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Cohort studyKidney international · 2026

A multi-national study examined the response sustainability to calcineurin inhibitors and long-term kidney survivals in children with genetic podocytopathies.

Georgia Malakasioti, Daniela Iancu, Chun Ting Au … CNI in Monogenic SRNS Study Investigators

NephrologyCohort study

Non-sustained calcineurin inhibitor response linked to significantly lower kidney failure risk.

A multi-national study examined the response sustainability to calcineurin inhibitors and long-term kidney survivals in children with genetic podocytopathies.

Georgia Malakasioti … CNI in Monogenic SRNS Study Investigators

Kidney international · 2026

Background

While calcineurin inhibitors (calcineurin inhibitors) reduce proteinuria in a subgroup of children with genetic podocytopathies, previous reports suggest that the response is short-lived and of no benefit to long-term outcomes.

Methods

We performed a retrospective cohort study of patients aged 0-18 years with genetic podocytopathies treated with calcineurin inhibitors for at least three months from 34 pediatric nephrology centers worldwide.

n = 136 patients

92%

Results

92%

lower risk of kidney failure

a temporary response meant a much lower risk of kidney failure than no response

n = 136 patients

More results

At least partial response was attained in 28% and 22% of patients at six months post-calcineurin inhibitors initiation and last follow-up, respectively.

Compared to non-response group, patients with at least partial response at six months had significantly lower urine protein-to-creatinine ratios across various timepoints post-calcineurin inhibitors.

More results

71%, 11% and 18% exhibited no response, non-sustained and sustained response, respectively, throughout the observation period.

Both sustained and non-sustained responses were significantly associated with superior kidney survival compared to no response.

“

Conclusion · 1 of 3

A subset of children with genetic podocytopathies experienced a sustained response to calcineurin inhibitors, and none developed kidney failure.

Conclusion · 2 of 3

Children with non-sustained response still demonstrated superior kidney survival compared to non-responders.

Conclusion · 3 of 3

Our findings may support a trial of calcineurin inhibitors in genetic podocytopathies, and continuing therapy among those showing clinical response.

Post

A multi-national study examined the response sustainability to calcineurin inhibitors and long-term kidney survivals in children with genetic podocytopathies.

Non-sustained calcineurin inhibitor response linked to significantly lower kidney failure risk.

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