keyword
MENU ▼
Read by QxMD icon Read
search

steroid resistant nephrotic syndrome

keyword
https://www.readbyqxmd.com/read/28529802/r229q-polymorphism-of-nphs2-gene-in-group-of-iraqi-children-with-steroid-resistant-nephrotic-syndrome
#1
Shatha Hussain Ali, Rasha Kasim Mohammed, Hussein Ali Saheb, Ban A Abdulmajeed
Background. The polymorphism R229Q is one of the most commonly reported podocin sequence variations among steroid-resistant nephrotic syndromes (SRNS). Aim of the Study. We investigated the frequency and risk of this polymorphism among a group of Iraqi children with SRNS and steroid-sensitive nephrotic syndrome (SSNS). Patients and Methods. A prospective case control study which was conducted in Al-Imamein Al-Kadhimein Medical City, spanning the period from the 1st of April 2015 to 30th of November 2015. Study sample consisted of 54 children having NS, divided into 2 groups: patients group consisted of 27 children with SRNS, and control group involved 27 children with SSNS...
2017: International Journal of Nephrology
https://www.readbyqxmd.com/read/28483237/biochemical-alteration-in-children-with-idiopathic-nephrotic-syndrome-associated-with-an-increased-risk-of-sensorineural-hearing-loss-additional-insights-in-cochlear-renal-relationship
#2
Ghada Mohamed El Mashad, Wafaa Moustafa M Abo El Fotoh, Ahmed Mahmoud Zein El Abedein, Fatma Abd El Raoof Abd El Sadek
OBJECTIVES: Children with Idiopathic Nephrotic Syndrome (INS) are at risk of hearing loss due to the adverse impact of medications and related immunological and genetic factors on both cochlea and kidney. So this work was planned to evaluate hearing status in children with INS and to clarify the possible associated risk factors by interpreting the clinical and laboratory profiles of those children. METHODS: Ninety children with INS aged 5-14 years [30 patients with steroid-sensitive nephrotic syndrome (SSNS), 30 patients with steroid dependent/frequently relapsing nephrotic syndrome (SDNS/FRNS), and 30 patients with steroid-resistant nephrotic syndrome (SRNS)], and 90 age and sex matched normal controls were enrolled into this study...
June 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28476686/mutation-spectrum-of-genes-associated-with-steroid-resistant-nephrotic-syndrome-in-chinese-children
#3
Ying Wang, Xiqiang Dang, Qingnan He, Yan Zhen, Xiaoxie He, Zhuwen Yi, Kuichun Zhu
Approximately 20% of children with idiopathic nephrotic syndrome do not respond to steroid therapy. More than 30 genes have been identified as disease-causing genes for the steroid-resistant nephrotic syndrome (SRNS). Few reports were from the Chinese population. The coding regions of genes commonly associated with SRNS were analyzed to characterize the gene mutation spectrum in children with SRNS in central China. The first phase study involved 38 children with five genes (NPHS1, NPHS2, PLCE1, WT1, and TRPC6) by Sanger sequencing...
May 2, 2017: Gene
https://www.readbyqxmd.com/read/28469399/a-novel-biomarker-panel-to-identify-steroid-resistance-in-childhood-idiopathic-nephrotic-syndrome
#4
Michael R Bennett, LaTawnya Pleasant, Christopher Haffner, Qing Ma, Wendy D Haffey, Jun Ying, Michael Wagner, Kenneth D Greis, Prasad Devarajan
Idiopathic nephrotic syndrome (NS) is the most common glomerular disorder of childhood. Response to initial treatment with corticosteroids is an indicator of prognosis, as resistant patients often present more progressive disease. In this cross-sectional pilot study, we set out to discover a panel of noninvasive biomarkers that could distinguish steroid-resistant nephrotic syndrome (SRNS) from steroid-sensitive nephrotic syndrome (SSNS). Information gleaned from such a panel could yield more individualized treatment plans and prevent unnecessary steroid exposure in patients unlikely to respond...
2017: Biomarker Insights
https://www.readbyqxmd.com/read/28457111/serum-tumor-necrosis-factor-alpha-levels-in-children-with-nephrotic-syndrome-a-pilot-study
#5
Avichai Weissbach, Ben Zion Garty, Irina Lagovsky, Irit Krause, Miriam Davidovits
BACKGROUND: Several studies link the pathogenesis of nephrotic syndrome to tumor necrosis factor-alpha (TNFα). However, data on the serum TNFα level in children with nephrotic syndrome are sparse. OBJECTIVES: To investigate serum TNFα levels and the effect of steroid therapy in children with nephrotic syndrome. METHODS: A prospective cohort pilot study of children with nephrotic syndrome and controls was conducted during a 1 year period...
January 2017: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/28450305/mechanisms-in-endocrinology-update-on-pathogenesis-of-primary-adrenal-insufficiency-beyond-steroid-enzyme-deficiency-and-autoimmune-adrenal-destruction
#6
Christa Flueck
Primary adrenal insufficiency (PAI) is potentially life threatening, but rare. In children genetic defects prevail, while adults suffer mostly from acquired forms. The spectrum of genetic defects has increased in recent years with the use of next generation sequencing methods and reaches now far beyond genetic defects in known enzymes of steroidogenesis. Cofactor disorders such as P450 oxidoreductase (POR) deficiency manifesting as a complex form of congenital adrenal hyperplasia with a broad clinical phenotype have come to the forth...
