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Nephrotic Syndrome in children

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https://www.readbyqxmd.com/read/28730378/enzymatic-activity-of-candida-spp-from-oral-cavity-and-urine-in-children-with-nephrotic-syndrome
#1
Dorota Olczak-Kowalczyk, Maria Roszkowska-Blaim, Maria Dąbkowska, Ewa Swoboda-Kopeć, Dariusz Gozdowski, Małgorzata Mizerska-Wasiak, Urszula Demkow, Małgorzata Pańczyk-Tomaszewska
Oral colonization with Candida spp. is not synonymous with a systemic active infection. The aim of the study was to evaluate enzymatic activity of Candida strains isolated from the oral cavity in patients with nephrotic syndrome (NS) and to compare it with the activity determined in urine. We studied 32 children with NS and 26 control healthy children. Children with NS were treated with glucocorticosteroids, cyclosporin A, mycophenolate mofetil or azathioprine. In all children, API-ZYM enzymatic tests were performed to evaluate hydrolytic enzymes of Candida isolated from the oral cavity and in urine...
July 21, 2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28725977/the-effect-of-vitamin-d-and-calcium-supplementation-in-pediatric-steroid-sensitive-nephrotic-syndrome
#2
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/28717938/rituximab-in-steroid-sensitive-nephrotic-syndrome-lessons-from-clinical-trials
#3
REVIEW
Kazumoto Iijima, Mayumi Sako, Koichi Kamei, Kandai Nozu
Idiopathic nephrotic syndrome is the most common chronic glomerular disease in children. A total of 80-90% of patients with childhood idiopathic nephrotic syndrome achieve remission with steroid therapy [steroid-sensitive nephrotic syndrome (SSNS)]. However, approximately 50% of children with SSNS develop frequently relapsing nephrotic syndrome (FRNS) or steroid-dependent nephrotic syndrome (SDNS). Children with FRNS or SDNS are usually treated with immunosuppressive agents, but 10-20% of children receiving immunosuppressive agents still show frequent relapses or steroid dependence during or after treatment, defined as complicated FRNS or SDNS...
July 17, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28717937/clinical-presentation-and-outcomes-of-childhood-onset-membranous-lupus-nephritis
#4
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/28712774/characterization-of-nphs2-gene-polymorphisms-associated-to-steroid-resistance-nephrotic-syndrome-in-indian-children
#5
Bhoomi B Joshi, Kinnari N Mistry, Sishir Gang, Prakash G Koringa, Chaitanya G Joshi
Nephrotic syndrome (NS) is the common glomerular disease in children. These children are treated with steroids, depending upon their behavior. They are either steroid sensitive (SSNS) or steroid resistant (SRNS). NPHS2 gene mutants are linked to the risk of autosomal recessive SRNS and in some cases to SSNS. The present study has been performed to screen single nucleotide polymorphisms (SNPs) of the NPHS2 gene in a group of 90 Indian children suffering with NS (30 SSNS, 30 SRNS and 30 Controls) by PCR method followed by direct exon sequencing...
July 13, 2017: Gene
https://www.readbyqxmd.com/read/28699616/levamisole-in-frequently-relapsing-and-steroid-dependent-nephrotic-syndrome
#6
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
#7
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/28685619/the-mtor-p70s6k1-signaling-pathway-in-renal-fibrosis-of-children-with-immunoglobulin-a-nephropathy
#8
Yuanyuan Xu, Yihong Ling, Fan Yang, Jiong Deng, Liping Rong, Mengjie Jiang, Xiaoyun Jiang
AIM: The purpose of this study was to explore whether mTOR/p70S6K1 signaling is activated in renal fibrosis of immunoglobulin A nephropathy. METHODS: Seventy-two children with immunoglobulin A nephropathy were divided into three groups according to their clinical features and pathological grades. Six normal renal specimens were included in the control group. The expression levels of angiotensin II, mTOR, p70S6K1, E-cadherin, and α-smooth muscle actin in renal tissues were determined by immunohistochemistry method, the potential correlations of these indexes and relationship between these indexes and the clinicopathological indexes were analyzed...
