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Nephrotic syndrome children

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https://www.readbyqxmd.com/read/28339709/clinicopathologic-correlations-of-renal-pathology-in-adult-population-of-poland
#1
Agnieszka Perkowska-Ptasinska, Artur Bartczak, Malgorzata Wagrowska-Danilewicz, Agnieszka Halon, Krzysztof Okon, Aldona Wozniak, Marian Danilewicz, Henryk Karkoszka, Andrzej Marszalek, Jolanta Kowalewska, Andrzej Mroz, Agnieszka Korolczuk, Andrzej Oko, Alicja Debska-Slizien, Beata Naumnik, Zbigniew Hruby, Marian Klinger, Kazimierz Ciechanowski, Marek Myslak, Wladyslaw Sulowicz, Andrzej Rydzewski, Andrzej Wiecek, Jacek Manitius, Tadeusz Gregorczyk, Stanislaw Niemczyk, Michal Nowicki, Ryszard Gellert, Tomasz Stompor, Monika Wieliczko, Krzysztof Marczewski, Leszek Paczek, Olga Rostkowska, Dominika Deborska-Materkowska, Grazyna Bogdanowicz, Andrzej Milkowski, Magdalena Durlik
Background: This is the first report on the epidemiology of biopsy-proven kidney diseases in Poland. Methods: The Polish Registry of Renal Biopsies has collected information on all (n = 9394) native renal biopsies performed in Poland from 2009 to 2014. Patients' clinical data collected at the time of biopsy, and histopathological diagnoses were used for epidemiological and clinicopathologic analysis. Results: There was a gradual increase in the number of native renal biopsies performed per million people (PMP) per year in Poland in 2009-14, starting from 36 PMP in 2009 to 44 PMP in 2014...
February 11, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28336816/glomerular-density-and-volume-in-renal-biopsy-specimens-of-children-with-proteinuria-relative-to-preterm-birth-and-gestational-age
#2
Kentaro Koike, Yohei Ikezumi, Nobuo Tsuboi, Go Kanzaki, Kotaro Haruhara, Yusuke Okabayashi, Takaya Sasaki, Makoto Ogura, Akihiko Saitoh, Takashi Yokoo
BACKGROUND AND OBJECTIVES: A low total nephron number, which is associated with low birth weight (LBW), may indicate increased susceptibility to early-onset renal diseases in children. However, few studies have assessed renal biopsy findings in LBW children. We examined the relationship between LBW and glomerular density (GD) and/or glomerular volume (GV) in renal biopsy samples as a surrogate for total nephron number. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Renal biopsy findings of children of LBW were compared with those of age-matched control subjects of normal birth weight (NBW) who were histopathologically diagnosed with FSGS or minimal change nephrotic syndrome (MCNS) from 1995 to 2011...
March 23, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/28335748/steroid-resistant-nephrotic-syndrome-as-the-initial-presentation-of-nail-patella-syndrome-a-case-of-a-de-novo-lmx1b-mutation
#3
Tomohiro Nakata, Ryo Ishida, Yuu Mihara, Atsuko Fujii, Yoshimoto Inoue, Tetsuro Kusaba, Tsuyoshi Isojima, Yutaka Harita, Chiaki Kanda, Sachiko Kitanaka, Keiichi Tamagaki
BACKGROUND: Nail-patella syndrome (NPS) is an autosomal dominant disorder caused by mutations in the LMX1B gene and is characterized by nail dysplasia, skeletal abnormalities, and nephropathy. We herein report a case of steroid-resistant nephrotic syndrome (SRNS) prior to overt orthopedic symptoms in a patient with NPS. CASE PRESENTATION: A 24-year-old woman presented to our hospital with knee pain. She had poorly developed nails, hypoplastic patellas, dislocation of the elbows, and iliac horns in the pelvis...
March 23, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28318625/mycophenolate-mofetil-is-inferior-to-tacrolimus-in-sustaining-remission-in-children-with-idiopathic-steroid-resistant-nephrotic-syndrome
#4
Aditi Sinha, Aarti Gupta, Mani Kalaivani, Pankaj Hari, Amit K Dinda, Arvind Bagga
Studies of nephrotic syndrome show that substitution of calcineurin inhibitors by mycophenolate mofetil (MMF) enables sustained remission and corticosteroid sparing and avoids therapy associated adverse effects. However, controlled studies in patients with steroid resistance are lacking. Here we examined the effect of switching from therapy with tacrolimus to MMF on disease course in an open-label, one-to-one randomized, controlled trial on children (one to 18 years old), recently diagnosed with steroid-resistant nephrotic syndrome, at a referral center in India...
