keyword
MENU ▼
Read by QxMD icon Read
search

SRNS

keyword
https://www.readbyqxmd.com/read/27885584/wt1-and-nphs2-gene-mutation-analysis-and-clinical-management-of-steroid-resistant-nephrotic-syndrome
#1
Aravind Selvin Kumar Ramanathan, Murali Vijayan, Srilakshmi Rajagopal, Padmaraj Rajendiran, Prabha Senguttuvan
Nephrotic syndrome (NS) is a kidney disease predominantly present in children with idiopathic condition; final stage of the disease progresses into end-stage renal disease. Generally, NS is treated using standard steroid therapy, however; most of the children are steroid sensitive and about 15-20% are non-responders (SRNS). Non-responsiveness of these children would be a risk with the possibility of mutational changes in podocyte genes (NPHS1, NPHS2, WT1, PLCE1). The mutation in podocyte genes is associated with SRNS...
November 25, 2016: Molecular and Cellular Biochemistry
https://www.readbyqxmd.com/read/27821253/bone-mineral-density-in-children-with-idiopathic-nephrotic-syndrome
#2
Ghada Mohamed El-Mashad, Mahmoud Ahmed El-Hawy, Sally Mohamed El-Hefnawy, Sanaa Mansour Mohamed
OBJECTIVES: To assess bone mineral density (BMD) in children with idiopathic nephrotic syndrome (NS) and normal glomerular filtration rate (GFR). METHODS: Cross-sectional case-control study carried out on 50 children: 25 cases of NS (16 steroid-sensitive [SSNS] and nine steroid-resistant [SRNS] under follow up in the pediatric nephrology unit of Menoufia University Hospital, which is tertiary care center, were compared to 25 healthy controls with matched age and sex...
November 5, 2016: Jornal de Pediatria
https://www.readbyqxmd.com/read/27806747/children-with-steroid-resistant-nephrotic-syndrome-long-term-outcomes-of-sequential-steroid-therapy
#3
Hui Zhang, Zheng Wang, Li Qun Dong, Yan Nan Guo
OBJECTIVE: This study aimed to investigate the long-term outcomes in children with steroid-resistant nephrotic syndrome (SRNS), who received methylprednisolone pulse therapy (MPT)-based sequential steroid therapy. In particular, we aimed to observe whether these patients had a high risk of adverse events. METHODS: We conducted a retrospective study over a 5-year period. The long-term outcomes for children with SRNS receiving sequential therapy were observed. RESULTS: Sixty-three children were diagnosed with SRNS and underwent MPT-based sequential steroid therapy...
September 2016: Biomedical and Environmental Sciences: BES
https://www.readbyqxmd.com/read/27783276/mizoribine-therapy-combined-with-steroids-and-mizoribine-blood-concentration-monitoring-for-idiopathic-membranous-nephropathy-with-steroid-resistant-nephrotic-syndrome
#4
Takao Saito, Masayuki Iwano, Koichi Matsumoto, Tetsuya Mitarai, Hitoshi Yokoyama, Noriaki Yorioka, Shinichi Nishi, Ashio Yoshimura, Hiroshi Sato, Satoru Ogahara, Yoshie Sasatomi, Yasufumi Kataoka, Shiro Ueda, Akio Koyama, Shoichi Maruyama, Masaomi Nangaku, Enyu Imai, Seiichi Matsuo, Yasuhiko Tomino
BACKGROUND: We designed a prospective and randomized trial of mizoribine (MZR) therapy combined with prednisolone (PSL) for idiopathic membranous nephropathy (IMN) with steroid-resistant nephrotic syndrome (SRNS). METHODS: Patients with IMN were divided into 2 groups, and MZR combined with PSL was administered for 2 years. PSL was initially prescribed at 40 mg/day and tapered. MZR was given once-a-day at 150 mg and 3-times-a-day at 50 mg each to groups 1 and 2...
