scholarly journals Congenital nephrotic syndrome may respond to cyclosporine A: A case report and review of literature

2017 ◽  
Vol 145 (7-8) ◽  
pp. 407-410 ◽  
Author(s):  
Bilsana Mulic ◽  
Gordana Milosevski-Lomic ◽  
Dusan Paripovic ◽  
Divna Kruscic ◽  
Mersudin Mulic ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Cyclosporine A ◽  
Partial Remission ◽  
Enzyme Inhibitor ◽  
Severe Disease ◽  
Congenital Nephrotic Syndrome ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Long Term Effect ◽  
Intravenous Gamma Globulin

Introduction. Congenital nephrotic syndrome (CNF) is manifested at birth or within the first three months of life. The Finnish-type of CNF is caused by the mutation of the NPHS1 gene, which encodes nephrin in the podocyte slit diaphragm. It is a very severe disease, for which immunosuppressive therapy is not advised. Here we describe a patient with CNF who responded to CsA by partial remission. Case outline. A girl aged 2.5 months presented with severe non-syndromic steroid-resistant nephrotic syndrome. She needed aggressive support including daily albumin infusions and diuretics. Substitution of vitamin D, thyroxin, and anticoagulants were regularly administered. She was also treated with angiotensin converting enzyme inhibitor, without clear benefits regarding proteinuria. In addition, she received intravenous gamma-globulin replacement therapy and antibiotics during frequent infections. While waiting for the results of genetic analyses and faced with many problems related to daily albumin infusions, infections, and thromboembolic complications, cyclosporine A (CsA) was introduced as an alternative to early nephrectomy and consequent renal failure. The patient responded by partial remission and CsA treatment continued at home without the albumin infusions. After almost five years since the beginning of the treatment, the patient?s renal function remains unreduced. Conclusion. Our case demonstrates that CsA can induce partial remission in patients with genetic forms of steroid-resistant nephrotic syndrome without influencing the glomerular filtration rate. However, its long-term effect and safety should carefully be monitored.

scholarly journals The effectiveness of cyclosporine A for patients with steroid-resistant nephrotic syndrome

Medicine ◽  
2021 ◽  
Vol 100 (49) ◽  
pp. e28186
Author(s):  
Juan Lv ◽  
Shizhi Luo ◽  
Yunxia Zhang ◽  
Enlai Dai
Keyword(s):  
Nephrotic Syndrome ◽  
Cyclosporine A ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome

Fifteen-year remission of a steroid-resistant nephrotic syndrome sustained by cyclosporine A

2007 ◽  
Vol 22 (4) ◽  
pp. 600-602 ◽  
Author(s):  
Jens Drube ◽  
Christoph Geerlings ◽  
Ruth Taylor ◽  
Michael Mengel ◽  
Jochen H. H. Ehrich
Keyword(s):  
Nephrotic Syndrome ◽  
Cyclosporine A ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome

Effect of Cyclosporine A on Hearing in Children with Steroid Resistant Nephrotic Syndrome

2018 ◽  
Vol 35 (3-4) ◽  
pp. 403-412
Author(s):  
Mohamed S. El-Farsy ◽  
Magid A. A. F. Ibrahim ◽  
Fathy N. Fatouh ◽  
Ahmed T. Abd El-Fattah
Keyword(s):  
Nephrotic Syndrome ◽  
Cyclosporine A ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome

Neurotoxicity of cyclosporine A in children with steroid-resistant nephrotic syndrome: is cytotoxic edema really an unfavorable predictor of permanent neurological damage?

2017 ◽  
Vol 129 (15-16) ◽  
pp. 579-582 ◽  
Author(s):  
Danica Batinić ◽  
Danko Milošević ◽  
Boris Filipović-Grčić ◽  
Marija Topalović-Grković ◽  
Nina Barišić ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Cyclosporine A ◽  
Cytotoxic Edema ◽  
Neurological Damage ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome

scholarly journals Whole Exome Sequencing of Patients with Steroid-Resistant Nephrotic Syndrome

2017 ◽  
Vol 13 (1) ◽  
pp. 53-62 ◽  
Author(s):  
Jillian K. Warejko ◽  
Weizhen Tan ◽  
Ankana Daga ◽  
David Schapiro ◽  
Jennifer A. Lawson ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Exome Sequencing ◽  
Whole Exome Sequencing ◽  
Genetic Diagnosis ◽  
Congenital Nephrotic Syndrome ◽  
Causative Mutation ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Whole Exome ◽  
Panel Sequencing

