• J. Intern. Med. · Feb 2024

    Is shrunken pore syndrome also a reality in children?

    • Mathilde Roussel, Justine Bacchetta, Anne Laure Sellier-Leclerc, Sandrine Lemoine, Aurélie De Mul, and Laurence Derain Dubourg.
    • Service de Néphrologie Rhumatologie et Dermatologie Pédiatrique, Centre de Référence des Maladies Rénales Rares, filière maladies rares ORKID and ERKNet, Hospices Civils de Lyon, Bron, France.
    • J. Intern. Med. 2024 Feb 1; 295 (2): 253258253-258.

    BackgroundShrunken pore syndrome (SPS) is defined as cystatin C-based-eGFR (eGFRcys)/creatinine-based-eGFR (eGFRcreat) <0.6 or 0.7 and is associated with an increased cardiovascular risk. SPS has been described in children, but no link to increased morbi-mortality was demonstrated.ObjectivesStudy the prevalence of SPS in a pediatric population using several glomerular filtration rate (GFR) estimating formulas and measured GFR and evaluate the potential link with cardiovascular risk.MethodsIn 307 renal risk pediatric patients, we studied prevalence of SPS either with CKiDU25creat and cyst or with FAScreat and cyst and EKFCcreat. The characteristics of patients with SPS (defined with Full-age spectrum equation (FAS) and/or European Kidney Function Consortium equation (EKFC)) were compared.Results And ConclusionThe prevalence of SPS varies widely depending on the threshold and the formulas used. Higher C-reactive protein (CRP) and phosphate levels and smaller size are observed in children with SPS defined with FAS and/or EKFC and might be associated with long-term increased cardiovascular risk. Further studies in wider general pediatric populations are warranted.© 2023 The Association for the Publication of the Journal of Internal Medicine.

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