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https://www.readbyqxmd.com/read/29305810/serum-sickness-with-refractory-nephrotic-syndrome-following-treatment-with-rituximab
#1
Ryo Maeda, Yukihiko Kawasaki, Shinichiro Ohara, Kazuhide Suyama, Mitsuaki Hosoya
Rituximab (RTX) is effective for treating childhood refractory nephrotic syndrome (NS), such as steroid-dependent (SD), frequently relapsing (FR), and steroid-resistant (SR) NS. While RTX has been proven to be effective in treating SDNS, FRNS, and SRNS, it may cause serum sickness, a rare illness characterized by fever, rash, and arthralgia, 10-14 days after primary antigen exposure or within a few days after secondary antigen exposure, by producing human anti-chimeric antibodies (HACAs). A 17-year-old girl with refractory SDNS treated with RTX and oral cyclosporine A was admitted with fever and arthralgia 10 days after the fifth RTX dose was administered...
January 5, 2018: CEN Case Reports
https://www.readbyqxmd.com/read/29277510/hla-dqa1-and-apol1-as-risk-loci-for-childhood-onset-steroid-sensitive-and-steroid-resistant-nephrotic-syndrome
#2
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
#3
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
#4
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/29207799/change-in-bone-mineral-density-and-role-of-vitamin-d-and-calcium-supplementation-during-treatment-of-first-episode-nephrotic-syndrome
#5
Virendra Kumar Yadav, Shobha Sharma, Pradeep Kumar Debata, Seema Patel, Bhaskar C Kabi, Kailash Chandra Aggrawal
Introduction: Nephrotic Syndrome (NS) is one of the common illnesses in childhood. Oral glucocorticoids are mainstay of treatment, which are known to cause various short term and long term adverse effects including osteoporosis. Children with first episode and infrequent relapsing NS are not prescribed Calcium (Ca) and Vitamin D (VitD) supplements routinely. Aim: To observe change in Bone Mineral Density (BMD) in children during first episode of NS and role of VitD and Ca supplementation during the treatment of first episode of NS...
September 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/29181713/genetic-testing-in-steroid-resistant-nephrotic-syndrome-why-who-when-and-how
#6
REVIEW
Rebecca Preston, Helen M Stuart, Rachel Lennon
Steroid-resistant nephrotic syndrome (SRNS) is a common cause of chronic kidney disease in childhood and has a significant risk of rapid progression to end-stage renal disease. The identification of over 50 monogenic causes of SRNS has revealed dysfunction in podocyte-associated proteins in the pathogenesis of proteinuria, highlighting their essential role in glomerular function. Recent technological advances in high-throughput sequencing have enabled indication-driven genetic panel testing for patients with SRNS...
November 27, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29177628/mycophenolate-mofetil-following-glucocorticoid-treatment-in-henoch-sch%C3%A3-nlein-purpura-nephritis-the-role-of-early-initiation-and-therapeutic-drug-monitoring
#7
Agnes Hackl, Jan U Becker, Lisa M Körner, Rasmus Ehren, Sandra Habbig, Eva Nüsken, Kai-Dietrich Nüsken, Kathrin Ebner, Max C Liebau, Carsten Müller, Martin Pohl, Lutz T Weber
BACKGROUND: Henoch-Schönlein purpura (HSP) is the most common vasculitis in childhood and traditionally considered as a self-limiting disease. However, renal involvement can unfavorably determine long-term prognosis. The reported regimens to treat HSP nephritis (HSPN) are diverse, indicating that the most effective treatment remains controversial. METHODS: This retrospective, single-center study involved 18 patients presenting with HSPN and nephrotic-range proteinuria...
