdehyde levels and bromo-deoxyuridine incorporation...
October 2017: Pediatric Research
https://www.readbyqxmd.com/read/28607687/glomerular-filtration-rate-trends-during-follow-up-in-children-with-steroid-sensitive-nephrotic-syndrome
#20
Sulaiman Alsaidi, Daniel Wagner, Silviu Grisaru, Julian Midgley, Lorraine Hamiwka, Andrew Wade, Alberto Nettel-Aguirre, Susan Samuel
BACKGROUND: Overall prognosis of children with steroid-sensitive nephrotic syndrome (SSNS) is regarded as generally favorable. However, only a few recent studies have evaluated changes in kidney function and blood pressure over time in children with SSNS. OBJECTIVES: We describe clinical features of SSNS patients and characterize changes in calculated estimated glomerular filtration rate (eGFR) and use of antihypertensive medications during follow-up. DESIGN: This is a retrospective cohort study...
2017: Canadian Journal of Kidney Health and Disease
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