April 27, 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28434126/relapse-of-nephrotic-syndrome-during-post-rituximab-peripheral-blood-b-lymphocyte-depletion
#7
Mai Sato, Koichi Kamei, Masao Ogura, Kenji Ishikura, Shuichi Ito
BACKGROUND: Rituximab is effective against complicated childhood steroid-dependent nephrotic syndrome (SDNS). Peripheral blood B-lymphocyte (B-cell) depletion is strongly correlated with persistent remission, relapse rarely occurring during B-cell depletion; however, we have encountered several such patients. METHODS: We retrospectively analyzed the characteristics and clinical course of 82 patients with SDNS treated with rituximab from January 2007 to December 2012 in our institution...
April 22, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/28427453/the-italian-society-for-pediatric-nephrology-sinepe-consensus-document-on-the-management-of-nephrotic-syndrome-in-children-part-i-diagnosis-and-treatment-of-the-first-episode-and-the-first-relapse
#8
REVIEW
Andrea Pasini, Elisa Benetti, Giovanni Conti, Luciana Ghio, Marta Lepore, Laura Massella, Daniela Molino, Licia Peruzzi, Francesco Emma, Carmelo Fede, Antonella Trivelli, Silvio Maringhini, Marco Materassi, Giovanni Messina, Giovanni Montini, Luisa Murer, Carmine Pecoraro, Marco Pennesi
This consensus document is aimed at providing an updated, multidisciplinary overview on the diagnosis and treatment of pediatric nephrotic syndrome (NS) at first presentation. It is the first consensus document of its kind to be produced by all the pediatric nephrology centres in Italy, in line with what is already present in other countries such as France, Germany and the USA. It is based on the current knowledge surrounding the symptomatic and steroid treatment of NS, with a view to providing the basis for a separate consensus document on the treatment of relapses...
April 21, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/28405841/focal-segmental-glomerulosclerosis-and-medullary-nephrocalcinosis-in-children-with-adck4-mutations
#9
Eujin Park, Hee Gyung Kang, Young Hun Choi, Kyoung Bun Lee, Kyung Chul Moon, Hyeon Joo Jeong, Michio Nagata, Hae Il Cheong
BACKGROUND: Mutations in the AarF domain containing kinase 4 gene (ADCK4), one of the novel genes causing steroid-resistant nephrotic syndrome (SRNS), usually manifest as isolated adolescent-onset focal segmental glomerulosclerosis (FSGS). ADCK4 interacts with components of the coenzyme Q10 (CoQ10) biosynthesis pathway. METHODS: The incidence and phenotypes of patients with ADCK4 mutations were investigated in a cohort of Korean pediatric patients with SRNS. RESULTS: Among the 53 patients enrolled in the study the incidence of ADCK4-associated FSGS was 7...
April 12, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28392820/genetics-of-hereditary-nephrotic-syndrome-a-clinical-review
#10
REVIEW
Tae-Sun Ha
Advances in podocytology and genetic techniques have expanded our understanding of the pathogenesis of hereditary steroid-resistant nephrotic syndrome (SRNS). In the past 20 years, over 45 genetic mutations have been identified in patients with hereditary SRNS. Genetic mutations on structural and functional molecules in podocytes can lead to serious injury in the podocytes themselves and in adjacent structures, causing sclerotic lesions such as focal segmental glomerulosclerosis or diffuse mesangial sclerosis...
March 2017: Korean Journal of Pediatrics
https://www.readbyqxmd.com/read/28385484/genetic-mutation-in-egyptian-children-with-steroid-resistant-nephrotic-syndrome
#11
Manal Micheal Thomas, Mohamed S Abdel-Hamid, Nermine Nabil Mahfouz, Emad Emil Ghobrial
BACKGROUND/PURPOSE: Nephrotic syndrome is the commonest etiology of proteinuria in children. Steroid-resistant nephrotic syndrome (SRNS) is defined by resistance to standard steroid therapy, and it continues to be one of the most intractable etiologies of renal failure. Molecular studies discovered specialized molecules in podocytes that play a role in proteinuria. Mutations in NPHS2 that encodes for podocin constitute a frequent cause of SRNS worldwide. This study aimed to screen for podocin mutations in SRNS Egyptian children and their parents...
April 3, 2017: Journal of the Formosan Medical Association, Taiwan Yi Zhi
https://www.readbyqxmd.com/read/28352006/assay-of-urinary-protein-carbonyl-content-can-predict-the-steroid-dependence-and-resistance-in-children-with-idiopathic-nephrotic-syndrome
#12
Niranjan Gopal, Bidhan Chandra Koner, Atanu Bhattacharjee, Vishnu Bhat, Sathish Babu Murugaiyan, Prakash H Muddegowda
Nephrotic syndrome in pediatric age is mostly idiopathic. Idiopathic nephrotic syndrome (INS) by default is treated with steroids from the very beginning. Some do not respond to steroids and are grouped later as either steroid-resistant (SR) or steroid-dependent (SD) cases. The protein selectivity index often fails to predict the SR and SD cases. The SD and SR cases of INS exhibit higher degrees of oxidative stress compared to steroid responders. Proteins get carbonylated when they are exposed to free radicals...
