keyword
MENU ▼
Read by QxMD icon Read
search

steroid resistant nephrotic syndrome

keyword
https://www.readbyqxmd.com/read/29141309/-interpretation-of-guidelines-for-the-diagnosis-and-treatment-of-steroid-resistant-nephrotic-syndrome
#1
C L Gao, Z K Xia
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/29141308/-guidelines-for-the-diagnosis-and-treatment-of-steroid-resistant-nephrotic-syndrome
#2
(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
#3
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/29127259/whole-exome-sequencing-of-patients-with-steroid-resistant-nephrotic-syndrome
#4
Jillian K Warejko, Weizhen Tan, Ankana Daga, David Schapiro, Jennifer A Lawson, Shirlee Shril, Svjetlana Lovric, Shazia Ashraf, Jia Rao, Tobias Hermle, Tilman Jobst-Schwan, Eugen Widmeier, Amar J Majmundar, Ronen Schneider, Heon Yung Gee, J Magdalena Schmidt, Asaf Vivante, Amelie T van der Ven, Hadas Ityel, Jing Chen, Carolin E Sadowski, Stefan Kohl, Werner L Pabst, Makiko Nakayama, Michael J G Somers, Nancy M Rodig, Ghaleb Daouk, Michelle Baum, Deborah R Stein, Michael A Ferguson, Avram Z Traum, Neveen A Soliman, Jameela A Kari, Sherif El Desoky, Hanan Fathy, Martin Zenker, Sevcan A Bakkaloglu, Dominik Müller, Aytul Noyan, Fatih Ozaltin, Melissa A Cadnapaphornchai, Seema Hashmi, Jeffrey Hopcian, Jeffrey B Kopp, Nadine Benador, Detlef Bockenhauer, Radovan Bogdanovic, Nataša Stajić, Gil Chernin, Robert Ettenger, Henry Fehrenbach, Markus Kemper, Reyner Loza Munarriz, Ludmila Podracka, Rainer Büscher, Erkin Serdaroglu, Velibor Tasic, Shrikant Mane, Richard P Lifton, Daniela A Braun, Friedhelm Hildebrandt
BACKGROUND AND OBJECTIVES: Steroid-resistant nephrotic syndrome overwhelmingly progresses to ESRD. More than 30 monogenic genes have been identified to cause steroid-resistant nephrotic syndrome. We previously detected causative mutations using targeted panel sequencing in 30% of patients with steroid-resistant nephrotic syndrome. Panel sequencing has a number of limitations when compared with whole exome sequencing. We employed whole exome sequencing to detect monogenic causes of steroid-resistant nephrotic syndrome in an international cohort of 300 families...
November 10, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29098400/histone-deacetylase-2-expression-and-activity-in-children-with-nephrotic-syndrome-with-different-glucocorticoid-response
#5
Feng-Jun Guan, Qian-Qian Peng, Lin-Li Wang, Xue-Bo Yan, Chen Dong, Xiao-Hua Jiang
BACKGROUND: Glucocorticosteroid (GC) is one of the most effective drugs available for the treatment of primary nephrotic syndrome (PNS) in children. However, some patients show little or no response to GC. The purpose of our research was to observe and describe the different levels of histone deacetylase-2 (HDAC2) expression in peripheral blood lymphocytes in children with PNS compared with various responses to the GC treatment, with the primary aim to assess the correlation between HDAC2 and GC resistance in PNS children...
November 2, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29080118/immunosuppressive-therapy-for-steroid-resistant-nephrotic-syndrome-a-bayesian-network-meta-analysis-of-randomized-controlled-studies
#6
Xinxin Jiang, Wei Shen, Xiujun Xu, Xiaogang Shen, Yiwen Li, Qiang He
BACKGROUND: The purpose of this study was to conduct a meta-analysis examining the efficacy of cyclophosphamide, cyclosporin, and tacrolimus in treating steroid resistant nephrotic syndrome. METHODS: Medline, Cochrane, EMBASE, and Google Scholar were searched until May 02, 2017 using the keywords: immunosuppressive therapy, steroid-resistant nephrotic syndrome, cyclophosphamide, cyclosporine A, and tacrolimus. Inclusion criteria were randomized controlled trials (RCTs) including patients with SRNS treated with an immunosuppressive therapy or placebo...
