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pediatric nephrotic syndrome

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https://www.readbyqxmd.com/read/29310412/kimura-disease-of-buccal-region-in-a-pediatric-patient-with-nephrotic-syndrome-a-case-report
#1
Dong Hoon Lee, Ga-Eon Kim, Eunmi Yang, Tae Mi Yoon, Joon Kyoo Lee, Sang Chul Lim
RATIONALE: Kimura disease is a rare benign, chronic inflammatory disorder that typically presents with slowly enlarging, nontender, subcutaneous swellings in the head and neck region. The occurrence of Kimura disease in the oral cavity is extremely rare. PATIENT CONCERNS: A 16-year-old boy presented with a complaint of a right painless buccal mass of 3 years' duration. DIAGNOSIS: The patient had been diagnosed with nephrotic syndrome and treated with corticosteroid at the age of 5 years...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29279700/minimal-change-disease-as-initial-presentation-of-alk-positive-anaplastic-large-cell-lymphoma-in-a-pediatric-patient
#2
Karla Diaz, William Slayton, Nirupama Gupta
The association between nephrotic syndrome (NS), hemophagocytic lymphohistiocytosis (HLH), and certain paraneoplastic syndromes has been documented in the literature. However, nephrotic changes as part of paraneoplastic syndromes are rare in lymphoid malignancies, particularly in non-Hodgkin lymphoma. We report the sudden onset of acute renal failure and NS in a 14-year-old male who initially presented with HLH and was subsequently diagnosed with ALK-positive anaplastic large-cell lymphoma (ALCL). The treatment of ALCL reversed both the HLH and NS findings...
September 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/29277510/hla-dqa1-and-apol1-as-risk-loci-for-childhood-onset-steroid-sensitive-and-steroid-resistant-nephrotic-syndrome
#3
Adebowale Adeyemo, Christopher Esezobor, Adaobi Solarin, Asiri Abeyagunawardena, Jameela A Kari, Sherif El Desoky, Larry A Greenbaum, Margret Kamel, Mahmoud Kallash, Cynthia Silva, Alex Young, Tracey E Hunley, Nilka de Jesus-Gonzalez, Tarak Srivastava, Rasheed Gbadegesin
BACKGROUND: Few data exist for the genetic variants underlying the risk for steroid-sensitive nephrotic syndrome (SSNS) in children. The objectives of this study were to evaluate HLA-DQA1 and APOL1 variants as risk factors for SSNS in African American children and use classic HLA antigen types and amino acid inference to refine the HLA-DQA1 association. STUDY DESIGN: Case-control study. SETTING & PARTICIPANTS: African American children with SSNS or steroid-resistant nephrotic syndrome (SRNS) were enrolled from Duke University and centers participating in the Midwest Pediatric Nephrology Consortium...
December 22, 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/29229167/glomerular-diseases-in-children
#4
REVIEW
Scott E Wenderfer, Joseph P Gaut
Unique challenges exist in the diagnosis and treatment of glomerular diseases with their onset during childhood. Mounting evidence supports the notion that earlier onset cases occur due to larger numbers of genetic risk alleles. Nearly all causes of adult-onset glomerulonephritis, nephrotic syndrome, and thrombotic microangiopathy have also been described in children, although the prevalence of specific causes differs. Postinfectious glomerulonephritis, Henoch-Schönlein purpura nephritis, and minimal change disease remain the most common causes of glomerular disease in younger children in the United States and can be diagnosed clinically without need for biopsy...
November 2017: Advances in Chronic Kidney Disease
https://www.readbyqxmd.com/read/29218062/preliminary-study-regarding-the-association-between-tumor-necrosis-factor-alpha-gene-polymorphisms-and-childhood-idiopathic-nephrotic-syndrome-in-romanian-pediatric-patients
#5
Ioana Tieranu, Monica I Dutescu, Constantin Bara, Cristian G Tieranu, Mihaela Balgradean, Olivia M Popa
Background: Childhood idiopathic nephrotic syndrome (INS) is one of the most common glomerular diseases, characterized by heavy proteinuria, hypoalbuminemia, dyslipidemia and generalized edema. Although some progresses were made regarding the pathogenesis of this disease, there are a lot of questions still left unanswered. Some of them involve the implications of several cytokines, including tumor necrosis factor alpha (TNF-alpha), in the development and clinical course of INS. Objective: Our objective was to analyze the role of two single nucleotide polymorphisms of TNF-alpha gene in the development of pediatric INS and their implication in the response to corticosteroid therapy...
