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pediatric proteinuria

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https://www.readbyqxmd.com/read/28087540/international-evidence-based-consensus-diagnostic-criteria-for-hhv-8-negative-idiopathic-multicentric-castleman-disease
#1
David C Fajgenbaum, Thomas S Uldrick, Adam Bagg, Dale Frank, David Wu, Gordan Srkalovic, David Simpson, Amy Y Liu, David Menke, Shanmuganathan Chandrakasan, Mary Jo Lechowicz, Raymond S M Wong, Sheila Pierson, Michele Paessler, Jean-François Rossi, Makoto Ide, Jason Ruth, Michael Croglio, Alexander Suarez, Vera Krymskaya, Amy Chadburn, Gisele Colleoni, Sunita Nasta, Raj Jayanthan, Christopher S Nabel, Corey Casper, Angela Dispenzieri, Alexander Fosså, Dermot Kelleher, Razelle Kurzrock, Peter Voorhees, Ahmet Dogan, Kazuyuki Yoshizaki, Frits van Rhee, Eric Oksenhendler, Elaine S Jaffe, Kojo S J Elenitoba-Johnson, Megan S Lim
Human Herpesvirus-8(HHV-8)-negative, idiopathic multicentric Castleman disease (iMCD) is a rare and life-threatening disorder involving systemic inflammatory symptoms, polyclonal lymphoproliferation, cytopenias, and multiple organ system dysfunction due to a cytokine storm often including interleukin-6. iMCD accounts for one-third to one-half of all cases of MCD and can occur in individuals of any age. Accurate diagnosis is challenging, as no standard diagnostic criteria or diagnostic biomarkers currently exist, and there is significant overlap with malignant, autoimmune, and infectious disorders...
January 13, 2017: Blood
https://www.readbyqxmd.com/read/28087478/renal-involvement-in-lysinuric-protein-intolerance-contribution-of-pathology-to-assessment-of-heterogeneity-of-renal-lesions
#2
Emmanuel Estève, Pauline Krug, Aurélie Hummel, Jean-Baptiste Arnoux, Olivia Boyer, Anais Brassier, Pascale de Lonlay, Vincent Vuiblet, Stéphanie Gobin, Rémi Salomon, Christine Piètrement, Jean-Paul Bonnefont, Aude Servais, Louise Galmiche
Lysinuric protein intolerance (LPI) is a rare autosomal recessive disease caused by mutations in the SLC7A7 gene encoding the light subunit of a cationic amino acid transporter. Symptoms mimic primary urea cycle defects but dysimmune symptoms are also described. Renal involvement in LPI was first described in the 1980's. In 2007, it appeared that it could concern as much as 75% of LPI patients and could lead to end stage renal disease. The most common feature is proximal tubular dysfunction and nephrocalcinosis but glomerular lesions are also reported...
January 10, 2017: Human Pathology
https://www.readbyqxmd.com/read/28051228/nephrotic-syndrome-in-children-risk-factors-for-steroid-dependence
#3
Manel Jellouli, Meriem Brika, Kamel Abidi, Meriem Ferjani, Ouns Naija, Yousra Hammi, Tahar Gargah
Background - Most patients with idiopathic nephrotic syndrome are steroid-responsive, about 50% relapse and often become steroid-dependent and exposed to long-term steroid complications. The aim of this study was to determine predictive risk factors for steroid dependence using clinical and biological variables present at onset of the disease. It may be useful to adapt the therapeutic strategy. Methods - Retrospective hospital-based cohort study in the department of pediatric nephrology of Charles Nicolle, Tunis, between 2002 and 2012 included 52 children with idiopathic steroid-responsive nephrotic...
July 2016: La Tunisie Médicale
https://www.readbyqxmd.com/read/27940460/minimal-change-disease
#4
Marina Vivarelli, Laura Massella, Barbara Ruggiero, Francesco Emma
Minimal change disease (MCD) is a major cause of idiopathic nephrotic syndrome (NS), characterized by intense proteinuria leading to edema and intravascular volume depletion. In adults, it accounts for approximately 15% of patients with idiopathic NS, reaching a much higher percentage at younger ages, up to 70%-90% in children >1 year of age. In the pediatric setting, a renal biopsy is usually not performed if presentation is typical and the patient responds to therapy with oral prednisone at conventional doses...