March 15, 2017: Kidney International
https://www.readbyqxmd.com/read/28314744/randomised-controlled-trial-comparing-ofatumumab-to-rituximab-in-children-with-steroid-dependent-and-calcineurin-inhibitor-dependent-idiopathic-nephrotic-syndrome-study-protocol
#5
Pietro Ravani, Alice Bonanni, Gian Marco Ghiggeri
INTRODUCTION: Oral steroids induce remission in about 90% of children with idiopathic nephrotic syndrome (INS), which is characterised by severe proteinuria and hypoalbuminaemia. Some children become steroid-dependent (SD) and require addition of calcineurin inhibitors (CNI) to maintain remission. Since these oral agents are toxic, alternative interventions are needed for long-term treatment. The anti-CD20 antibody rituximab has shown promising steroid-sparing properties in clinical trials, but benefits are less convincing in complicated forms of SD-INS...
March 17, 2017: BMJ Open
https://www.readbyqxmd.com/read/28314579/keeping-the-customers-stratified-moving-toward-genetics-based-treatment-options-in-childhood-ns
#6
Megan Puckelwartz, H William Schnaper
In this issue, Bierzynska et al. report the results of analyzing a cohort of children with steroid-resistant nephrotic syndrome (SRNS) of childhood, correlating genetic studies with clinical outcomes. This approach has the potential to advance both our research into the causes and treatments of childhood SRNS and our ability to stratify patients into groups that are likely to be responsive to specific treatments. Here, we discuss some of the strengths and limitations of this report.
April 2017: Kidney International
https://www.readbyqxmd.com/read/28299461/efficacy-of-higher-dose-levamisole-in-maintaining-remission-in-steroid-dependant-nephrotic-syndrome
#7
Asiri S Abeyagunawardena, Umeshi Karunadasa, Heshan Jayaweera, Shenal Thalgahagoda, Sampath Tennakoon, Shamali Abeyagunawardena
OBJECTIVE: Levamisole (LEV) has been used successfully on an alternate-day regime of 2.5 mg/kg in steroid-dependant nephrotic syndrome (SDNS) to maintain remission. This pilot study was carried out between 2010 and 2015 at a single center in Sri Lanka to evaluate the efficacy of LEV prescribed at 2.5 mg/kg daily, which is double the alternate-day dose. METHODS: Sequential children with SDNS, relapsing more than twice in the preceding 12 months and previously treated with LEV and low-dose alternate-day prednisolone (0...
March 15, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28298181/early-onset-of-adck4-glomerulopathy-with-renal-failure-a-case-report
#8
Ksenija Lolin, Benedetta D Chiodini, Elise Hennaut, Brigitte Adams, Karin Dahan, Khalid Ismaili
BACKGROUND: We present a rare early presentation of a ADCK4-related glomerulopathy. This case is of interest as potentially treatable if genetic results are timely obtained. CASE PRESENTATION: We report the case of a 5-year-old boy who was identified with significant proteinuria by a urinary routine screening program for school children. Physical examination revealed dysplastic ears and abnormal folded pinna. Albumin level was 41 g/L (39-53 g/L), and urine proteins/creatinine ratio was 2...
March 16, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/28288230/-somatotropic-axis-and-molecular-markers-of-mineral-metabolism-in-children-undergoing-chronic-peritoneal-dialysis
#9
María Luisa Ceballos Osorio, Francisco Cano Schuffeneger
Growth failure is one of the most relevant complications in children with chronic kidney disease (CKD). Among others, growth hormone (GH) resistance and bone mineral disorders have been identified as the most important causes of growth retardation. OBJECTIVES: 1. To characterize bone mineral metabolism and growth hormone bio-markers in CKD children treated with chronic peritoneal dialysis (PD). 2. To evaluate height change with rhGH treatment. PATIENTS AND METHOD: A longitudinal 12-month follow-up in prepuberal PD children...
February 2017: Revista Chilena de Pediatría
https://www.readbyqxmd.com/read/28270642/kidney-disease-profile-of-syrian-refugee-children
#10
Mehtap Akbalik Kara, Beltinge Demircioglu Kilic, Nilgun Col, Ayse Aysima Ozcelik, Mithat Buyukcelik, Ayse Balat
INTRODUCTION: Although preventative nephrology is the effective management of childhood kidney diseases, it is hard to provide it in this undesirable conditions. In this study, we aimed to document the kidney disease profile of Syrian refugee children admitted to our hospital. MATERIALS AND METHODS: One hundred and thirty Syrian refugee children were admitted to the Pediatric Nephrology Department of the University of Gaziantep from September 2012 to January 2015...