October 25, 2016: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/27760769/a-small-molecule-screening-to-detect-potential-therapeutic-targets-in-human-podocytes
#5
Eugen Widmeier, Weizhen Tan, Merlin Airik, Friedhelm Hildebrandt
INTRODUCTION: Steroid-resistant nephrotic syndrome (SRNS) inevitably progresses to end-stage kidney disease, requiring dialysis or transplantation for survival. However, treatment modalities and drug discovery remain limited. Mutations in over 30 genes have been discovered as monogenic causes of SRNS. Most of these genes are predominantly expressed in the glomerular epithelial cell, the podocyte, placing it at the center of the pathogenesis of SRNS. Podocyte migration rate (PMR) represents a relevant intermediate phenotype of disease in monogenic causes of SRNS...
October 19, 2016: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/27726125/interventions-for-idiopathic-steroid-resistant-nephrotic-syndrome-in-children
#6
REVIEW
Elisabeth M Hodson, Sophia C Wong, Narelle S Willis, Jonathan C Craig
BACKGROUND: The majority of children who present with their first episode of nephrotic syndrome achieve remission with corticosteroid therapy. Children who fail to respond may be treated with immunosuppressive agents including calcineurin inhibitors (cyclosporin or tacrolimus) and with non-immunosuppressive agents such as angiotensin-converting enzyme inhibitors (ACEi). Optimal combinations of these agents with the least toxicity remain to be determined. This is an update of a review first published in 2004 and updated in 2006 and 2010...
October 11, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27719739/early-recognition-of-gonadal-dysgenesis-in-congenital-nephrotic-syndrome%C3%A2
#7
Supamit Ukarapong, Gary Berkovitz, Kenneth McElreavey, Anu Bashamboo, Yong Bao
Mutation of the Wilms tumor suppressor gene (WT1) has been recognized as one of the etiologies of steroid-resistant nephrotic syndrome (SRNS). The mutation is also responsible for gonadal dysgenesis in 46,XY individuals. Early recognition of the presence of Y chromosome is of particular importance because of the high risk of gonadal tumor. We present here three cases of steroid-resistant nephrotic syndrome with WT1 mutation and 46,XY karyotype. Patient 1 and 2 have intron splice site (IVS9+5G > A) mutation...
October 10, 2016: Clinical Nephrology
https://www.readbyqxmd.com/read/27617138/the-genetics-of-nephrotic-syndrome
#8
REVIEW
Michelle N Rheault, Rasheed A Gbadegesin
Nephrotic syndrome (NS) is a common pediatric kidney disease and is defined as massive proteinuria, hypoalbuminemia, and edema. Dysfunction of the glomerular filtration barrier, which is made up of endothelial cells, glomerular basement membrane, and visceral epithelial cells known as podocytes, is evident in children with NS. While most children have steroid-responsive nephrotic syndrome (SSNS), approximately 20% have steroid-resistant nephrotic syndrome (SRNS) and are at risk for progressive kidney dysfunction...
March 2016: Journal of Pediatric Genetics
https://www.readbyqxmd.com/read/27589479/supraspinal-control-of-spinal-reflex-responses-to-body-bending-during-different-behaviours-in-lampreys
#9
Li-Ju Hsu, Pavel V Zelenin, Grigori N Orlovsky, Tatiana G Deliagina
KEY POINTS: Spinal reflexes are substantial components of the motor control system in all vertebrates and centrally driven reflex modifications are essential to many behaviours, but little is known about the neuronal mechanisms underlying these modifications. To study this issue, we took advantage of an in vitro brainstem-spinal cord preparation of the lamprey (a lower vertebrate), in which spinal reflex responses to spinal cord bending (caused by signals from spinal stretch receptor neurons) can be evoked during different types of fictive behaviour...
September 2, 2016: Journal of Physiology
https://www.readbyqxmd.com/read/27573339/novel-nphs2-variant-in-patients-with-familial-steroid-resistant-nephrotic-syndrome-with-early-onset-slow-progression-and-dominant-inheritance-pattern
#10
Maija Suvanto, Jaakko Patrakka, Timo Jahnukainen, Pia-Maria Sjöström, Matti Nuutinen, Pekka Arikoski, Janne Kataja, Marjo Kestilä, Hannu Jalanko
BACKGROUND: Steroid-resistant nephrotic syndrome (SRNS) is a common cause of end-stage renal disease in children but also occurs as an adult-onset condition. In a subset of SRNS patients, pathogenic variants are found in genes coding for podocyte foot process proteins. The aim of this study was to define the role of pathogenic variants in Finnish patients with familial and sporadic SRNS. METHODS: We analyzed SRNS-related genes NPHS1, NPHS2, NEPH1, ACTN4, TRPC6, INF2, WT1, CD2AP, LAMB2, and PLCE1 for disease-causing variants using direct sequencing of exons and intron/exon boundaries in all members of a family with dominant SRNS with early onset and slow progression to end-stage renal disease...