December 9, 2016: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/27938596/-focal-segmental-glomerulosclerosis-after-renal-transplantation-in-a-child-with-anca-associated-glomerulonephritis-case-report-and-literature-review
#5
L P Rong, L Z Chen, Q Fu, W F Chen, L Z Sun, X Y Jiang, Y Mo
Objective: To study the clinical features and treatment of focal segmental glomerulosclerosis (FSGS) after renal transplantation in a child with ANCA-associated glomerulonephritis. Method: The clinical and pathological data of the patient treated in the Department of Pediatrics as well as in the Department of Organ Transplantation in November 2015 in the First Affiliated Hospital of Sun Yat-sen University, who was diagnosed with de novo FSGS after renal transplantation with a primary disease ANCA-associated glomerulonephritis, was analyzed retrospectively...
December 2, 2016: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/27921164/m-type-phospholipase-a2-receptor-pla2r-glomerular-staining-in-pediatric-idiopathic-membranous-nephropathy
#6
Shoichiro Kanda, Shigeru Horita, Takeshi Yanagihara, Akira Shimizu, Motoshi Hattori
BACKGROUND: Identifying M-type phospholipase A2 receptor (PLA2R) is a landmark breakthrough for understanding adult idiopathic membranous nephropathy (iMN). However, potential roles for PLA2R in pediatric iMN have not been well investigated. METHODS: A total of 34 pediatric iMN patients who underwent kidney biopsy between 1972 and 2015 were enrolled in this study. The study cohort consisted of 15 children aged from 3 to 9 years and 19 aged from 10 to 15 years...
December 5, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/27889724/phenotype-of-dent-disease-in-a-cohort-of-indian-children
#7
Swati Bhardwaj, Ranjeet Thergaonkar, Aditi Sinha, Pankaj Hari, Cheong Hi, Arvind Bagga
OBJECTIVE: To describe the clinical and genotypic features of Dent disease in children diagnosed at our center over a period of 10 years. DESIGN: Case series. SETTING: Pediatric Nephrology Clinic at a referral center in Northern India. METHODS: The medical records of patients with Dent disease diagnosed and followed up at this hospital from June 2005 to April 2015 were reviewed. The diagnosis of Dent disease was based on presence of all three of the following: (i) low molecular weight proteinuria, (ii) hypercalciuria and (iii) one of the following: nephrolithiasis, hematuria, hypophosphatemia or renal insufficiency, with or without mutation in CLCN5 or OCRL1 genes...
November 15, 2016: Indian Pediatrics
https://www.readbyqxmd.com/read/27826732/renin-angiotensin-ii-aldosterone-system-blockers-and-time-to-renal-replacement-therapy-in-children-with-ckd
#8
Alison G Abraham, Aisha Betoko, Jeffrey J Fadrowski, Christopher Pierce, Susan L Furth, Bradley A Warady, Alvaro Muñoz
BACKGROUND: Clinical care decisions to treat chronic kidney disease (CKD) in a growing child must often be made without the benefit of evidence from clinical trials. We used observational data from the Chronic Kidney Disease in Children cohort to estimate the effectiveness of renin-angiotensin II-aldosterone system blockade (RAAS) to delay renal replacement therapy (RRT) in children with CKD. METHODS: A total of 851 participants (median age: 11 years, median glomerular filtration rate [GFR]: 52 ml/min/1...
November 8, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/27784126/monitoring-of-complement-activation-biomarkers-and-eculizumab-in-complement-mediated-renal-disorders
#9
C Wehling, O Amon, M Bommer, B Hoppe, K Kentouche, G Schalk, R Weimer, M Wiesener, B Hohenstein, B Tönshoff, R Büscher, H Fehrenbach, Ö-N Gök, M Kirschfink
Various complement-mediated renal disorders are treated currently with the complement inhibitor eculizumab. By blocking the cleavage of C5, this monoclonal antibody prevents cell damage caused by complement-mediated inflammation. We included 23 patients with atypical haemolytic uraemic syndrome (aHUS, n = 12), C3 glomerulopathies (C3G, n = 9) and acute antibody-mediated renal graft rejection (AMR, n = 2), treated with eculizumab in 12 hospitals in Germany. We explored the course of complement activation biomarkers and the benefit of therapeutic drug monitoring of eculizumab...
February 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/27752908/differences-in-pathological-characteristics-and-laboratory-indicators-in-adult-and-pediatric-patients-with-henoch-sch%C3%A3-nlein-purpura-nephritis
#10
Zhi Liu, Yu-Dan Wei, Yue Hou, Ying Xu, Xiu-Jiang Li, Yu-Jun Du
We aimed to investigate the differences in renal histopathological changes and laboratory parameters between adult and pediatric patients with Henoch-Schönlein purpura nephritis (HSPN), and to analyze the correlation between laboratory parameters and renal histopathological grading. A total of 139 patients diagnosed with HSPN between September 2010 and December 2014 at the First Hospital of Jilin University, China, were retrospectively reviewed. The clinical and pathological characteristics were examined and compared between the adult and the pediatric patients...