March 2017: Iranian Journal of Kidney Diseases
https://www.readbyqxmd.com/read/28257282/wt1-mutation-associated-nephropathy-a%C3%A2-single-center-experience%C3%A2
#11
Zhihui Yue, Haiyan Wang, Hongrong Lin, Juan Yang, Ting Liu, Yulin Liu, Huamu Chen, Liangzhong Sun
This study explored Wilms' tumor 1 (WT1) mutations in children with, or suspected of having, steroid-resistant nephrotic syndrome (SRNS), referred to or treated in our hospital in the past 6 years as well as the correlation between genotype and phenotype in WT1 mutation-associated nephropathy in Chinese patients. In total, 76 patients participated in the study. WT1 mutations were identified in 15 patients, 5 of whom harbored splice-site mutations in intron 9. Four of these 5 patients exhibited early onset of nephropathy and rapid deterioration of renal function...
March 3, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28254584/outcomes-and-risk-factors-for-graft-loss-lessons-learned-from-1-056-pediatric-kidney-transplants-at-the-university-of-minnesota
#12
Srinath Chinnakotla, Priya Verghese, Blanche Chavers, Michelle N Rheault, Varvara Kirchner, Ty Dunn, Clifford Kashtan, Thomas Nevins, Michael Mauer, Timothy Pruett
BACKGROUND: Advances in immunosuppression, surgical techniques, and management of infections in children receiving kidney transplants have impacted outcomes. STUDY DESIGN: We analyzed a prospectively maintained database of pediatric kidney transplants. RESULTS: From June 1963 through October 2016, we performed 1,056 pediatric kidney transplants. Of these 129 were < 2 years old. The most common indications for transplant were congenital anomalies (dysplastic kidneys), obstructive uropathy and congenital nephrotic syndrome...
January 11, 2017: Journal of the American College of Surgeons
https://www.readbyqxmd.com/read/28250989/apolipoprotein-c-i-levels-are-associated-with-the-urinary-protein-urinary-creatinine-ratio-in-pediatric-idiopathic-steroid-sensitive-nephrotic-syndrome-a-case-control-study
#13
Jun Odaka, Takahiro Kanai, Takane Ito, Takashi Saito, Jun Aoyagi, Hiroyuki Betsui, Takanori Yamagata
Humoral factors may cause idiopathic steroid-sensitive nephrotic syndrome (ISSNS). In the present study, we analyzed serum proteins using mass spectrometry (MS) to identify proteins associated with the pathophysiology of pediatric ISSNS. We collected serial serum samples from 33 children during each ISSNS phase; Phase A1 is the acute phase prior to steroid treatment (STx), Phase A2 represents the remission period with STx, and Phase A3 represents the remission period after completion of STx. Children with normal urinalyses (Group B) and children with a nephrotic syndrome other than ISSNS (Group C) served as controls...
2017: International Journal of Nephrology
https://www.readbyqxmd.com/read/28213606/parental-health-literacy-and-outcomes-of-childhood-nephrotic-syndrome
#14
Karlota Borges, Cathryn Sibbald, Neesha Hussain-Shamsy, Jovanka Vasilevska-Ristovska, Tonny Banh, Viral Patel, Josefina Brooke, Monica Piekut, Michele Reddon, Kimberly Aitken-Menezes, Ashley McNaughton, Rachel J Pearl, Valerie Langlois, Seetha Radhakrishnan, Christoph P B Licht, Tino D Piscione, Leo Levin, Damien Noone, Diane Hebert, Rulan S Parekh
OBJECTIVE: Determine the association of parental health literacy with treatment response among children with nephrotic syndrome. METHODS: This was a cohort study of children aged 1-18 with nephrotic syndrome and their parent. Health literacy was measured using the validated Short Test of Functional Health Literacy in Adults assessing reading comprehension and numeracy. Outcomes included initial relapse-free period, frequently relapsing disease, relapse rate, second-line medication use, and complete remission after therapy...
February 17, 2017: Pediatrics
https://www.readbyqxmd.com/read/28210837/toll-like-receptor-3-tlr-3-tlr-4-and-cd80-expression-in-peripheral-blood-mononuclear-cells-and-urinary-cd80-levels-in-children-with-idiopathic-nephrotic-syndrome
#15
Om P Mishra, Ravindra Kumar, Gopeshwar Narayan, Pradeep Srivastava, Abhishek Abhinay, Rajniti Prasad, Ankur Singh, Vineeta V Batra
BACKGROUND: The aims of this study were (1) to detect toll-like receptor (TLR)-3, TLR-4 and CD80 expression in peripheral blood mononuclear cells (PBMCs) and estimate urinary CD80 levels in children with idiopathic nephrotic syndrome and (2) to investigate the utility of these markers to differentiate between biopsy-proven minimal change disease (MCD) and focal segmental glomeruloscelerosis (FSGS). METHODS: The study included 70 patients with idiopathic nephrotic syndrome (NS), of whom 40 had steroid-sensitive NS (SSNS; 25 with active NS, 15 in remission) and 30 had steroid-resistant NS (SRNS) patients, and 23 healthy controls...