Background and objectivesSteroid-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.Design, setting, participants, & measurementsThree hundred thirty-five individuals with steroid-resistant nephrotic syndrome from 300 families were recruited from April of 1998 to June of 2016. Age of onset was restricted to <25 years of age. Exome data were evaluated for 33 known monogenic steroid-resistant nephrotic syndrome genes.ResultsIn 74 of 300 families (25%), we identified a causative mutation in one of 20 genes known to cause steroid-resistant nephrotic syndrome. In 11 families (3.7%), we detected a mutation in a gene that causes a phenocopy of steroid-resistant nephrotic syndrome. This is consistent with our previously published identification of mutations using a panel approach. We detected a causative mutation in a known steroid-resistant nephrotic syndrome gene in 38% of consanguineous families and in 13% of nonconsanguineous families, and 48% of children with congenital nephrotic syndrome. A total of 68 different mutations were detected in 20 of 33 steroid-resistant nephrotic syndrome genes. Fifteen of these mutations were novel. NPHS1, PLCE1, NPHS2, and SMARCAL1 were the most common genes in which we detected a mutation. In another 28% of families, we detected mutations in one or more candidate genes for steroid-resistant nephrotic syndrome.ConclusionsWhole exome sequencing is a sensitive approach toward diagnosis of monogenic causes of steroid-resistant nephrotic syndrome. A molecular genetic diagnosis of steroid-resistant nephrotic syndrome may have important consequences for the management of treatment and kidney transplantation in steroid-resistant nephrotic syndrome.

scholarly journals Efficacy and safety of cyclosporine a for patients with steroid-resistant nephrotic syndrome: a meta-analysis

2019 ◽  
Vol 20 (1) ◽  
Author(s):  
Hong-Yan Li ◽  
Xialan Zhang ◽  
Tianbiao Zhou ◽  
Zhiqing Zhong ◽  
Hongzhen Zhong
Keyword(s):  
Nephrotic Syndrome ◽  
Serum Creatinine ◽  
Cyclosporine A ◽  
Plasma Cholesterol ◽  
Meta Analysis ◽  
Cochrane Library ◽  
Efficacy And Safety ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
The Cochrane Library

Abstract Background The purpose of this study was to determine efficacy and safety of cyclosporine A (CsA) for patients with steroid-resistant nephrotic syndrome (SRNS). Methods The Cochrane Library and PubMed were searched to extract the associated studies on Oct 10, 2018, and the meta-analysis method was used to pool and analyze the applicable investigations included in this study. The P(opulation) I(ntervention) C(omparison) O(utcome) of the study were defined as follows: P: Patients with SRNS; I: treated with CsA, cyclophosphamide (CYC), tacrolimus (TAC) or placebo/not treatment (P/NT); C: CsA vs. placebo/nontreatment (P/NT), CsA vs. CYC, CsA vs. TAC; O: complete remission (CR), total remission (TR; complete or partial remission (PR)), urine erythrocyte number, proteinuria levels, albumin, proteinuria, serum creatinine, and plasma cholesterol, etc. Data were extracted and pooled using RevMan 5.3. Results In the therapeutic regimen of CsA vs. placebo/nontreatment (P/NT), the results indicated that the CsA group had high values of CR, TR, and low values of proteinuria, serum creatinine, and plasma cholesterol when compared with those in the placebo group. In comparing CsA vs. cyclophosphamide (CYC), the results indicated that the CsA group had higher TR than the CYC group. In comparing CsA vs. tacrolimus (TAC), the results revealed insignificant differences in CR, and TR between the CsA and TAC groups. The safety of CsA was also assessed. The incidence of gum hyperplasia in CsA group was higher than that in the P/NT group, with no differences in incidence of infections or hypertension between CsA and P/NT groups. There was no difference in the incidence of hypertension between the CsA and TAC groups. Conclusions CsA is an effective and safe agent in the therapy of patients with SRNS.

scholarly journals Treatment of Steroid and Cyclosporine-Resistant Idiopathic Nephrotic Syndrome in Children

2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
A. A. Nikibakhsh ◽  
H. Mahmoodzadeh ◽  
M. Karamyyar ◽  
S. Hejazi ◽  
M. Noroozi ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Complete Remission ◽  
Partial Remission ◽  
Significant Risk ◽  
Second Step ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Risk Of Progression ◽  
End Stage ◽  
Total Remission

Steroid-resistant nephrotic syndrome (SRNS) in children carries a significant risk of progression to end-stage renal failure (ESRF). We report a two-step protocol adapted in children with SRNS. Thirty-seven SRNS were treated with cyclosporine A (CyA) in association with prednisolone (alternate day) for 6 months (first-step treatment). Twelve patients (32.4%) went into complete remission, and 2 (5.4%) got partial remission. The other 23 cases who were steroid and CyA resistant entered a second-step treatment with withdrawing steroids, with CyA (5 mg/kg/day) in association with mycophenolate mofetil (MMF) 30 mg/kg/day for 6 months. Complete remission was observed in 11 cases (47.82%) and partial remission in 2 cases (8.7%). After two steps of treatment, 27/37 children went into total remission. In steroid and CyA-resistant INS, the association of MMF with CyA was able to induce remission in about half cases without relevant side effects.

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