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Age-dependent reference values of urinary porphyrins in children

E I Minder1, X Schneider-Yin

  • 1Zentrallabor and Swiss Porphyrin Reference Laboratory SGKC/IFCC, Stadtspital Triemli, Zürich, Switzerland.

European Journal of Clinical Chemistry and Clinical Biochemistry : Journal of the Forum of European Clinical Chemistry Societies
|May 1, 1996
PubMed

Insights

This study establishes age-dependent reference ranges for urinary uroporphyrin, coproporphyrin I, and coproporphyrin III in children. These detailed ranges aid in diagnosing specific disorders, unlike total porphyrin measurements.

Area of Science:

  • Biochemistry
  • Pediatric Medicine
  • Clinical Chemistry

Background:

  • Urinary porphyrins are crucial biomarkers for heme synthesis and detoxification pathways.
  • Establishing age-specific reference ranges is vital for accurate clinical interpretation in pediatric populations.
  • Previous studies lacked comprehensive age-dependent data for individual urinary porphyrins.

Purpose of the Study:

  • To determine age-dependent reference ranges for uroporphyrin, coproporphyrin I, and coproporphyrin III in children.
  • To investigate the unique age-related excretion patterns of these major urinary porphyrins.
  • To highlight the clinical utility of individual porphyrin quantification in pediatric diagnostics.

Main Methods:

  • High-performance liquid chromatography (HPLC) was used to quantify porphyrins.
  • Urine samples were collected from 198 children (0.5–16 years) and 18 newborns.
  • Statistical analysis was performed to establish age-specific reference ranges.

Main Results:

  • All three porphyrins (uroporphyrin, coproporphyrin I, coproporphyrin III) exhibited distinct age-dependencies.
  • Elevated coproporphyrin I in newborns was linked to immature excretion systems.
  • Coproporphyrin III showed the strongest correlation with childhood heme synthesis and peaked between ages 1-2.

Conclusions:

  • Quantifying individual urinary porphyrins provides diagnostic insights beyond total porphyrin levels.
  • The established reference ranges are valuable for diagnosing pediatric disorders, including a case of bronze baby syndrome.
  • Age-specific porphyrin profiles are essential for understanding heme metabolism and related pathologies in children.

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