September 2017: Mædica
https://www.readbyqxmd.com/read/29212948/elevated-urinary-creld2-is-associated-with-endoplasmic-reticulum-stress-mediated-kidney-disease
#6
Yeawon Kim, Sun-Ji Park, Scott R Manson, Carlos Af Molina, Kendrah Kidd, Heather Thiessen-Philbrook, Rebecca J Perry, Helen Liapis, Stanislav Kmoch, Chirag R Parikh, Anthony J Bleyer, Ying Maggie Chen
ER stress has emerged as a signaling platform underlying the pathogenesis of various kidney diseases. Thus, there is an urgent need to develop ER stress biomarkers in the incipient stages of ER stress-mediated kidney disease, when a kidney biopsy is not yet clinically indicated, for early therapeutic intervention. Cysteine-rich with EGF-like domains 2 (CRELD2) is a newly identified protein that is induced and secreted under ER stress. For the first time to our knowledge, we demonstrate that CRELD2 can serve as a sensitive urinary biomarker for detecting ER stress in podocytes or renal tubular cells in murine models of podocyte ER stress-induced nephrotic syndrome and tunicamycin- or ischemia-reperfusion-induced acute kidney injury (AKI), respectively...
December 7, 2017: JCI Insight
https://www.readbyqxmd.com/read/29141308/-guidelines-for-the-diagnosis-and-treatment-of-steroid-resistant-nephrotic-syndrome
#7
(no author information available yet)
No abstract text is available yet for this article.
November 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29130775/results-of-native-and-transplant-kidney-biopsies-of-children-in-a-single-center-over-a-15-years-period
#8
Emel Isiyel, Kibriya Fidan, Bahar Buyukkaragoz, Meltem Akcaboy, Yasar Kandur, Ipek Isik Gonul, Necla Buyan, Sevcan Bakkaloglu, Oguz Soylemezoglu
Renal biopsy is an important diagnostic procedure in pediatric nephrology. This study retrospectively investigates the indications, results and complications in a single tertiary children's hospital in Turkey. We evaluated the native and transplant kidney biopsies in Gazi University Pediatric Nephrology Department between 2001 and 2015. A total of 196 biopsies (144 natives and 52 transplants) were included into the study. The mean age of the patients was respectively 10.8 ± 3.5, 13.9 ± 1.5 years...
November 2017: Renal Failure
https://www.readbyqxmd.com/read/29119640/il-23-actived-%C3%AE-%C3%AE-t-cells-affect-th17-cells-and-regulatory-t-cells-by-secreting-il-21-in-children-with-primary-nephrotic-syndrome
#9
Li Zhang, Junli Yan, Baohui Yang, Gaofu Zhang, Mo Wang, Shifang Dong, Wei Liu, Haiping Yang, Qiu Li
OBJECTIVE: This study (1) analyzed the percentage of γδ T cells, γδ T cell subsets, Th17 cells, and regulatory T cells (Treg cells), and (2) determined the role of IL-23 in primary nephrotic syndrome (PNS) patients with active disease and in remission. MATERIALS AND METHODS: Eighty-four PNS patients and 51 healthy age-matched controls were included in this study. The percentage of γδ T cells, γδ T cell subsets, Th17 cells, and Treg cells in peripheral blood mononuclear cells (PBMCs) were analyzed by fluorescence-activated cell sorting...
November 9, 2017: Scandinavian Journal of Immunology
https://www.readbyqxmd.com/read/29069848/efficacy-and-safety-of-immunosuppressive-medications-for-steroid-resistant-nephrotic-syndrome-in-children-a-systematic-review-and-network-meta-analysis
#10
Shaojun Li, Haiping Yang, Pengfei Guo, Xiaoxiao Ao, Junli Wan, Qiu Li, Liping Tan
BACKGROUND: Conventional meta-analyses and randomized controlled trials have shown inconsistent results regarding the efficacy of immunosuppressants for pediatric steroid-resistant nephrotic syndrome (SRNS). OBJECTIVE: To conduct a network meta-analysis aimed at evaluating the efficacy and safety of available immunosuppressive agents in pediatric patients with SRNS. STUDY METHODS: MEDLINE, the Cochrane Central Register of Controlled Trials, and EMBASE were searched on January 2017...
September 22, 2017: Oncotarget
https://www.readbyqxmd.com/read/29050108/-evidence-based-guideline-on-diagnosis-and-treatment-of-steroid-sensitive-relapsing-steroid-dependent-nephrotic-syndrome-in-children-2016
#11
(no author information available yet)
No abstract text is available yet for this article.