November 25, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29155175/fcgr2a-single-nucleotide-polymorphism-confers-susceptibility-to-childhood-onset-idiopathic-nephrotic-syndrome
#8
Giovanni M Rossi, Francesco Bonatti, Alessia Adorni, Federico Alberici, Monica Bodria, Alice Bonanni, Gian M Ghiggeri, Davide Martorana, Augusto Vaglio
Childhood-onset idiopathic nephrotic syndrome affects 1.15-3.4 children/100,000 children/year in Western Countries. Immune-mediated mechanisms, particularly T cell-mediated, are thought to play a key pathogenic role. The genetic basis of the disease is still poorly understood. We tested the association between single nucleotide polymorphisms (SNPs) of four genes encoding Fc gamma receptors (FCGR2A, FCGR2B, FCGR3A, FCGR3B) and idiopathic nephrotic syndrome in a case-control study of paediatric patients. Children with idiopathic nephrotic syndrome (aged 1-16 years) were included...
January 2018: Immunology Letters
https://www.readbyqxmd.com/read/29081077/effect-of-cesarean-section-on-relapse-of-childhood-idiopathic-nephrotic-syndrome
#9
Takahisa Kimata, Jiro Kino, Sohsaku Yamanouchi, Chikushi Suruda, Shoji Tsuji, Kazunari Kaneko
In parallel with the increase in the prevalence of childhood chronic diseases, the rate of cesarean delivery has risen during the past decades. This study tested the hypothesis that children delivered by cesarean section (CS) have a higher risk of relapse of idiopathic nephrotic syndrome (INS). Fifty-six children with INS were categorized into three groups. Group A consisted of patients with INS who had no relapses after the onset of INS; group B consisted of patients with INS who had infrequent relapse; and group C consisted of patients with INS who had frequent relapse...
October 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/29038887/mutations-in-inf2-may-be-associated-with-renal-histology-other-than-focal-segmental-glomerulosclerosis
#10
Anja K Büscher, Nora Celebi, Peter F Hoyer, Hanns-Georg Klein, Stefanie Weber, Julia Hoefele
BACKGROUND: In 2010, INF2 mutations were associated with autosomal-dominant focal segmental glomerulosclerosis (FSGS), clinically presenting with moderate proteinuria in adolescence. However, in the meantime, cases with more severe clinical courses have been described, including progression to end-stage renal disease (ESRD) during childhood. INF2 mutations in patients with isolated FSGS are clustered in exons 2 to 4, encoding the diaphanous inhibitory domain, involved in the regulation of the podocyte actin cytoskeleton...
October 6, 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
#11
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/29022104/deriving-and-understanding-the-risk-of-post-transplant-recurrence-of-nephrotic-syndrome-in-the-light-of-current-molecular-and-genetic-advances
#12
REVIEW
Agnieszka Bierzynska, Moin A Saleem
After renal transplantation, recurrence of the original disease is the second most common cause of graft loss, after rejection. The most dramatic manifestation of this phenomenon is in patients with nephrotic syndrome (NS). NS is a descriptive term describing a clinical picture centred on proteinuria arising from damage to the glomerular filtration barrier (GFB). There are many different drivers of that damage, ranging from immune dysregulation to genetic disorders and chronic disease/infections. The main categories in childhood are "idiopathic" (presumed immune mediated) and genetic NS, with further stratification of the idiopathic group according to steroid responses...
October 11, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28965242/uteroglobin-gene-polymorphism-g38a-may-be-a-risk-factor-in-childhood-idiopathic-nephrotic-syndrome
#13
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/28963315/reducing-steroids-in-relapsing-nephrotic-syndrome-the-restern-study-protocol-of-a-national-double-blind-randomised-placebo-controlled-non-inferiority-intervention-study
#14
A M Schijvens, E M Dorresteijn, N Roeleveld, R Ter Heine, J A E van Wijk, A H M Bouts, M G Keijzer-Veen, N C A J van de Kar, L P W J van den Heuvel, M F Schreuder
INTRODUCTION: Oral corticosteroids are the first-line treatment for idiopathic childhood nephrotic syndrome. Most children experience several relapses, needing repeated courses of corticosteroid therapy. This exposes them to side effects and long-term complications. For most patients, long-term prognosis is for complete resolution of the disease over time and maintenance of normal kidney function. Therefore, it is vital to focus on minimising adverse events of the disease and its therapy...
