Five-year outcome of children with idiopathic nephrotic syndrome: the NEPHROVIR population-based cohort study

IntroductionIdiopathic nephrotic syndrome (INS) is the most common glomerulopathy that results in childhood chronic kidney disease in China, but the relationships between different clinical phenotypes and immunological genetic variants observed in patients with INS are ambiguous and have not been well studied. A cohort study combined with whole exome sequencing might further identify the effects of immunological genetic variants on clinical phenotypes and treatment outcomes.Methods and analysisWe describe a 3 year prospective observational single-centre cohort study to be conducted in the Children’s Hospital of Chongqing Medical University in China. This study will recruit and investigate 336 patients with childhood-onset INS presenting with different clinical phenotypes. Whole exome sequencing will be conducted when patients progress to a confirmed clinical phenotype during follow-up. Relevant clinical and epidemiological data, as well as conventional specimens, will be collected at study entry and 1 month, 3 months, 6 months, 1 year, 2 years and 3 years after disease onset. After this cohort is generated, the immunological genetic variants of steroid-sensitive nephrotic syndrome without frequent relapse, steroid-resistant nephrotic syndrome and steroid-dependent/frequent relapse nephrotic syndrome will be evaluated.Ethics and disseminationThe study protocol is approved by Ethics Committee of Children’s Hospital of Chongqing Medical University (reference number 2018–140). The results will be disseminated through peer-reviewed journals and conference presentations.Trial registration numberChiCTR1800019795

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Clinical Features of Idiopathic Nephrotic Syndrome and Factors Associated with its Relapse in Children: A Population-Based Study

Journal of Comprehensive Pediatrics ◽

10.5812/compreped.108024 ◽

2020 ◽

Vol 11 (4) ◽

Author(s):

Abolhassan Seyezadeh ◽

Mohamad Reza Tohidi ◽

Somaye Sheikhi ◽

Mohammad Saleh Seyedzadeh ◽

Sara Hookari

Keyword(s):

Nephrotic Syndrome ◽

Recurrence Rate ◽

Clinical Features ◽

Idiopathic Nephrotic Syndrome ◽

Population Based ◽

Steroid Resistance ◽

Filtration Barrier ◽

The Mean ◽

First Recurrence

Background: Idiopathic Nephrotic Syndrome (INS), which is caused by a defect in the glomerular filtration barrier, is the most common chronic glomerular disease in children. Objectives: The present study aimed to assess the clinical features of INS and some recurrence-related factors in children. Methods: This population-based, cross-sectional study was conducted on 302 children with INS referring to the Pediatric Nephrology Clinic of Imam Reza Hospital of Kermanshah city, Iran, during 1998-2018. Results: The mean age (SD) at the time of diagnosis and the follow-up duration were 4.87 (2.89) years and 49.83 (37.52) months, respectively. The numbers of boys and girls were 185 (61.9%) and 114 (38.1%), respectively. The mean number (SD) of recurrences, annual recurrence rate during the follow-up, and the time to the first recurrence after responding to treatment were 1.71 (1.91), 0.48 (0.77), and 10.15 (10.63) months, respectively. The most common type of INS was steroid-dependent/frequent relapse, with a frequency of 151 (50.5%). Furthermore, 33 (11.0%) and 266 (89.0%) patients were resistant and respondent to treatment, respectively. There was a statistically significant relationship between age at the time of diagnosis and the type of INS (P = 0.007). Conclusions: This study revealed a statistically significant association between higher age and steroid resistance. However, in steroid responders, there was no relationship between gender, age at the time of diagnosis, and the time to the first recurrence, and the recurrence rate.

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Premature birth carries a higher risk of nephrotic syndrome: a cohort study

Scientific Reports ◽

10.1038/s41598-021-00164-2 ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

Chih-Chia Chen ◽

Tsung Yu ◽

Hsin-Hsu Chou ◽

Yuan-Yow Chiou ◽

Pao-Lin Kuo

Keyword(s):

Nephrotic Syndrome ◽

Cohort Study ◽

Gestational Age ◽

Preterm Births ◽

Subsequent Development ◽

Maternal Diabetes ◽

Population Based ◽

Term Infants ◽

Serious Infections ◽

Nephrotic Range Proteinuria

AbstractThe pathogenesis of nephrotic syndrome is unclear. We conducted a nationwide population-based cohort study to examine the associations between preterm births and subsequent development of NS. NS was defined as ≥ 3 records with ICD-9-CM codes for NS in hospital admission or outpatient clinic visits. To avoid secondary nephrotic syndrome or nephritis with nephrotic range proteinuria, especially IgA nephropathy, we excluded patients with associated codes. A total of 78,651 preterm infants (gestational age < 37 weeks) and 786,510 matched term infants born between 2004 and 2009 were enrolled and followed until 2016. In the unadjusted models, preterm births, maternal diabetes, and pregnancy induced hypertension were associated with subsequent NS. After adjustment, preterm births remained significantly associated with NS (p = 0.001). The risk of NS increased as the gestational age decreased (p for trend < 0.001). Among the NS population, preterm births were not associated with more complications (Hypertension: p = 0.19; Serious infections: p = 0.63, ESRD: p = 0.75) or a requirement for secondary immunosuppressants (p = 0.61). In conclusion, preterm births were associated with subsequent NS, where the risk increased as the gestational age decreased. Our study provides valuable information for future pathogenesis studies.

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