July 2017: Journal of the Renin-angiotensin-aldosterone System: JRAAS
https://www.readbyqxmd.com/read/28658201/targeted-next-generation-sequencing-in-brazilian-children-with-nephrotic-syndrome-submitted-to-renal-transplant
#9
Luciana S Feltran, Patricia Varela, Elton D Silva, Camila L Veronez, Maria C Franco, Alvaro P Filho, Maria F Camargo, Paulo C Koch Nogueira, Joao B Pesquero
BACKGROUND: The aims of this study were to identify the genetic mutations profile in Brazilian children with nephrotic syndrome and to determine a genotype-phenotype correlation in this disease. METHODS: Next generation sequencing (NGS) and mutation analysis were performed on 24 genes related to nephrotic syndrome in a cross-sectional study involving 95 children who underwent kidney transplantation due to nephrotic syndrome, excluding congenital cases. RESULTS: A total of 149 variants were identified in 22 out of 24 sequenced genes...
June 28, 2017: Transplantation
https://www.readbyqxmd.com/read/28652469/blood-pressure-and-visit-to-visit-blood-pressure-variability-among-individuals-with-primary-proteinuric-glomerulopathies
#10
Christine B Sethna, Kevin E C Meyers, Laura H Mariani, Kevin J Psoter, Crystal A Gadegbeku, Keisha L Gibson, Tarak Srivastava, Matthias Kretzler, Tammy M Brady
Hypertension and blood pressure variability (BPV; SD and average real variability) in primary proteinuric glomerulopathies are not well described. Data were from 433 participants in the NEPTUNE (Nephrotic Syndrome Study Network). Hypertensive BP status was defined as previous history of hypertension or BP ≥140/90 mm Hg for adults/≥95th percentile for children at baseline. BPV was measured in participants with ≥3 visits in the first year. Two-hundred ninety-six adults (43 years [interquartile range, 32-57...
August 2017: Hypertension
https://www.readbyqxmd.com/read/28646993/improving-the-evidence-for-the-management-of-childhood-nephrotic-syndrome
#11
Brendan D Crawford, Debbie S Gipson
Management of idiopathic nephrotic syndrome in children is based on a series of clinical trials. The trial by Sinha and colleagues in this issue is 1 of many needed to improve the evidence base for induction and maintenance therapies in this population. While key questions remain about identifying the appropriate therapy for each patient, clinical trials provide an opportunity to extend evidence-based practice that minimizes toxicity and optimizes patient health.
July 2017: Kidney International
https://www.readbyqxmd.com/read/28643120/the-incidence-of-the-nephrotic-syndrome-in-childhood-in-germany
#12
Ingo Franke, Malik Aydin, Corinna Elke Llamas Lopez, Lisa Kurylowicz, Rainer Ganschow, Michael Lentze, Mark Born
BACKGROUND: The incidence of childhood nephrotic syndrome (NS) in Germany is not well known. METHODS: An ESPED-based nationwide collection of epidemiological data of children in 2005 and 2006. RESULT: The mean age of NS at onset was 5.5 ± 3.7 years. The gender ratio of boys to girls was 1.8:1. The average length of stay was 15.5 ± 11.2 days, with younger children remaining significantly longer in hospital. Steroid-resistance was more common in children ≥8 years (p = 0...
June 22, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/28630858/pulsed-vincristine-therapy-in-steroid-resistant-nephrotic-syndrome
#13
Shenal Thalgahagoda, Shamali Abeyagunawardena, Heshan Jayaweera, Umeshi Ishanthika Karunadasa, Asiri Samantha Abeyagunawardena
Steroid-resistant nephrotic syndrome (SRNS) poses a therapeutic challenge for the paediatric nephrologist. As relentless progression to renal failure occurs with continued proteinuria, such patients will be treated with different cytotoxic medications with variable success rates and side-effects. We present here our findings on administering the anticancer drug vincristine for SRNS patients at a single centre in Sri Lanka. Methods. Between 2002 and 2007, fifty-four children presenting with steroid and cyclophosphamide resistance were treated with vincristine at 1...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28615961/the-role-of-novel-biomarkers-in-childhood-idiopathic-nephrotic-syndrome-a-narrative-review-of-published-evidence
#14
REVIEW
Samuel N Uwaezuoke
Two histological subtypes of idiopathic nephrotic syndrome are commonly recognized in children, namely minimal change nephropathy and focal segmental glomerulosclerosis. Children with minimal change nephropathy (the majority of whom are steroid-sensitive) and focal segmental glomerulosclerosis (the majority of whom are steroid-resistant) require early identification in order to ensure appropriate therapeutic intervention and better outcome. Although renal biopsy and histology remain the ideal diagnostic steps to identify these histological subtypes, reports indicate that serum and urinary biomarkers are now being utilized in the investigation of childhood idiopathic nephrotic syndrome...
