Steroid resistance in childhood nephrotic syndrome

Childhood nephrotic syndrome is a serious disease with the high risk of mortality. The prevalence worldwide is approximately 2 to 7 cases per 100,000 children. Hitherto, the patients are treatmed with steroid or immunosuppressive therapy and renal transplantation. However, many patients are steroid resistant and have a high risk of recurrence. The mutations in seven genes (NHPS1, NPHS2, CD2AP, PLCE1, ACTN4, TRPC6, INF2) have been implicated in different forms of nephrotic syndrome. Among them, two genes, NPHS1 and NPHS2, encoding for nephrin and podocin protein of the glomerular filtration barrier, have a particularly serious influence in almost of cases and steroid resistance in patients. So that, in the world there were a lot of researches conducted to identify gene mutations that related to disease manifestations and the response to treatment in the patients who have different genetic background. Mutations in NPHS1 and NPHS2 are considered the main cause of 75% cases with nephrotic syndrome. And NPHS2 mutations related to 40% cases with steroid resistance in patients. These results will help the doctors to have an effective treatment for the patients. However, in Vietnam no study has yet been done to detect gene mutations in patients with nephrotic syndrome. In this article, we summarize the research which have been published to give an overview of the genetic basis of causing and treatment for childhood nephrotic syndrome.

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The increasing incidence of initial steroid resistance in childhood nephrotic syndrome

Pediatric Nephrology ◽

10.1007/s00467-011-2083-7 ◽

2012 ◽

Vol 27 (6) ◽

pp. 927-932 ◽

Cited By ~ 25

Author(s):

Beata Banaszak ◽

Paweł Banaszak

Keyword(s):

Nephrotic Syndrome ◽

Steroid Resistance ◽

Childhood Nephrotic Syndrome

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Reciprocal Relationship Between HDAC2 and P-Glycoprotein/MRP-1 and Their Role in Steroid Resistance in Childhood Nephrotic Syndrome

Frontiers in Pharmacology ◽

10.3389/fphar.2019.00558 ◽

2019 ◽

Vol 10 ◽

Cited By ~ 2

Author(s):

Harshit Singh ◽

Vikas Agarwal ◽

Saurabh Chaturvedi ◽

Durga Prasanna Misra ◽

Akhilesh Kumar Jaiswal ◽

...

Keyword(s):

Nephrotic Syndrome ◽

Reciprocal Relationship ◽

Steroid Resistance ◽

Childhood Nephrotic Syndrome ◽

P Glycoprotein

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UrApp for Childhood Nephrotic Syndrome Management (Incident Cohort)

Case Medical Research ◽

10.31525/ct1-nct04075656 ◽

2019 ◽

Author(s):

Keyword(s):

Nephrotic Syndrome ◽

Childhood Nephrotic Syndrome ◽

Incident Cohort

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Steroid treatment for the first episode of childhood nephrotic syndrome: comparison of the 8 and 12 weeks regimen using an individual patient data meta-analysis

European Journal of Pediatrics ◽

10.1007/s00431-021-04035-w ◽

2021 ◽

Author(s):

Anne M. Schijvens ◽

Nynke Teeninga ◽

Eiske M. Dorresteijn ◽

Steven Teerenstra ◽

Nicholas J. Webb ◽

...

Keyword(s):

Nephrotic Syndrome ◽

Individual Patient Data ◽

Meta Analysis ◽

Steroid Treatment ◽

First Episode ◽

Childhood Nephrotic Syndrome ◽

Steroid Sensitive Nephrotic Syndrome ◽

Steroid Sensitive ◽

First Relapse ◽

Steroid Regimen

AbstractSteroids are the cornerstone of the treatment of childhood nephrotic syndrome. The optimal duration for the first episode remains a matter of debate. The aim of this study is to determine whether the 8 weeks International Study of Kidney Disease in Children (ISKDC) regimen is equally effective as the 12 weeks steroid regimen from the German society of pediatric nephrology (Arbeitsgemeinschaft für Pädiatrische Nephrologie [APN]). An individual patient data (IPD) meta-analysis of randomized controlled trials reporting on prednisolone treatment for a first episode of childhood nephrotic syndrome was conducted. European trials aimed at investigating the ISKDC and/or APN steroid regimen were selected. The lead investigators of the selected trials were requested to provide the IPD of the specific treatment groups. Four trials included European cohorts using dosing schedules according to the regimens studied. IPD of two trials were available. A significant difference was found in time to first relapse after cessation of steroid treatment between the 8 and 12 weeks treatment group with a median time to relapse of 29 and 63 days, respectively. Moreover, relapse rate ratios during total follow-up were 51% higher for the 8 weeks regimen. Finally, younger children have a significantly lower time to first relapse and frequently relapsing nephrotic syndrome.Conclusions: The results of this IPD meta-analysis suggest that the 8 weeks steroid regimen for a first episode of steroid-sensitive nephrotic syndrome may not be equally effective as the 12 weeks steroid regimen. Moreover, this study highlights the importance of using uniform definitions to enable accurate comparison and interpretation of trial results.Trial registration: Registration number: CRD42020199244, date of registration 16-08-2020 What is Known:• Steroids are the cornerstone of the treatment of childhood nephrotic syndrome, however the optimal duration for the first episode remains a matter of debate.• Currently, the 8 weeks ISKDC protocol and 12 weeks APN protocol are among the most frequently used protocols in Europe. What is New:• The 8 weeks steroid regimen for a first episode of steroid-sensitive nephrotic syndrome may not be equally effective as the 12 weeks steroid regimen for the treatment of a first episode of nephrotic syndrome.• Younger children have a significantly shorter time to first relapse and time to frequent relapsing nephrotic syndrome.

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