October 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29045960/pharmacokinetics-of-once-daily-prolonged-release-formulation-of-tacrolimus-in-children-with-primary-nephrotic-syndrome
#12
Y Han, S Q DU, H J Xiao, Y Zhou, J Ding, J J Ding, Y M Cui
OBJECTIVE: Tacrolimus prolonged-release(PR) formulation is a new once-daily formulation of the calcineurin inhibitor tacrolimus, which is currently used in adult liver or kidney transplant patients,and is also gradually widely used in children with nephrotic syndrome.The present study was undertaken to preliminarily investigate the pharmacokinetic characteristics of tacrolimus PR in pediatric nephrotic syndrome recipients. METHODS: This single-center open-label prospective study was performed in pediatric nephrotic syndrome recipients...
October 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/29034405/immunohistochemical-and-serological-characterization-of-membranous-nephropathy-in-children-and-adolescents
#13
Anne K Dettmar, Thorsten Wiech, Markus J Kemper, Armin Soave, Michael Rink, Jun Oh, Rolf A K Stahl, Elion Hoxha
BACKGROUND: Membranous nephropathy (MN) is a common cause of nephrotic syndrome in adults, but is less frequent in children. Antibodies against four antigens leading to MN have been described in children: phospholipase A2 receptor 1 (PLA2R1), thrombospondin type-1 domain-containing 7A (THSD7A), neutral endopeptidase (NEP), and cationic bovine serum albumin (BSA). METHODS: Twelve children with MN were included in this study. Sera of all patients were analyzed for antibodies against PLA2R1, THSD7A, NEP, and BSA...
October 15, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29031489/-pediatric%C3%A2-nephrology-what%C3%A2-should-an%C3%A2-adult%C3%A2-nephrologist-know-about%C3%A2-these%C3%A2-diseases
#14
Sandrine Lemoine, Pierre Cochat, Aurélia Bertholet-Thomas, Charlène Levi, Catherine Bonnefoy, Anne-Laure Sellier-Leclerc, Justine Bacchetta
In nephrology, some diseases begin specifically during childhood; however, they must be known by adult nephrologists so that to ensure continuity and homogeneity for their management. The aim of this review is therefore to propose a brief overview of the main pediatric diseases, for which a specific knowledge is warranted, and notably pediatric idiopathic nephrotic syndrome, cystinosis, primary hyperoxaluria and hereditaries tubulopathies.
October 11, 2017: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/28992850/association-of-single-nucleotide-polymorphism-in-the-fkbp5-gene-with-response-to-steroids-in-pediatric-patients-with-primary-nephrotic-syndrome%C3%A2
#15
Ni Du, Fang Yang, Liangzhi Li, Xiaoxiao Liu, Liangzhong Sun, Sui Zhang, Xianhong He, Yuewu Tang, Jihong Shi, Chunqiu Liu, Xianxiang Zhang
AIM: This study aimed to assess the association of single-nucleotide polymorphism (SNP) of FKBP5 with response to steroids in children with primary nephrotic syndrome (NS). MATERIALS AND METHODS: A total of 66 primary NS patients (cases) and 68 healthy individuals (controls) were enrolled in this study. The FKBP5 polymorphism rs4713916 (T/C) was analyzed by polymerase chain reaction (PCR) and sequencing after amplification of regions that potentially contain the SNP...
October 10, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28965242/uteroglobin-gene-polymorphism-g38a-may-be-a-risk-factor-in-childhood-idiopathic-nephrotic-syndrome
#16
Beltinge Demircioglu Kılıc, Mithat Buyukcelik, Sibel Oguzkan Balcı, Sacide Pehlivan, Seval Kul, Nilgun Col, Ayse Balat
BACKGROUND: Uteroglobin (UG) is a multifunctional protein with anti-inflammatory properties. The aim of this study was to first evaluate the role of UG gene G38A polymorphism in childhood idiopathic nephrotic syndrome (INS), and determine whether this variation may be related to the occurrence of INS or a steroid response. METHODS: One hundred and thirty-six children diagnosed with INS in Gaziantep University, Department of Pediatric Nephrology, and 70 healthy volunteers were included...
September 30, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28951877/predictability-of-urinary-cd80-in-the-relapse-of-primary-nephrotic-syndrome
#17
Juan Liao, Xiao-Chuan Wu, Qia Cheng, Can-Lin Li, Zhu-Wen Yi, Yan Cao, Lan-Jun Shuai
PURPOSE: The current study is aimed at investigating whether urinary CD80 is reliable to predict the recurrence of pediatric PNS. MATERIALS AND METHODS: A total of 128 children, 105 males and 23 females, were enrolled in this study. Urinary samples were collected from SSNS and SRNS patients and 25 healthy children as controls. Urinary CD80 was measured by ELISA and adjusted for urinary creatinine excretion. RESULTS: Urinary CD80 in relapse stage of SSNS was significantly higher, and the urinary CD80 of paired relapse and remission stages of each SSNS patient were also significantly different...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28943081/a-large-retrospective-review-of-persistent-proteinuria-in-children
#18
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
#19
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
#20
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
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