2017: International Journal of Nephrology and Renovascular Disease
https://www.readbyqxmd.com/read/28614261/a-prisma-compliant-meta-analysis-of-mdr1-polymorphisms-and-idiopathic-nephrotic-syndrome-susceptibility-and-steroid-responsiveness
#15
REVIEW
Shi-Sheng Han, Yan-Qiu Xu, Yan Lu, Xiang-Chen Gu, Yi Wang
BACKGROUND: Studies have investigated rs1128503, rs1045642, and rs2032582 in multidrug resistance protein 1 (MDR1) for association with susceptibility to idiopathic nephrotic syndrome (INS) and steroid resistance. However, because these findings were inconsistent, we performed a meta-analysis to determine whether there was evidence of a role of these MDR1 variants in INS. METHODS: The PubMed, Embase, and Web of Science databases were systematically searched to identify studies that examined MDR1 polymorphisms with susceptibility to INS and/or to steroid resistance...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28613279/nox-mediated-impairment-of-pdgf-induced-dna-synthesis-in-peripheral-blood-lymphocytes-of-children-with-idiopathic-nephrotic-syndrome
#16
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...
June 14, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28607687/glomerular-filtration-rate-trends-during-follow-up-in-children-with-steroid-sensitive-nephrotic-syndrome
#17
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
https://www.readbyqxmd.com/read/28600697/neurotoxicity-of-cyclosporine%C3%A2-a-in-children-with-steroid-resistant-nephrotic-syndrome-is-cytotoxic-edema-really-an-unfavorable-predictor-of-permanent-neurological-damage
#18
Danica Batinić, Danko Milošević, Boris Filipović-Grčić, Marija Topalović-Grković, Nina Barišić, Daniel Turudić
BACKGROUND: Cyclosporine A-associated neurotoxicity has been reported mainly after organ transplantation. Only a small number of children with steroid-resistant nephrotic syndrome and cyclosporine A-associated neurotoxicity have been reported. PATIENTS: We report three children, aged 4, 11, and 15, with steroid-resistant nephrotic syndrome and cyclosporine A-associated neurotoxicity. In two of the patients, primary diagnosis was idiopathic nephrotic syndrome, and in one it was IgA nephropathy...
June 9, 2017: Wiener Klinische Wochenschrift
https://www.readbyqxmd.com/read/28566477/long-term-outcome-of-steroid-resistant-nephrotic-syndrome-in-children
#19
Agnes Trautmann, Sven Schnaidt, Beata S Lipska-Ziętkiewicz, Monica Bodria, Fatih Ozaltin, Francesco Emma, Ali Anarat, Anette Melk, Marta Azocar, Jun Oh, Bassam Saeed, Alaleh Gheisari, Salim Caliskan, Jutta Gellermann, Lina Maria Serna Higuita, Augustina Jankauskiene, Dorota Drozdz, Sevgi Mir, Ayse Balat, Maria Szczepanska, Dusan Paripovic, Alexandra Zurowska, Radovan Bogdanovic, Alev Yilmaz, Bruno Ranchin, Esra Baskin, Ozlem Erdogan, Giuseppe Remuzzi, Agnieszka Firszt-Adamczyk, Elzbieta Kuzma-Mroczkowska, Mieczyslaw Litwin, Luisa Murer, Marcin Tkaczyk, Helena Jardim, Anna Wasilewska, Nikoleta Printza, Kibriya Fidan, Eva Simkova, Halina Borzecka, Hagen Staude, Katharina Hees, Franz Schaefer
We investigated the value of genetic, histopathologic, and early treatment response information in prognosing long-term renal outcome in children with primary steroid-resistant nephrotic syndrome. From the PodoNet Registry, we obtained longitudinal clinical information for 1354 patients (disease onset at >3 months and <20 years of age): 612 had documented responsiveness to intensified immunosuppression (IIS), 1155 had kidney biopsy results, and 212 had an established genetic diagnosis. We assessed risk factors for ESRD using multivariate Cox regression models...
May 31, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28553650/recent-treatment-advances-and-new-trials-in-adult-nephrotic-syndrome
#20
REVIEW
Eva Königshausen, Lorenz Sellin
The etiology of nephrotic syndrome is complex and ranges from primary glomerulonephritis to secondary forms. Patients with nephrotic syndrome often need immunosuppressive treatment with its side effects and may progress to end stage renal disease. This review focuses on recent advances in the treatment of primary causes of nephrotic syndrome (idiopathic membranous nephropathy (iMN), minimal change disease (MCD), and focal segmental glomerulosclerosis (FSGS)) since the publication of the KDIGO guidelines in 2012...
2017: BioMed Research International
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