October 27, 2017: Clinical and Experimental Nephrology
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
#7
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/29058690/advillin-acts-upstream-of-phospholipase-c-%C3%AF%C2%B51-in-steroid-resistant-nephrotic-syndrome
#8
Jia Rao, Shazia Ashraf, Weizhen Tan, Amelie T van der Ven, Heon Yung Gee, Daniela A Braun, Krisztina Fehér, Sudeep P George, Amin Esmaeilniakooshkghazi, Won-Il Choi, Tilman Jobst-Schwan, Ronen Schneider, Johanna Magdalena Schmidt, Eugen Widmeier, Jillian K Warejko, Tobias Hermle, David Schapiro, Svjetlana Lovric, Shirlee Shril, Ankana Daga, Ahmet Nayir, Mohan Shenoy, Yincent Tse, Martin Bald, Udo Helmchen, Sevgi Mir, Afig Berdeli, Jameela A Kari, Sherif El Desoky, Neveen A Soliman, Arvind Bagga, Shrikant Mane, Mohamad A Jairajpuri, Richard P Lifton, Seema Khurana, Jose C Martins, Friedhelm Hildebrandt
Steroid-resistant nephrotic syndrome (SRNS) is a frequent cause of chronic kidney disease. Here, we identified recessive mutations in the gene encoding the actin-binding protein advillin (AVIL) in 3 unrelated families with SRNS. While all AVIL mutations resulted in a marked loss of its actin-bundling ability, truncation of AVIL also disrupted colocalization with F-actin, thereby leading to impaired actin binding and severing. Additionally, AVIL colocalized and interacted with the phospholipase enzyme PLCE1 and with the ARP2/3 actin-modulating complex...
October 23, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/29058154/clinical-and-genetic-heterogeneity-in-familial-steroid-sensitive-nephrotic-syndrome
#9
Guillaume Dorval, Olivier Gribouval, Vanesa Martinez-Barquero, Eduardo Machuca, Marie-Josèphe Tête, Véronique Baudouin, Stéphane Benoit, Imen Chabchoub, Gérard Champion, Dominique Chauveau, Hassib Chehade, Chokri Chouchane, Sylvie Cloarec, Pierre Cochat, Karin Dahan, Jacques Dantal, Yahsou Delmas, Georges Deschênes, Phillippe Dolhem, Dominique Durand, Zelal Ekinci, Khalil El Karoui, Michel Fischbach, Jean-Pierre Grunfeld, Vincent Guigonis, Mongia Hachicha, Julien Hogan, Maryvonne Hourmant, Aurélie Hummel, Nassim Kamar, Thierry Krummel, Didier Lacombe, Brigitte Llanas, Laurent Mesnard, Nabil Mohsin, Patrick Niaudet, Hubert Nivet, Paloma Parvex, Christine Pietrement, Loic de Pontual, Claire Pouteil Noble, David Ribes, Pierre Ronco, Eric Rondeau, Marion Sallee, Michel Tsimaratos, Tim Ulinski, Rémi Salomon, Corinne Antignac, Olivia Boyer
BACKGROUND: Familial steroid-sensitive nephrotic syndrome (SSNS) is a rare condition. The disease pathophysiology remains elusive. However, bi-allelic mutations in the EMP2 gene were identified, and specific variations in HLA-DQA1 were linked to a high risk of developing the disease. METHODS: Clinical data were analyzed in 59 SSNS families. EMP2 gene was sequenced in families with a potential autosomal recessive (AR) inheritance. Exome sequencing was performed in a subset of 13 families with potential AR inheritance...
October 23, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28979773/efficacy-of-mycophenolate-treatment-in-adults-with-steroid-dependent-frequently-relapsing-idiopathic-nephrotic-syndrome
#10
Diego Sandoval, Rafael Poveda, Juliana Draibe, Laureà Pérez-Oller, Montserrat Díaz, José Ballarín, Anna Saurina, Helena Marco, Josep Bonet, Xoana Barros, Xavier Fulladosa, Joan Torras, Josep M Cruzado
BACKGROUND: This study assessed the efficacy of therapy with mycophenolate (MF) and reduced doses of steroids in adults with steroid-dependent/frequently relapsing idiopathic nephrotic syndrome (SD/FR-INS). METHODS: Twenty-nine nephrotic patients (including 16 males and 13 females; mean age: 40 years, range: 18-74) were treated. Starting doses of MF were 2000 mg/day for mofetil MF (1500 mg/day in one patient) or 1440 mg/day for sodium MF. The initial prednisone (PDN) dose was 10 mg/day in 14 patients, 5 mg/day in two patients and no steroids in one patient...
October 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/28965242/uteroglobin-gene-polymorphism-g38a-may-be-a-risk-factor-in-childhood-idiopathic-nephrotic-syndrome
#11
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/28949280/childhood-nephrotic-syndrome-in-tropical-africa-then-and-now
#12
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
#13
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/28921387/analysis-of-24-genes-reveals-a-monogenic-cause-in-11-1-of-cases-with-steroid-resistant-nephrotic-syndrome-at-a-single-center
#14
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
https://www.readbyqxmd.com/read/28879428/difficult-to-treat-idiopathic-nephrotic-syndrome-established-drugs-open-questions-and-future-options
#15
REVIEW
Markus J Kemper, Lisa Valentin, Michael van Husen
The idiopathic nephrotic syndrome in childhood can be classified according to the International Study of Kidney Disease in Children (ISKDC) based on the response to steroids. Typically, steroid-sensitive nephrotic syndrome (SSNS) is characterised by minimal changes in disease (MCD) histology, whereas in steroid-resistant nephrotic syndrome (SRNS) focal segmental glomerulosclerosis (FSGS) is the most prevalent lesion. Patients with SSNS may develop frequent relapses and/or steroid dependency, which can be difficult to treat...
