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Changing the Outcome of a Pediatric Disease: Part I — Clinical Features of ADPKD

  • Pediatric Nephrology (E Nehus and BP Dixon, Section Editors)
  • Published:
Current Treatment Options in Pediatrics Aims and scope Submit manuscript

Abstract

Purpose of Review

The goal of this article is to review genetic and clinical features of autosomal dominant polycystic kidney disease (ADPKD) with specific focus on affected children.

Recent Findings

Children manifest many of the same features of ADPKD that impact affected adults. Our understanding of how defective regulation and function of polycystin-1 and/or -2 induce kidney cystogenesis in ADPKD forms the rationale for therapeutic interventions. Of critical importance in recent decades is the realization that the primary target of intervention should be structural kidney disease (as measured by total kidney volume (TKV)), rather than the late clinical feature of declining kidney function. Other markers of high risk for disease progression in childhood may include genetic mutation type and presence of hypertension, proteinuria, and/or glomerular hyperfiltration.

Summary

ADPKD is a common pediatric disease. Our knowledge of its genetic features and clinical impact in children is growing. We must establish valid and reliable tools to identify children with ADPKD who are at the greatest risk of end-stage kidney disease (ESKD) so that safe and effective treatment options can be timed appropriately to alter the long-term outcome.

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Authors and Affiliations

  1. Rocky Mountain Pediatric Kidney Center, Rocky Mountain Hospital for Children at Presbyterian/St. Luke’s Medical Center, 2055 N High St #270, Denver, CO, 80205, USA

    Melissa A. Cadnapaphornchai MD

  2. Department of Pediatric Nephrology and Organ Transplantation, University Hospitals Leuven, Leuven, Belgium

    Djalila Mekahli MD, PhD

  3. PKD Research Group, Laboratory of Pediatrics, Department of Development and Regeneration, KU Leuven, Leuven, Belgium

    Djalila Mekahli MD, PhD

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  1. Melissa A. Cadnapaphornchai MD
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Correspondence to Melissa A. Cadnapaphornchai MD.

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MAC has participated in an advisory board and consultancy (Otsuka), outside the submitted work. DM, represented by University Hospitals Leuven and KU Leuven University (Belgium), received an educational grant (Otsuka, Galapagos), and participated in an advisory board (Genzyme, Otsuka and Galapagos) and consultancy (Otsuka, Reata), all outside the submitted work.

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Cadnapaphornchai, M.A., Mekahli, D. Changing the Outcome of a Pediatric Disease: Part I — Clinical Features of ADPKD. Curr Treat Options Peds 8, 65–76 (2022). https://doi.org/10.1007/s40746-022-00244-z

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