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Is shrunken pore syndrome also a reality in children?

Mathilde Roussel1, Justine Bacchetta1,2,3, Anne Laure Sellier-Leclerc1

  • 1Service 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.

Journal of Internal Medicine
|December 7, 2023
PubMed

Insights

Shrunken pore syndrome (SPS) prevalence in children varies by formula, but higher CRP and phosphate levels in affected children suggest potential long-term cardiovascular risk. Further research is needed in broader pediatric groups.

Area of Science:

  • Pediatric Nephrology
  • Cardiovascular Risk Assessment
  • Glomerular Filtration Rate Estimation

Background:

  • Shrunken pore syndrome (SPS) is characterized by a low ratio of cystatin C-based to creatinine-based estimated glomerular filtration rate (eGFRcys/eGFRcreat).
  • While SPS has been observed in children, a definitive link to increased morbidity or mortality has not been established.
  • SPS is associated with elevated cardiovascular risk in adult populations.

Purpose of the Study:

  • To determine the prevalence of SPS in a pediatric cohort using various glomerular filtration rate (GFR) estimating equations.
  • To investigate the potential association between SPS and cardiovascular risk factors in children.
  • To compare the characteristics of pediatric patients diagnosed with SPS using different diagnostic criteria.

Main Methods:

  • The study included 307 pediatric patients with renal risk factors.
  • SPS prevalence was assessed using combinations of creatinine-based eGFR equations (CKiDU25, Full-age spectrum (FAS), European Kidney Function Consortium (EKFC)) and cystatin C-based eGFR.
  • Patient characteristics were compared based on SPS definition using FAS and/or EKFC equations.

Main Results:

  • The prevalence of SPS differed significantly based on the chosen GFR estimating formulas and thresholds.
  • Children diagnosed with SPS using FAS and/or EKFC equations exhibited higher C-reactive protein (CRP) and phosphate levels.
  • Smaller body size was also noted in children with SPS, potentially indicating long-term cardiovascular risks.

Conclusions:

  • SPS prevalence in pediatric populations is highly dependent on the diagnostic criteria and formulas employed.
  • Elevated inflammatory markers (CRP) and phosphate levels in pediatric SPS patients suggest a potential link to future cardiovascular complications.
  • Further investigation in larger, general pediatric populations is warranted to confirm these findings and elucidate the long-term implications of SPS.
Abstract

Keywords:
cardiovascular riskchildrencreatininecystatin Cglomerular filtration rateshrunken pore syndrome

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