Loading ...

TOPLINE:

Genetic steroid-resistant nephrotic syndrome (SRNS) and high proteinuria (protein-creatinine ratio > 8000 mg/g) at disease onset, along with a low estimated glomerular filtration rate (eGFR) and persistent hypoalbuminaemia at early follow-up, emerged as key predictors of unfavourable outcomes in SRNS in children.

METHODOLOGY:

• Researchers conducted a retrospective study to identify predictive factors for remission states and kidney survival in patients with SRNS.
• They included data of 65 patients with SRNS (mean age at disease onset, 4 years; 57% boys) treated at a paediatric outpatient clinic in Berlin between 2000 and 2023.
• Clinical, biochemical, and treatment data were analysed. Additionally, SRNS gene panel results were examined and pathogenic variants were found in 29% of patients (genetic group), whereas 51% of patients had negative screening results (non-genetic group).
• The primary outcomes included remission status (complete, partial, or no remission), kidney survival (chronic kidney disease [CKD] stage I-IV), and kidney failure (CKD stage V or death).
• The median follow-up duration was 5 years and 10 months, with assessments at initial presentation, 3 months, 12 months, 2 years, 3 years, 5 years, and 10 years.

TAKEAWAY:

• At the last follow-up, 40% of patients achieved complete remission, 18% achieved partial remission, and 42% had no remission. In the genetic SRNS group, 79% of patients did not achieve remission.
• The kidney survival rates at 5 and 10 years were 71% and 56%, respectively. At the last follow-up, 74% of patients in the genetic group experienced progression to kidney failure within a median of 7.4 months, whereas 27% in the non-genetic group experienced progression within a median of 27.9 months (P < .001).
• Proteinuria with a protein-creatinine ratio > 8000 mg/g (adjusted odds ratio [aOR], 0.04; P = .035), a genetic SRNS diagnosis (aOR, 0.09; P = .030), hypoalbuminaemia, and a reduced eGFR at 3-month and 1-year follow-ups (all P < .05) were associated with remission status.
• These factors were also strongly associated with kidney failure (all P < .05), with hypoalbuminaemia at the 1-year follow-up linked to a nearly 50-fold increased risk for kidney failure (P = .010).

IN PRACTICE:

"The course of SRNS is highly variable and remains challenging to predict due to significant heterogeneity in disease pathophysiology," the authors wrote. "Genetic SRNS and high nephrotic-range proteinuria (> 8000 mg/g) at disease onset, as well as low eGFR and persistent hypoalbuminaemia at early follow-up, were the most important independent predictors for unfavourable outcomes, characterised by NR [no remission] and low kidney survival rates," they added.

SOURCE:

This study was led by Emil Zerkowitz, Charité Universitätsmedizin, Berlin, Germany. It was published online on March 1, 2025, in Pediatric Nephrology.

LIMITATIONS:

The retrospective design and potential for selection bias were the main limitations of this study. Furthermore, quantitative proteinuria measurements were unavailable for some patients at initial presentation, and the study relied on semiquantitative dipstick testing at some follow-up points. The sample size was relatively small, especially in the genetic cohort, possibly limiting the applicability of the findings.

DISCLOSURES:

Open access funding was provided by Projekt DEAL. One author received support from the Else Kröner-Fresenius Foundation and is part of the Clinician Scientist Program at the Berlin Institute of Health at Charité.

This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication.