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Adult survivors of idiopathic childhood onset nephrotic syndrome

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Abstract

Like many pediatric chronic health conditions, idiopathic childhood onset nephrotic syndrome (iCONS) and late effects of iCONS medical management may continue to impact the affected population in adulthood. Approximately 15% of adult survivors of steroid-sensitive iCONS continue to relapse. Long-term kidney health is associated with steroid response patterns as well as pathology findings of FSGS, tubulointerstitial fibrosis, tubular atrophy, and global glomerulosclerosis. Long-term cardiovascular disease burden is largely unknown in adult survivors, but risk factors starting in childhood, including hypertension, dyslipidemia, and obesity, are common in iCONS. Reproductive health concerns, including azo-/oligospermia and successful pregnancies, are largely related to prior exposure to cytotoxic therapies. Additional investigations are needed to complete the assessment and initiate the mitigation of the late effects of treatment-sensitive and treatment-resistant iCONS.

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Authors and Affiliations

  1. Division of Nephrology, Department of Pediatrics, University of Michigan, Ann Arbor, MI, USA

    Dorota M. Marchel & Debbie S. Gipson

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  1. Dorota M. Marchel
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  2. Debbie S. Gipson
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The concept of the article was developed by Dr. Debbie Gipson. Dr. Dorota Marchel performed literature search and drafter the article, which was crtically reviewed by Dr. Debbie Gipson.

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Correspondence to Dorota M. Marchel.

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1. C; 2. B; 3. D; 4. C; 5. C

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Marchel, D.M., Gipson, D.S. Adult survivors of idiopathic childhood onset nephrotic syndrome. Pediatr Nephrol 36, 1731–1737 (2021). https://doi.org/10.1007/s00467-020-04773-3

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