August 29, 2016: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/27504551/comparison-of-efficacy-of-tacrolimus-versus-cyclosporine-in-childhood-steroid-resistant-nephrotic-syndrome
#11
Syed Sajid Hussain Shah, Farkhanda Hafeez
OBJECTIVE: To compare the efficacy of tacrolimus versus cyclosporine (Calcineurin Inhibitors) in the management of childhood steroid-resistant nephritic syndrome (SRNS). STUDY DESIGN: Quasi-experimental study. PLACE AND DURATION OF STUDY: Department of Paediatric Nephrology at The Children's Hospital and Institute of Child Health, Lahore, from August 2014 to September 2015. METHODOLOGY: Patients of either gender aged 1 - 12 years, with the diagnosis of mesangioproliferative glomerulonephritis (MesangioPGN), focal segmental glomerulosclerosis (FSGS) or minimal-change disease (MCD) were included...
July 2016: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/27470160/genetics-of-childhood-steroid-sensitive-nephrotic-syndrome
#12
Alana M Karp, Rasheed A Gbadegesin
The pathogenesis of childhood-onset nephrotic syndrome (NS), disparity in incidence of NS among races, and variable responses to therapies in children with NS have defied explanation to date. In the last 20 years over 50 genetic causes of steroid-resistant nephrotic syndrome (SRNS) have been identified, and at least two disease loci for two pathologic variants of SRNS (focal segmental glomerulosclerosis and membranous nephropathy) have been defined. However, the genetic causes and risk loci for steroid-sensitive nephrotic syndrome (SSNS) remain elusive, partly because SSNS is relatively rare and also because cases of SSNS vary widely in phenotypic expression over time...
July 29, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/27442391/steroid-resistant-nephrotic-syndrome-genetic-consideration
#13
REVIEW
Velibor Tasic, Zoran Gucev, Momir Polenakovic
Nephrotic syndrome is defined as the association of massive proteinuria, hypoalbuminaemia, edema, and hyperlipidemia. It is separated to steroid-sensitive or steroid-resistant (SRNS) forms in respect to the response to intensive steroid therapy. SRNS usually progresses to end-stage renal failure. According to the North American Pediatric Renal Trials and Collaborative Studies SRNS constitutes the second most frequent cause of ESRD in the first two decades of life. Unfortunately, there is no curative treatment for majority of patients...
2015: Prilozi (Makedonska Akademija Na Naukite i Umetnostite. Oddelenie za Medicinski Nauki)
https://www.readbyqxmd.com/read/27422620/rituximab-for-nephrotic-syndrome-in-children
#14
Kazumoto Iijima, Mayumi Sako, Kandai Nozu
Idiopathic nephrotic syndrome is the most common chronic glomerular disease in children. At least 20 % of children with this syndrome show frequent relapses and/or steroid dependence during or after immunosuppressive therapies, a condition defined as complicated frequently relapsing/steroid-dependent nephrotic syndrome (FRNS/SDNS). Approximately 1-3 % of children with idiopathic nephrotic syndrome are resistant to steroids and all immunosuppressive agents, a condition defined as refractory steroid-resistant nephrotic syndrome (SRNS); these SRNS children have a high risk of end-stage renal failure...
July 15, 2016: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/27319672/directions
#15
(no author information available yet)
Occupational health nursing is one area of the profession that appeals to Registered General Nurses/State Registered Nurses (RGNs/SRNs) who wish to remain within nursing but for personal reasons must relocate or to accommodate family obligations and responsibilities must tailor their working time to hours which may be unavailable or impractical in other settings.