February 16, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28209267/assessment-of-volume-status-and-appropriate-fluid-replenishment-in-the-setting-of-nephrotic-syndrome
#16
Pierluigi Marzuillo, Stefano Guarino, Andrea Apicella, Rosaria Marotta, Vincenzo Tipo, Laura Perrone, Angela La Manna, Giovanni Montini
BACKGROUND: When the permeability of the glomerular filtration barrier increases, leading to proteinuria, nephrotic syndrome (NS) occurs. First episodes or relapses of NS can be concurrent with acute gastroenteritis (AGE) infections. This condition can cause further deterioration of the hypovolemic state, as intravascular water is lost through both AGE-related vomiting/diarrhea and NS-related fluid shifting into the interstitium. In this case report, we wish to raise the issues about the difficult management of children presenting with both NS and AGE...
April 2017: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28204946/clinical-outcomes-in-children-with-henoch-sch%C3%A3-nlein-purpura-nephritis-without-crescents
#17
Jean Daniel Delbet, Julien Hogan, Bilal Aoun, Iulia Stoica, Rémi Salomon, Stéphane Decramer, Isabelle Brocheriou, Georges Deschênes, Tim Ulinski
BACKGROUND: Henoch-Schönlein purpura is the most common vasculitis in children. Its long-term prognosis depends on renal involvement. The management of Henoch-Schönlein purpura nephritis (HSPN) remains controversial. This study reports the prognosis of children with HSPN presenting with class 2 International Study of Kidney Disease in Children (ISKDC) nephritis. METHODS: All children with HSPN class 2 diagnosed between 1995 and 2015 in four pediatric nephrology centers were included, and clinical and biological data were collected from the medical files...
February 15, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28204945/spectrum-of-mutations-in-chinese-children-with-steroid-resistant-nephrotic-syndrome
#18
Fang Wang, Yanqin Zhang, Jianhua Mao, Zihua Yu, Zhuwen Yi, Li Yu, Jun Sun, Xiuxiu Wei, Fangrui Ding, Hongwen Zhang, Huijie Xiao, Yong Yao, Weizhen Tan, Svjetlana Lovric, Jie Ding, Friedhelm Hildebrandt
BACKGROUND: The aim of this study was to elucidate whether genetic screening test results of pediatric patients with steroid-resistant nephrotic syndrome (SRNS) vary with ethnicity. METHODS: Using high-throughput DNA sequencing, 28 nephrotic syndrome-related genes were analyzed in 110 chil-dren affected by SRNS and 10 children with isolated proteinuria enrolled by 5 centers in China (67 boys, 53 girls). Their age at disease onset ranged from 1 day to 208 months (median, 48...
February 15, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28188379/a-novel-mutation-of-laminin-%C3%AE-2-lamb2-in-two-siblings-with-renal-failure
#19
Farah A Falix, Carlien A M Bennebroek, Bert van der Zwaag, Ruth Lapid-Gortzak, Sandrine Florquin, Michiel J S Oosterveld
This report describes a novel mutation of LAMB2, the gene associated with Pierson syndrome (microcoria-congenital nephrosis syndrome), in two female siblings. The c.970T>C p.(Cys324Arg) mutation in the LAMB2 gene affects one of the eight highly conserved cysteine residues within the first EGF-like module of the laminin β2 protein. These residues form disulfide bonds in order to achieve a correct 3D structure of the protein. The reported phenotype is considered a relatively mild variant of Pierson syndrome and is associated with later-onset (18 months) therapy-resistant nephrotic syndrome leading to renal failure, and ocular abnormalities consisting of high myopia, microcoria, diverse retinal abnormalities, hence a low level of visual acuity...
February 10, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28181337/deficiency-of-the-sphingosine-1-phosphate-lyase-sgpl1-is-associated-with-congenital-nephrotic-syndrome-and-congenital-adrenal-calcifications
#20
Andreas R Janecke, Ruijuan Xu, Elisabeth Steichen-Gersdorf, Siegfried Waldegger, Andreas Entenmann, Thomas Giner, Iris Krainer, Lukas A Huber, Michael W Hess, Yaacov Frishberg, Hila Barash, Shay Tzur, Nira Schreyer-Shafir, Rivka Sukenik-Halevy, Tania Zehavi, Annick Raas-Rothschild, Cungui Mao, Thomas Müller
We identified two unrelated consanguineous families with three children affected by the rare association of congenital nephrotic syndrome (CNS) diagnosed in the first days of life, of hypogonadism, and of prenatally detected adrenal calcifications, associated with congenital adrenal insufficiency in one case. Using exome sequencing and targeted Sanger sequencing, two homozygous truncating mutations, c.1513C>T (p.Arg505*) and c.934delC (p.Leu312Phefs*30), were identified in SGPL1-encoding sphingosine-1-phosphate (S1P) lyase 1...
April 2017: Human Mutation
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