March 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28337616/follow-up-results-of-patients-with-adck4-mutations-and-the-efficacy-of-coq10-treatment
#13
Mustafa Atmaca, Bora Gulhan, Emine Korkmaz, Mihriban Inozu, Oguz Soylemezoglu, Cengiz Candan, Aysun Karabay Bayazıt, Ahmet Midhat Elmacı, Gonul Parmaksiz, Ali Duzova, Nesrin Besbas, Rezan Topaloglu, Fatih Ozaltin
BACKGROUND: ADCK4-related glomerulopathy is an important differential diagnosis in adolescents with steroid-resistant nephrotic syndrome (SRNS) and/or chronic kidney disease (CKD) of unknown origin. We screened adolescent patients to determine the frequency of ADCK4 mutation and the efficacy of early CoQ10 administration. METHODS: A total of 146 index patients aged 10-18 years, with newly diagnosed non-nephrotic proteinuria, nephrotic syndrome, or chronic renal failure and end-stage kidney disease (ESKD) of unknown etiology were screened for ADCK4 mutation...
March 24, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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
#14
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/28326197/childhood-nephrotic-syndrome-management-and-outcome-a-single-center-retrospective-analysis
#15
Chia-Shi Wang, Jia Yan, Robert Palmer, James Bost, Mattie Feasel Wolf, Larry A Greenbaum
There is a paucity of information on outpatient management and risk factors for hospitalization and complications in childhood nephrotic syndrome (NS). We described the management, patient adherence, and inpatient and outpatient usage of 87 pediatric NS patients diagnosed between 2006 and 2012 in the Atlanta Metropolitan Statistical Area. Multivariable analyses were performed to examine the associations between patient characteristics and disease outcome. We found that 51% of the patients were treated with two or more immunosuppressants...
2017: International Journal of Nephrology
https://www.readbyqxmd.com/read/28321320/calcineurin-inhibitors-in-the-treatment-of-primary-focal-segmental-glomerulosclerosis-a-systematic-review-and-meta-analysis-of-the-literature
#16
REVIEW
Louis-Philippe Laurin, Patrick H Nachman, Bethany J Foster
PURPOSE OF REVIEW: Primary focal segmental glomerulosclerosis (FSGS) is the most common cause of nephrotic syndrome in adults. Glucocorticoids have been evaluated in the treatment of primary FSGS in numerous retrospective studies. Evidence suggesting a role for including calcineurin inhibitors (CNIs) in early therapy remains limited. The aim of this study was to systematically review the literature examining the efficacy of CNIs in the treatment of primary FSGS both as first-line therapy and as an adjunctive agent in steroid-resistant patients, with respect to remission in proteinuria and renal survival...
2017: Canadian Journal of Kidney Health and Disease
https://www.readbyqxmd.com/read/28318625/mycophenolate-mofetil-is-inferior-to-tacrolimus-in-sustaining-remission-in-children-with-idiopathic-steroid-resistant-nephrotic-syndrome
#17
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/28314579/keeping-the-customers-stratified-moving-toward-genetics-based-treatment-options-in-childhood-ns
#18
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/28303389/variability-of-diagnostic-criteria-and-treatment-of-idiopathic-nephrotic-syndrome-across-european-countries
#19
Georges Deschênes, Marina Vivarelli, Licia Peruzzi
The aim of the surveys conducted by the Idiopathic Nephrotic Syndrome Working Group of the ESPN was to study the possible variability of treatment in Europe at different stages of the disease by means of questionnaires sent to members of the Working Group. Four surveys have been completed: treatment of the first flare, treatment of the first relapse and the issue of steroid dependency, use of rituximab, and the management of steroid-resistant patients. A uniform treatment of the first flare was applied in only three countries, and ten additional centers have adopted one of the three main protocols...
May 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28299339/cytoplasmic-localization-of-wt1-and-decrease-of-mirna-16-1-in-nephrotic-syndrome
#20
Pablo Zapata-Benavides, Mariela Arellano-Rodríguez, Juan José Bollain-Y-Goytia, Moisés Armides Franco-Molina, Gloria Azucena Rangel-Ochoa, Esperanza Avalos-Díaz, Rafael Herrera-Esparza, Cristina Rodríguez-Padilla
Nephrotic syndrome (NS) is a glomerular disease that is defined by the leakage of protein into the urine and is associated with hypoalbuminemia, hyperlipidemia, and edema. Steroid-resistant NS (SRNS) patients do not respond to treatment with corticosteroids and show decreased Wilms tumor 1 (WT1) expression in podocytes. Downregulation of WT1 has been shown to be affected by certain microRNAs (miRNAs). Twenty-one patients with idiopathic NS (68.75% were SSNS and 31.25% SRNS) and 10 healthy controls were enrolled in the study...
2017: BioMed Research International
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"