# Langdurig beloop jeugdige nefrotisch syndroom: recidieven en cardiovasculair risico in volwassenheid

*geplaatst 2026-10-04 · Nierziekte · Kidney international · doi 10.1016/j.kint.2026.06.054 · https://hartvaat.nl/2026/09/26/langdurig-beloop-jeugdige-nefrotisch-syndroom-recidieven-en-cardiovasculair-risi/*

Deze overzichtsstudie beschrijft het langetermijnbeloop van idiopathisch nefrotisch syndroom bij kinderen en de uitdagingen bij de overgang naar de volwassenzorg. Hoewel de meeste patiënten met een steroïd-gevoelige vorm (SSNS) in remissie gaan, kampt ongeveer een kwart met recidieven in het volwassen leven, terwijl steroïd-resistente vormen (SRNS) een drie- tot viervoudig hoger risico lopen op nierfalen en ernstige complicaties zoals hypertensie, obesitas en verhoogd cardiovasculair risico. Het onderzoek benadrukt het belang van vroege risicostratificatie, genetische diagnostiek en gestructureerde zorgtransitie om nier- en hart-vaatcomplicaties op lange termijn te voorkomen.

## English: Long-term outcomes of childhood nephrotic syndrome.

This narrative review synthesizes long-term outcomes of childhood-onset idiopathic nephrotic syndrome and highlights key challenges in transitioning to adult care. While most patients with steroid-sensitive disease achieve remission, approximately 25% experience relapses in adulthood, whereas steroid-resistant forms carry a three- to fourfold higher risk of progressive chronic kidney disease, hypertension, and elevated cardiovascular risk. The findings underscore the need for early risk stratification, targeted genetic testing, and structured care pathways to mitigate long-term renal and cardiometabolic complications.

## Abstract (original, from the publication)

Idiopathic nephrotic syndrome (INS) is the most common chronic glomerular disease in children. Corticosteroids remain first-line therapy, and the initial response, differentiating steroid-sensitive (SSNS) from steroid-resistant (SRNS) forms, is the strongest prognostic factor. While most childhood-onset INS results from an incompletely defined dysregulation of the immune system, up to one-third of children with initial SRNS have a monogenic etiology. SSNS typically follows a relapsing-remitting course, with one-half of the patients having frequent relapses or steroid-dependence (FRNS/SDNS). Although earlier studies suggested that most SSNS patients achieve sustained remission by puberty, approximately one-fourth experience at least one relapse in adulthood. Key predictors of persistent relapsing disease include FRNS/SDNS in childhood and the need for second-line immunosuppression. The disease may evolve into secondary steroid-resistance in <10% patients with SSNS. Kidney failure and death remain uncommon. In contrast, SRNS is associated with higher risks of therapy-and disease-related complications, including growth impairment, obesity, hypertension and cardiovascular risk, osteoporosis, cataracts, calcineurin inhibitor-induced nephrotoxicity, acute kidney injury and progressive chronic kidney disease. The risk of kidney failure is three- to fourfold higher in patients with monogenic and/or multidrug-resistant SRNS. This review synthesizes current evidence on the long-term outcomes of childhood-onset INS, highlights risk stratification across clinical phenotypes, genetic diagnosis and treatment response, and challenges during transition to adult care.

Auteurs: Olivia Boyer, Aditi Sinha, Nestor Oliva-Dámaso, Andrew S Bomback, Evgenia Preka, Arvind Bagga

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Bron: Kidney international, https://doi.org/10.1016/j.kint.2026.06.054. Bijgewerkt 2026-09-28T01:03:55Z. Citeer vrij, met bronvermelding en een link naar hartvaat.nl (de url van het record). Samenvattingen zijn redactioneel werk van HartVaat; de oorspronkelijke publicaties blijven van hun uitgevers (doi). Geen medisch advies.