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https://www.readbyqxmd.com/read/28511470/study-on-steroid-induced-ocular-findings-in-children-with-nephrotic-syndrome
#1
Vijay Agrawal, Kusum Devpura, Laxmikant Mishra, Sajan Agarwal
INTRODUCTION: Long term use of corticosteroids in Nephrotic Syndrome (NS) is associated with ocular complications such as Posterior Subcapsular Cataract (PSC), glaucoma, increased intra-ocular pressure, ptosis, mydriasis, eyelid skin atrophy, keratisis, thinning of cornea and sclera, repeated hordeolum exacerbations. AIM: To determine ocular abnormalities in children with NS and their association with steroid dosing and duration. MATERIALS AND METHODS: Hundred patients with NS in the age group of 2-18 years, with no evidence of other systemic disease, were considered for the study...
March 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28508981/partial-remission-by-cyclosporine-monotherapy-in-a-patient-with-membranous-nephropathy-superimposed-diabetic-nephropathy
#2
Yoshiyuki Oshiro, Hisataka Tanaka, Fumiko Kawasaki, Niro Okimoto
It has been noted that cyclosporine A (CsA) is an effective drug for membranous nephropathy (MN). Diabetes is a common disease that sometimes causes nephrotic syndrome. We report the case of an 89-year-old woman with type 2 diabetes mellitus who exhibited nephrotic syndrome. Examination of a renal biopsy indicated MN and she was prescribed CsA as monotherapy. Her edema subsided and she achieved partial remission. This is the first report of a patient in diabetic condition with MN having achieved partial remission after CsA monotherapy without steroid therapy...
November 2016: CEN Case Reports
https://www.readbyqxmd.com/read/28487395/safety-of-rituximab-compared-with-steroids-and-cyclophosphamide-for-idiopathic-membranous-nephropathy
#3
Jan A J G van den Brand, Piero Ruggenenti, Antonietta Chianca, Julia M Hofstra, Annalisa Perna, Barbara Ruggiero, Jack F M Wetzels, Giuseppe Remuzzi
Guidelines recommend steroid plus cyclical cyclophosphamide (St-Cp) therapy for patients with idiopathic membranous nephropathy at high risk of progression to ESRD. Rituximab (Rtx) may be a safer alternative. In this retrospective, observational cohort study, we compared time to any adverse event (primary outcome); serious or nonserious events; partial and complete remission of the nephrotic syndrome; and a composite of doubling of serum creatinine, ESRD, or death between 100 Rtx-treated patients and 103 patients who received daily St-Cp We monitored patients with standardized protocols and adjusted for baseline characteristics by Cox regression...
May 9, 2017: Journal of the American Society of Nephrology: JASN
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
#4
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
#5
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
#6
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/28466679/effect-of-two-prophylactic-bolus-vitamin-d-dosing-regimens-1000%C3%A2-iu-day-vs-400%C3%A2-iu-day-on-bone-mineral-content-in-new-onset-and-infrequently-relapsing-nephrotic-syndrome-a-randomised-clinical-trial
#7
Sravani Muske, Sriram Krishnamurthy, Sadish Kumar Kamalanathan, Medha Rajappa, K T Harichandrakumar, Palanisamy Sivamurukan
OBJECTIVES: To examine the efficacy of two vitamin D dosages (1000 vs. 400 IU/day) for osteoprotection in children with new-onset and infrequently-relapsing nephrotic syndrome (IFRNS) receiving corticosteroids. METHODS: This parallel-group, open label, randomised clinical trial enrolled 92 children with new-onset nephrotic syndrome (NS) (n = 28) or IFRNS (n = 64) to receive 1000 IU/day (Group A, n = 46) or 400 IU/day (Group B, n = 46) vitamin D (administered as a single bolus initial supplemental dose) by block randomisation in a 1:1 allocation ratio...
May 3, 2017: Paediatrics and International Child Health
https://www.readbyqxmd.com/read/28457111/serum-tumor-necrosis-factor-alpha-levels-in-children-with-nephrotic-syndrome-a-pilot-study
#8
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
#9
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
#10
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
#11
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/28416778/steroid-pulse-therapy-for-de-novo-minimal-change-disease-during-pregnancy
#12
Hiroshi Sato, Yuna Asami, Reona Shiro, Masato Aoki, Miki Yasuda, Saeko Imai, Rie Sakai, Kenji Oida, Kanako Kawaharamura, Hiroko Yano, Nao Taguchi, Takako Suzuki, Masaya Hirose
BACKGROUND Nephrotic syndrome occurs very rarely, in only about 0.01%-0.02% of all pregnancies, and de novo minimal change disease during pregnancy is especially rare. Nephrotic syndrome and, especially, minimal change disease are highly responsive to steroids, and preterm labor may be avoidable if the maternal condition is improved with steroid therapy. Therefore, prompt diagnosis and proper management are critical to maternal and fetal outcome when severe proteinuria occurs during pregnancy. CASE REPORT A 30-year-old pregnant Japanese woman presented with systemic edema, oliguria, and severe proteinuria and hypoalbuminemia at 25 weeks of gestation, although she was normotensive...