September 6, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28830667/plasma-exchange-in-kidney-transplantation-still-a-valuable-option-for-nephrotic-syndrome-recurrence
#16
REVIEW
Licia Peruzzi, Roberto Albiani, Karol Giancaspero
About 30% of the cases of steroid resistant nephrotic syndrome display a genetically determined disease and will not recur after kidney transplant; the other cases with fully or partially immunological pathogenesis display a high risk of post transplant recurrence. Although lots of studies were carried out in the last 50 years the pathogenetic mechanism is still obscure and the therapeutic approach mostly empirical. The cornerstones principles of the therapies are based on removal of a still undefined "permeability factor" through plasma-exchange or other apheresis techniques and inhibition of its synthesis by the immunological system through different drugs...
July 8, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28815356/dent-disease-in-poland-what-we-have-learned-so-far
#17
Marcin Zaniew, Małgorzata Mizerska-Wasiak, Iga Załuska-Leśniewska, Piotr Adamczyk, Katarzyna Kiliś-Pstrusińska, Adam Haliński, Jan Zawadzki, Beata S Lipska-Ziętkiewicz, Krzysztof Pawlaczyk, Przemysław Sikora, Michael Ludwig, Maria Szczepańska
PURPOSE: Dent disease (DD) is a rare tubulopathy characterized by proximal tubular dysfunction leading to chronic kidney disease (CKD). The aim of the study was to characterize patients with DD in Poland. METHODS: A retrospective analysis of a national cohort with genetically confirmed diagnosis. RESULTS: Of 24 males, all patients except one carried mutations in the CLCN5 gene; in one patient a mutation in the OCRL gene was disclosed. Molecular diagnosis was delayed 1 year on average (range 0-21 years)...
November 2017: International Urology and Nephrology
https://www.readbyqxmd.com/read/28803697/tumor-necrosis-factor-alpha-gene-polymorphisms-and-haplotypes-in-egyptian-children-with-nephrotic-syndrome
#18
Doaa M Youssef, Amal S El-Shal, Samia Hussein, Khaled Salah, Abd El Rahman E Ahmed
BACKGROUND: Nephrotic syndrome (NS) characterized by complex pathogenesis and clinical course with relapses; and needs novel breakthroughs for decades. Polymorphisms of cytokines genes including tumor necrosis factor alpha (TNF-α)may influence susceptibility to NS as well as different patients' steroid responses. In the current study, we demonstrated the potential roles of TNF-α promoter gene polymorphisms [-238, -308, -863] and haplotypes in susceptibility to childhood NS. Also, elucidating their possible influence on patients' steroid response and serum TNF-α level...
August 10, 2017: Cytokine
https://www.readbyqxmd.com/read/28796785/low-renal-but-high-extrarenal-phenotype-variability-in-schimke-immuno-osseous-dysplasia
#19
Beata S Lipska-Ziętkiewicz, Jutta Gellermann, Olivia Boyer, Olivier Gribouval, Szymon Ziętkiewicz, Jameela A Kari, Mohamed A Shalaby, Fatih Ozaltin, Jiri Dusek, Anette Melk, Aysun K Bayazit, Laura Massella, Lidia Hyla-Klekot, Sandra Habbig, Astrid Godron, Maria Szczepańska, Beata Bieniaś, Dorota Drożdż, Rasha Odeh, Wioletta Jarmużek, Katarzyna Zachwieja, Agnes Trautmann, Corinne Antignac, Franz Schaefer
Schimke immuno-osseous dysplasia (SIOD) is a rare multisystem disorder with early mortality and steroid-resistant nephrotic syndrome (SRNS) progressing to end-stage kidney disease. We hypothesized that next-generation gene panel sequencing may unsurface oligosymptomatic cases of SIOD with potentially milder disease courses. We analyzed the renal and extrarenal phenotypic spectrum and genotype-phenotype associations in 34 patients from 28 families, the largest SMARCAL1-associated nephropathy cohort to date. In 11 patients the diagnosis was made unsuspectedly through SRNS gene panel testing...
2017: PloS One
https://www.readbyqxmd.com/read/28792718/mdr-1-and-cyp3a5-polymorphisms-in-pediatric-idiopathic-nephrotic-syndrome-impact-on-susceptibility-and-response-to-steroids-preliminary-results
#20
Amira Moussa, Sameh Mabrouk, Haithem Hamdouni, Maroua Ajmi, Miniar Tfifha, Asma Omezzine, Saoussen Abroug, Ali Bouslama
BACKGROUND: Oral steroid treatment is the first line of therapy for childhood nephrotic syndrome (NS). However, resistance to this treatment has been observed in some patients. Here, we investigated the association of two steroid metabolism-related genes with susceptibility to childhood NS and the steroid response. METHODS: We genotyped the single nucleotide polymorphisms (SNP) of MDR-1 [C1236T (rs1128503), G2677T/A (rs2032582), and C3435T (rs1045642)] and the CYP3A5 gene (A6986G) in 63 NS patients and 110 age and gender matched controls by PCR-RFLP...
July 1, 2017: Clinical Laboratory
keyword
keyword
43341
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"