Pediatric reference data on activity of urinary N-acetyl-β-D-hexosaminidase and its isoenzymes

Adv Med Sci. 2018 Mar;63(1):94-99. doi: 10.1016/j.advms.2017.06.007. Epub 2017 Aug 29.


Purpose: The objective of the study was to establish age - dependent values of the urinary lysosomal exoglycosidases activities: N-acetyl-β-D-hexosaminidase (HEX) and its isoenzyme A (HEX A) as well as isoenzyme B (HEX B) in healthy children and adolescents.

Material and methods: The study was performed using a random sample of 203 healthy children and adolescents (girls=99, boys=104), aged six months to 17.9 years. The activities of HEX, HEX A and HEX B were determined by a colorimetric method. The activities of the urinary HEX and its isoenzymes were expressed in pKat/μg of creatinine (pKat/μg Cr).

Results: Median concentrations of urinary HEX, and its HEX A, HEX B isoenzymes in particular age groups were analyzed using ANOVA. Urinary HEX, HEX A and HEX B activities (pKat/μg Cr) were the highest in children below 3 years, in comparison to remaining age groups. There were statistically significant negative correlations between urinary HEX, HEX A as well as HEX B and age (r=-0.24, p<0.001 (HEX); r=-0.20, p<0.01 (HEX A); r=-0.26, p<0.001 (HEX B), respectively. We constructed the reference values for urinary activity of HEX, HEX A and HEX B (pKat/μg Cr) in centiles according to age, in three-year intervals.

Conclusions: Reported data present, for the first time, reference values for urinary activities of HEX and its isoenzymes HEX A and HEX B in children and adolescent.

Keywords: Age; Lysosomal exoglycosidases; N-acetyl-β-D-hexosaminidase (HEX); Normative ranges; Urine; isoenzyme A of N-acetyl-β-D-hexosaminidase (HEX A); isoenzyme B of N-acetyl-β-D-hexosaminidase (HEX B).

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Creatinine / urine
  • Female
  • Humans
  • Infant
  • Isoenzymes / urine*
  • Linear Models
  • Male
  • Reference Values
  • beta-N-Acetylhexosaminidases / urine*


  • Isoenzymes
  • Creatinine
  • beta-N-Acetylhexosaminidases