April 18, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28408801/effects-of-%C3%AE-d-mannuronic-acid-as-a-novel-non-steroidal-anti-inflammatory-medication-within-immunosuppressive-properties-on-il17-ror%C3%AE-t-il4-and-gata3-gene-expressions-in-rheumatoid-arthritis-patients
#13
Anis Barati, Ahmad Reza Jamshidi, Hossein Ahmadi, Zahra Aghazadeh, Abbas Mirshafiey
Rheumatoid arthritis (RA) is the most common form of chronic inflammatory arthritis characterized by pain, swelling and destruction of joints, with a resultant disability. Disease-modifying anti-rheumatic drugs (DMARDs) and biological drugs can interfere with the disease process. In this study, the effect of β-d-mannuronic acid (M2000) as a novel non-steroidal anti-inflammatory drug (NSAID) with immunosuppressive and anti-inflammatory effects together with antioxidant effects was evaluated on IL17, RORγt, IL4 and GATA3 gene expression in 12 RA patients...
2017: Drug Design, Development and Therapy
https://www.readbyqxmd.com/read/28405841/focal-segmental-glomerulosclerosis-and-medullary-nephrocalcinosis-in-children-with-adck4-mutations
#14
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
#15
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/28387313/cost-effectiveness-of-administering-rituximab-for-steroid-dependent-nephrotic-syndrome-and-frequently-relapsing-nephrotic-syndrome-a-preliminary-study-in-japan
#16
Tomoyuki Takura, Takashi Takei, Kosaku Nitta
With regard to the use of rituximab for patients with steroid-dependent nephrotic syndrome and frequently relapsing nephrotic syndrome, not only has the regimen not been clinically verified but also there is a lack of health economics evidence. Therefore, we conducted a prospective clinical study on 30 patients before (with steroids and immunosuppressants) and after introducing rituximab therapy. Relapse rates and total invoiced medical expenses were selected as the primary endpoints for treatment effectiveness and treatment costs, respectively...
April 7, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28385484/genetic-mutation-in-egyptian-children-with-steroid-resistant-nephrotic-syndrome
#17
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/28378029/nephrotoxicity-in-children-with-frequently-relapsing-nephrotic-syndrome-receiving-long-term-cyclosporine-treatment
#18
Yuko Hamasaki, Fumiyo Komaki, Kenji Ishikura, Riku Hamada, Tomoyuki Sakai, Hiroshi Hataya, Kentaro Ogata, Takashi Ando, Masataka Honda
BACKGROUND: Steroid-sparing drugs, such as cyclosporine, are recommended as treatment for children with frequently relapsing nephrotic syndrome (FRNS) and steroid-related toxicities. We recently reported a high rate of relapsing nephrotic syndrome 2 years after discontinuation of cyclosporine treatment, suggesting that long-term treatment is necessary. Cyclosporine-associated nephrotoxicity (CAN) is a potential side effect of long-term cyclosporine treatment. METHODS: We retrospectively reviewed pediatric patients with FRNS treated with cyclosporine for ≥3 years at a single center between 1999 and 2012...
April 4, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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
#19
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/28341877/short-courses-of-daily-prednisolone-during-upper-respiratory-tract-infections-reduce-relapse-frequency-in-childhood-nephrotic-syndrome
#20
Asiri S Abeyagunawardena, R S Thalgahagoda, Pathum V Dissanayake, Shamali Abeyagunawardena, Y A Illangasekera, Umeshi I Karunadasa, Richard S Trompeter
BACKGROUND: Relapses of childhood nephrotic syndrome (NS) are frequently precipitated by viral upper respiratory tract infections (URTIs). A review of the literature reveals that in patients with steroid-dependent NS on alternate day corticosteroids, a short course of daily corticosteroid therapy during the course of an URTI may reduce relapse frequency. OBJECTIVE: To assess the effect of a short course of low-dose corticosteroid therapy during the course of an URTI on relapse frequency in patients with steroid-sensitive NS who have not been taking any treatment for a minimum period of 3 months...
March 24, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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