October 31, 1987: Nursing Standard
https://www.readbyqxmd.com/read/27300205/genotype-phenotype-analysis-of-pediatric-patients-with-wt1-glomerulopathy
#16
Yo Han Ahn, Eu Jin Park, Hee Gyung Kang, Seong Heon Kim, Hee Yeon Cho, Jae Il Shin, Joo Hoon Lee, Young Seo Park, Kyo Sun Kim, Il-Soo Ha, Hae Il Cheong
BACKGROUND: WT1 is one of the genes commonly reported as mutated in children with steroid-resistant nephrotic syndrome (SRNS). We analyzed genotype-phenotype correlations in pediatric SRNS patients with WT1 mutations. METHODS: From 2001 to 2015, WT1 mutations were detected in 21 out of 354 children with SRNS by genetic screening (5.9 %). The patients were grouped into missense (n = 11) and KTS splicing (n = 10) mutation groups. RESULTS: Nine (82 %) patients with missense mutations presented with congenital/infantile nephrotic syndrome, while 8 (80 %) with KTS splicing mutations presented with childhood-onset SRNS...
June 14, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/27252935/long-term-outcomes-of-childhood-onset-nephrotic-syndrome
#17
REVIEW
Rebecca Hjorten, Zohra Anwar, Kimberly Jean Reidy
There are limited studies on long-term outcomes of childhood onset nephrotic syndrome (NS). A majority of children with NS have steroid-sensitive nephrotic syndrome (SSNS). Steroid-resistant nephrotic syndrome (SRNS) is associated with a high risk of developing end-stage renal disease. Biomarkers and analysis of genetic mutations may provide new information for prognosis in SRNS. Frequently relapsing and steroid-dependent NS is associated with long-term complications, including dyslipidemia, cataracts, osteoporosis and fractures, obesity, impaired growth, and infertility...
2016: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/27209298/lessons-from-genetics-is-it-time-to-revise-the-therapeutic-approach-to-children-with-steroid-resistant-nephrotic-syndrome
#18
REVIEW
Francesca Becherucci, Benedetta Mazzinghi, Aldesia Provenzano, Luisa Murer, Sabrina Giglio, Paola Romagnani
Primitive nephrotic syndrome is one of the most common glomerular diseases in childhood and represents the clinical manifestation of various pathologic changes in the kidney. In children, nephrotic syndrome is classified based on the initial response to empiric corticosteroid treatment, which is considered as the best predictor of patients' final outcome. The advent of next-generation sequencing technology showed that genetic alterations in structural genes of the podocyte can be recognized in a significant proportion of not only familial or syndromic patients with steroid-resistant nephrotic syndrome (SRNS), but also of sporadic cases, raising the question of whether it is time to update current protocols of patient care...
August 2016: Journal of Nephrology
https://www.readbyqxmd.com/read/27190346/nup107-mutations-in-children-with-steroid-resistant-nephrotic-syndrome
#19
Eujin Park, Yo Han Ahn, Hee Gyung Kang, Noriko Miyake, Hiroyasu Tsukaguchi, Hae Il Cheong
BACKGROUND: NUP107 is a novel gene associated with autosomal recessive steroid-resistant nephrotic syndrome (SRNS) with focal segmental glomerulosclerosis (FSGS) in children. The frequency of NUP107 mutations in children with SR-FSGS remains unknown. METHODS: Nine families with two siblings affected by childhood-onset SRNS or proteinuria were recruited. FSGS was confirmed by a kidney biopsy in at least one affected sibling in all families. Additionally, 69 sporadic pediatric cases with biopsy-proven SR-FSGS who had not responded to any treatment were included...
May 17, 2016: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/27130200/patterns-of-childhood-steroid-sensitive-and-steroid-resistant-nephrotic-syndrome-in-saudi-children-clinicopathological-study-of-87-cases
#20
Abdulla A Alharthi
Nephrotic syndrome (NS) is one of the most common causes of chronic kidney disease in children. Mostly, NS is controlled by steroids. In spite of this, 10% to 20% of the patients have steroid resistant NS (SRNS), and the rest have steroid sensitive NS (SSNS). Eighty-seven children with NS (66 SSNS; 21SRNS) were retrospectively studied within the past 10 years in Taif region of Saudi Arabia. Regarding outcome, 66 (76%) patient responded to the steroid therapy, while 21 (24%) patients characterize as SRNS. Out of 66 SSNS, 25 children revealed complete remission, 26 were diagnosed as steroid dependent, and 15 were identified as frequent relapses...
April 29, 2016: Clinical Pediatrics
keyword
keyword
109105
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"