October 2016: Journal of Huazhong University of Science and Technology. Medical Sciences
https://www.readbyqxmd.com/read/27714465/iga-nephropathy-with-presentation-of-nephrotic-syndrome-at-onset-in-children
#11
Yuko Shima, Koichi Nakanishi, Masashi Sato, Taketsugu Hama, Hironobu Mukaiyama, Hiroko Togawa, Ryojiro Tanaka, Kandai Nozu, Mayumi Sako, Kazumoto Iijima, Hiroyuki Suzuki, Norishige Yoshikawa
BACKGROUND: Despite a low incidence, nephrotic syndrome (NS) can present with IgA nephropathy (IgAN). The clinical characteristics and long-term outcomes of pediatric patients with IgAN presenting with NS (NS-IgAN) at onset have not been fully elucidated. METHODS: We retrospectively analyzed 426 patients, and compared clinical and pathological (Oxford) findings between those with NS-IgAN and those with non-NS-IgAN. RESULTS: Among 426 patients, 30 (7...
October 6, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/27704256/achieving-remission-of-proteinuria-in-childhood-ckd
#12
Piero Ruggenenti, Paolo Cravedi, Antonietta Chianca, MariaRosa Caruso, Giuseppe Remuzzi
BACKGROUND: A multidrug treatment strategy that targets urinary proteins with an angiotensin-converting enzyme (ACE) inhibitor and angiotensin receptor blocker (ARB) up-titrated to the respective maximum tolerated dose combined with intensified blood pressure (BP) control has been found to prevent renal function loss in adults with proteinuric nephropathies. Herein, we investigated the effects of this treatment protocol in the pediatric patient population. METHODS: From May 2002 to September 2014 we included in this observational, longitudinal, cohort study 20 consecutive children with chronic nephropathies and 24-h proteinuria of >200 mg who had received ramipril and losartan up-titrated to the respective maximum approved and tolerated doses [mean (standard deviation) dose:2...
February 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/27696583/yield-and-complications-of-kidney-biopsies-over-two-decades-in-a-tertiary-pediatric-center
#13
Roei Hod Feins, Anna Tobar, Miriam Davidovits
BACKGROUND: Kidney biopsy serves as an adjunct for the diagnosis of renal disease. However, it is not always productive. This study evaluated the yield and risks of kidney biopsies performed in 1995-2014 at a tertiary pediatric medical center. METHODS: The medical files of all patients who underwent closed percutaneous biopsy for various indications in native or transplanted kidneys were retrospectively reviewed for patient characteristics, technical and histopathologic findings, biopsy yield, and biopsy complications...
October 3, 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/27617138/the-genetics-of-nephrotic-syndrome
#14
REVIEW
Michelle N Rheault, Rasheed A Gbadegesin
Nephrotic syndrome (NS) is a common pediatric kidney disease and is defined as massive proteinuria, hypoalbuminemia, and edema. Dysfunction of the glomerular filtration barrier, which is made up of endothelial cells, glomerular basement membrane, and visceral epithelial cells known as podocytes, is evident in children with NS. While most children have steroid-responsive nephrotic syndrome (SSNS), approximately 20% have steroid-resistant nephrotic syndrome (SRNS) and are at risk for progressive kidney dysfunction...
March 2016: Journal of Pediatric Genetics
https://www.readbyqxmd.com/read/27590627/the-experience-of-canakinumab-in-renal-amyloidosis-secondary-to-familial-mediterranean-fever
#15
Betul Sozeri, Nesrin Gulez, Malik Ergin, Erkin Serdaroglu
INTRODUCTION: Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by self-limited recurrent attacks of fever and serositis. Patients may develop renal amyloidosis. Colchicine prevents attacks and renal amyloidosis. Five to 10 % of the patients with FMF are resistant or intolerant to colchicine. CASE DESCRIPTION: Herein, we reported our experience with clinical-laboratory features and treatment responses of a pediatric FMF patient with amyloidosis treated with canakinumab...