September 27, 2017: BMJ Open
https://www.readbyqxmd.com/read/28949280/childhood-nephrotic-syndrome-in-tropical-africa-then-and-now
#15
Wasiu A Olowu, Adebowale Ademola, Adebukola B Ajite, Yauba M Saad
This descriptive and comparative review examines the changing epidemiology, treatment, renal and patient outcome of childhood nephrotic syndrome (NS) in tropical Africa (TpAfr). In the 1960s to 1980s, corticosteroid-resistant non-minimal change disease (nMCD) including quartan malaria nephropathy (QMN) was the dominant renal histopathology type. The overall incidence of NS was 0.35-1.34% of hospital admissions. Median age at onset of NS ranged between 4.0 and 12.0 years while the mean (SD) age range was 5.8 (3...
September 26, 2017: Paediatrics and International Child Health
https://www.readbyqxmd.com/read/28941627/-childhood-steroid-dependent-idiopathic-nephrotic-syndrome-predictive-factors-for-the-need-of-immunosuppressive-treatment
#16
M Golay, A Douillard, N Nagot, M Fila, L Ichay, F Dalla Vale, J Tenenbaum, D Morin
BACKGROUND AND AIMS: More than half of the children with idiopathic nephrotic syndrome become steroid-dependent (or frequent relapsers) and will later require the use of complementary treatment aiming to reduce steroids' side effects and to limit the number of proteinuria relapses. It appears important to identify these children as early as possible in order to adapt their treatment. The aim of this study was to analyze the population of children, under 18 years of age, diagnosed between 1/01/2000 and 31/05/2015 with an idiopathic nephrotic syndrome and followed at the Montpellier University Hospital to search for criteria predictive of steroid-sparing agent use...
November 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28937086/pulmonary-thromboembolism-as-first-presentation-of-childhood-membranous-nephropathy
#17
Mohit Madken, Shankar Prasad Nagaraju, Ravindra Prabhu Attur, Mahesha Vankalakunti
Nephrotic syndrome is associated with an increased risk of thromboembolism. Pulmonary thromboembolism has been described in nephrotic syndrome with or without deep vein thrombosis. In this case report, we describe an unusual first presentation of childhood membranous nephropathy with massive pulmonary thrombus with pulmonary infarction with right renal vein thrombosis.
September 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28916546/approach-to-membranous-lupus-nephritis-a-survey-of-pediatric-nephrologists-and-pediatric-rheumatologists
#18
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...
November 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28914167/nephrotic-syndrome-in-infants-and-children-pathophysiology-and-management
#19
Mallory L Downie, Claire Gallibois, Rulan S Parekh, Damien G Noone
Nephrotic syndrome is defined by nephrotic-range proteinuria (≥40 mg/m2/hour or urine protein/creatinine ratio ≥200 mg/mL or 3+ protein on urine dipstick), hypoalbuminaemia (<25 g/L) and oedema. This review focuses on the classification, epidemiology, pathophysiology, management strategies and prognosis of idiopathic nephrotic syndrome of childhood, and includes a brief overview of the congenital forms.
November 2017: Paediatrics and International Child Health
https://www.readbyqxmd.com/read/28904948/recent-progress-in-deciphering-the-etiopathogenesis-of-primary-membranous-nephropathy
#20
REVIEW
Andreas Kronbichler, Jun Oh, Björn Meijers, Gert Mayer, Jae Il Shin
Primary membranous nephropathy (MN) is the leading cause of nephrotic syndrome in adults. Discovery of several antibodies has contributed to an increased understanding of MN. Antibodies against the M-type phospholipase A2 receptor (PLA2R) are present in 50-100% with primary MN and are associated with a lower frequency of spontaneous remission. High levels are linked with a higher probability of treatment resistance, higher proteinuria, and impaired renal function, as well as a more rapid decline of kidney function during follow-up...
2017: BioMed Research International
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