Clinical course of proteinuria due to cubilin variants: a large multicenter pediatric cohort

In this multicenter, large pediatric cohort, proteinuria associated with CUBN variants generally followed a benign course over short to mid-term follow-up even without sustained ACEi/ARB therapy. Embedding targeted CUBN testing into the evaluation of children with asymptomatic proteinuria and normal kidney function may reduce unnecessary kidney biopsies and prolonged medication, while improving family counseling. We outline a stepwise care pathway → early genetic screening → conservative monitoring with periodic eGFR and proteinuria assessment → consideration of ACEi/ARB discontinuation and recommend prospective validation and cost-effectiveness studies.

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Publication Details

Journal
Pediatric Nephrology
Published
2026-03-04
DOI
https://doi.org/10.1007/s00467-026-07227-4
Primary Topic
Renal Diseases and Glomerulopathies
Type
article
Field-Weighted Citation Impact
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article

Clinical course of proteinuria due to cubilin variants: a large multicenter pediatric cohort

Emre Leventoğlu, Aylin Inal, Mehtap Kaya, NURDAN YILDIZ et al.
Pediatric Nephrology
Renal Diseases and Glomerulopathies
article

Clinical course of proteinuria due to cubilin variants: a large multicenter pediatric cohort

Emre Leventoğlu, Aylin Inal, Mehtap Kaya, NURDAN YILDIZ, Seçil Kezer, İlknur Girişgen, Özde Nisa Türkkan, İbrahim Gökçe, Ceren Alavanda, Mehtap Akbalik Kara, ZEYNEP NAGEHAN YÜRÜK YILDIRIM, Bahriye ATMIŞ, Serçin Güven, Sevcan HATİPOĞLU, Okan Akaci, PINAR ATA, AYŞE AĞBAŞ, Nimet Sasmaz Nurdag, Ismail Dursun, Serim Pul, Ayse Seda Pınarbası, Neslihan Cicek
article en

Abstract

In this multicenter, large pediatric cohort, proteinuria associated with CUBN variants generally followed a benign course over short to mid-term follow-up even without sustained ACEi/ARB therapy. Embedding targeted CUBN testing into the evaluation of children with asymptomatic proteinuria and normal kidney function may reduce unnecessary kidney biopsies and prolonged medication, while improving family counseling. We outline a stepwise care pathway → early genetic screening → conservative monitoring with periodic eGFR and proteinuria assessment → consideration of ACEi/ARB discontinuation and recommend prospective validation and cost-effectiveness studies.

Pediatric Nephrology
Ministry of Health (TR), Dokuz Eylül University (TR), Konya Numune Hastanesi (TR), Dr Lütfi Kırdar Kartal Eğitim ve Araştırma Hastanesi (TR), Marmara Üniversitesi Eğitim ve Araştırma Hastanesi (TR), Diyarbakır Askeri Hastanesi (TR), Ümraniye Eğitim ve Araştırma Hastanesi (TR), Istanbul University-Cerrahpaşa (TR), Konya Eğitim ve Araştırma Hastanesi (TR), Education Training And Research (US), Sağlık Bilimleri Üniversitesi (TR), S.B.Ü. Bursa Yüksek İhtisas Eğitim ve Araştırma Hastanesi (TR), Pamukkale University (TR), Cukurova University (TR), Gaziantep University (TR), Istanbul University (TR), Marmara University (TR), University of Health Sciences Antigua (AG), Erciyes University (TR)
Openalex Percentile: Top 42%
Renal Diseases and Glomerulopathies
DOIOpen Access PDFOpenAlex
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