December 2016: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/27573641/iga-nephropathy-in-children-a-multicenter-study-in-poland
#16
M Mizerska-Wasiak, A Turczyn, A Such, K Cichoń-Kawa, J Małdyk, M Miklaszewska, J Pietrzyk, A Rybi-Szumińska, A Wasilewska, A Firszt-Adamczyk, R Stankiewicz, M Szczepańska, B Bieniaś, M Zajączkowska, A Pukajło-Marczyk, D Zwolińska, K Siniewicz-Luzeńczyk, M Tkaczyk, K Gadomska-Prokop, R Grenda, U Demkow, M Pańczyk-Tomaszewska
IgA nephropathy (IgAN) is the most common form of glomerulonephritis in pediatric population. The clinical presentation of the disease in children ranges from microscopic hematuria to end-stage kidney disease. The aim of the study was to retrospectively assess clinical and kidney biopsy features in children with IgAN. We assessed a cohort of 140 children, 88 boys, 52 girls with the diagnosis of IgAN in the period of 2000-2015, entered into the national Polish pediatric IgAN registry. The assessment included the following: proteinuria, hematuria, glomerular filtration rate (GFR), arterial blood pressure, and the renal pathological changes according to the Oxford classification and crescents formation, as modifiable and unmodifiable risk factors...
2016: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/27561690/immune-mediated-nephropathies-in-kidney-transplants-recurrent-or-de-novo-diseases
#17
Isabel Roberti, Shefali Vyas
IMN contribute to ESRD in 13% children with renal transplant (txp). Recurrent or de novo IMN can cause graft dysfunction and/or failure, but the details regarding incidence, therapy, and outcome remain poorly understood. Retrospective single-center study of all pediatric kidney txp was carried out since 1998. Clinical presentation, pathology, therapy, and graft outcomes of children with recurrent or de novo IMN were reviewed. IMN was the primary etiology of ESRD in 28 of the 149 txp recipients. Eleven children had biopsy-proven post-txp IMN-six were recurrent and five had de novo...
August 25, 2016: Pediatric Transplantation
https://www.readbyqxmd.com/read/27528553/the-relatively-poor-correlation-between-random-and%C3%A2-24-hour-urine-protein-excretion-in-patients-with%C3%A2-biopsy-proven-glomerular-diseases
#18
Marie C Hogan, Heather N Reich, Peter J Nelson, Sharon G Adler, Daniel C Cattran, Gerald B Appel, Debbie S Gipson, Matthias Kretzler, Jonathan P Troost, John C Lieske
Random urine protein creatinine ratios are used to estimate 24-hour urine protein excretion, which is considered a diagnostic gold standard. However, few studies are available of the sensitivity and specificity of this estimation in patients with glomerular proteinuria. To clarify this, we measured the urine protein and creatinine centrally in random and 24-hour urine collections at biopsy and longitudinally every 6 months in individuals participating in the Nephrotic Syndrome Study Network (NEPTUNE) cohort with glomerular disease...
November 2016: Kidney International
https://www.readbyqxmd.com/read/27490751/focal-segmental-glomerulosclerosis-in-pediatric-kidney-transplantation-30%C3%A2-years-experience
#19
Roxana Cleper, Irit Krause, Nathan Bar Nathan, Maya Mor, Amit Dagan, Irith Weissman, Yaakov Frishberg, Ruty Rachamimov, Eitan Mor, Miriam Davidovits
: From 1982 to 2011, 53 kidney transplantations (KT) for pediatric focal segmental glomerulosclerosis (FSGS) were recorded in the National Israeli Kidney Transplant Registry (NIKTR): 22-primary (1◦) FSGS, 25-proved/suspected genetic-secondary (2◦) FSGS, six lost/incomplete files/other. Half (56%) of 23 patients with 2◦ FSGS were Israeli-Arabs vs 29% of 1◦ FSGS KT recipients. 1◦ FSGS recurrence occurred in 64% (14/22) of 22 KT in 17 patients aged (median) 14 years vs 1/25 of 2◦ FSGS (P<...
October 2016: Clinical Transplantation
https://www.readbyqxmd.com/read/27478910/assessment-of-chronic-renal-injury-from-melamine-associated-pediatric-urolithiasis-an-eighteen-month-prospective-cohort-study
#20
Jian Gao, Fei Wang, Xinyu Kuang, Rong Chen, Jia Rao, Bin Wang, Wenyan Li, Haimei Liu, Qian Shen, Xiang Wang, Hong Xu
BACKGROUND: The illegal use of melamine in powdered baby formula resulted in a widespread outbreak of melamine-associated pediatric urolithiasis and kidney damage in China in 2008. We conducted this study because more needs to be known about the long-term effects of melamine-associated urolithiasis and kidney damage. OBJECTIVES: To determine the prognosis and long-term implications of chronic kidney damage in children with urolithiasis resulting from melamine consumption...
July 2016: Annals of Saudi Medicine
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