Re-treatment with high-dose prednisolone after rituximab infusion for childhood-onset steroid-resistant nephrotic syndrome

2014 ◽  
Vol 29 (7) ◽  
pp. 1291-1292 ◽  
Author(s):  
Shuichiro Fujinaga ◽  
Taichi Hara
Keyword(s):  
Nephrotic Syndrome ◽  
High Dose ◽  
Childhood Onset ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome

scholarly journals Rapid Detection of Monogenic Causes of Childhood-Onset Steroid-Resistant Nephrotic Syndrome

2014 ◽  
Vol 9 (6) ◽  
pp. 1109-1116 ◽  
Author(s):  
Svjetlana Lovric ◽  
Humphrey Fang ◽  
Virginia Vega-Warner ◽  
Carolin E. Sadowski ◽  
Heon Yung Gee ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Rapid Detection ◽  
Childhood Onset ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome

scholarly journals Biallelic Mutations in Nuclear Pore Complex Subunit NUP107 Cause Early-Childhood-Onset Steroid-Resistant Nephrotic Syndrome

2015 ◽  
Vol 97 (4) ◽  
pp. 555-566 ◽  
Author(s):  
Noriko Miyake ◽  
Hiroyasu Tsukaguchi ◽  
Eriko Koshimizu ◽  
Akemi Shono ◽  
Satoko Matsunaga ◽  
...  
Keyword(s):  
Early Childhood ◽  
Nephrotic Syndrome ◽  
Nuclear Pore Complex ◽  
Nuclear Pore ◽  
Childhood Onset ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Biallelic Mutations

scholarly journals Nephrin Mutations Can Cause Childhood-Onset Steroid-Resistant Nephrotic Syndrome

2008 ◽  
Vol 19 (10) ◽  
pp. 1871-1878 ◽  
Author(s):  
Aurélie Philippe ◽  
Fabien Nevo ◽  
Ernie L. Esquivel ◽  
Dalia Reklaityte ◽  
Olivier Gribouval ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Childhood Onset ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome

scholarly journals Corticosteroids and obesity in steroid-sensitive and steroid-resistant nephrotic syndrome

2015 ◽  
Vol 55 (4) ◽  
pp. 194 ◽  
Author(s):  
Nina Lestari ◽  
Neti Nurani ◽  
Madarina Julia
Keyword(s):  
Nephrotic Syndrome ◽  
Central Obesity ◽  
Corticosteroid Treatment ◽  
High Dose ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Steroid Sensitive Nephrotic Syndrome ◽  
Prevalence Of Obesity ◽  
Steroid Sensitive ◽  
Obesity In Children

Background Children with nephrotic syndrome need high-dose corticosteroids to achieve remission. Studies have estimated a 35-43% risk of obesity in these patients after corticosteroid treatment.Objective To determine the prevalence of obesity in children who received corticosteroids for nephrotic syndrome, and to compare the risk of obesity in children with steroid-sensitive nephrotic syndrome (SSNS) and steroid-resistant nephrotic syndrome (SRNS).Methods We performed a retrospective cohort study in 50 children with SSNS or SRNS who received corticosteroid treatment. Obesity was defined to be a BMI-for-age Z-score above +2.0 SD, according to the WHO Growth Reference 2007. Central obesity was defined to be a waist-to-height ratio > 0.50.Results The overall prevalence of obesity was 22%, with 29% and 14% in the SSNS and SRNS groups, respectively. The overall prevalence of central obesity was 50%, with 54% and 46% in the SSNS and SRNS groups, respectively. The cumulative steroid doses in this study were not significantly different between the SSNS and SRNS groups. There were also no significant differences between groups for risk of obesity (RR 2.53; 95%CI 0.58 to 10.99) or central obesity (RR 1.39; 95%CI 0.45 to 4.25).Conclusion In children with nephrotic syndrome who received corticosteroids, the prevalence of obesity is 22% and of central obesity is 50%. In a comparison of SSNS and SRNS groups, cumulative steroid dose as well as risks of obesity and central obesity do not significantly differ between groups.

scholarly journals HLA-DQA1 and APOL1 as Risk Loci for Childhood-Onset Steroid-Sensitive and Steroid-Resistant Nephrotic Syndrome

2018 ◽  
Vol 71 (3) ◽  
pp. 399-406 ◽  
Author(s):  
Adebowale Adeyemo ◽  
Christopher Esezobor ◽  
Adaobi Solarin ◽  
Asiri Abeyagunawardena ◽  
Jameela A. Kari ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Childhood Onset ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Steroid Sensitive ◽  
Hla Dqa1

A rare cause of steroid-resistant nephrotic syndrome in a child: Answers

2019 ◽  
Vol 35 (4) ◽  
pp. 621-623
Author(s):  
Lale Guliyeva ◽  
Yılmaz Tabel ◽  
Ali Düzova ◽  
Nusret Akpolat ◽  
Seza Özen ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome

Whole-Exome Sequencing Solved over 2-Decade Kidney Disease Enigma

Nephron ◽  
2021 ◽  
pp. 1-6
Author(s):  
Suramath Isaranuwatchai ◽  
Ankanee Chanakul ◽  
Chupong Ittiwut ◽  
Chalurmpon Srichomthong ◽  
Vorasuk Shotelersuk ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Kidney Disease ◽  
Exome Sequencing ◽  
Whole Exome Sequencing ◽  
Treatment Plan ◽  
Unknown Etiology ◽  
Pediatric Case ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Whole Exome

Chronic kidney disease of unknown etiology (CKDu) has been a problem in renal practice as indefinite diagnosis may lead to inappropriate management. Here, we report a 54-year-old father diagnosed with CKDu at 33 years old and his 8-year-old son with steroid-resistant nephrotic syndrome. Using whole-exome sequencing, both were found to be heterozygous for c.737G>A (p.Arg246Gln) in LMX1B. The diagnosis of LMX1B-associated nephropathy has led to changes in the treatment plan with appropriate genetic counseling. The previously reported cases with this particular mutation were also reviewed. Most children with LMX1B-associated nephropathy had nonnephrotic proteinuria with normal renal function. Interestingly, our pediatric case presented with steroid-resistant nephrotic syndrome at 8 years old and progressed to ESRD requiring peritoneal dialysis at the age of 15 years. Our report emphasized the need of genetic testing in CKDu for definite diagnosis leading to precise management.

scholarly journals MP032NEXT GENERATION GENE PANEL SCREENING IN STEROID-RESISTANT NEPHROTIC SYNDROME

2016 ◽  
Vol 31 (suppl_1) ◽  
pp. i353-i353
Author(s):  
Beata S. Lipska-Ziętkiewicz ◽  
Olivia Boyer ◽  
Olivier Gribouval ◽  
Mansoureh Tabatabaei ◽  
Cecile Fourrage ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Gene Panel ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome
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