New tubular injury markers in children with a solitary functioning kidney.

BACKGROUND: The present study aimed to assess whether the urinary profiles of the lysosomal exoglycosidases N­acetyl­ß­hexosaminidase (HEX) and its isoenzymes A (HEX A) and B (HEX B), α-fucosidase (FUC), ß-galactosidase (GAL), α-mannosidase (MAN), and ß- glucuronidase (GLU) are useful biomarkers of tubular dysfunction in children with a solitary functioning kidney (SFK). METHODS: We measured the urinary activity of HEX, its isoenzymes HEX A, HEX B, and FUC, GAL, MAN, and GLU in 52 patients with SFK. Patients were subdivided into two groups: congenital SFK (cSFK)-unilateral renal agenesis and acquired SFK (aSFK)-unilateral nephrectomy. The reference group (RG) contained 60 healthy sex- and age-matched children. RESULTS: Urinary activity of all exoglycosidases in SFK was significantly higher than in RG (p < 0.05). There were no differences in exoglycosidase activity between cSFK and aSFK (p > 0.05). HEX and its isoenzymes HEX A and HEX B correlated negatively with estimated glomerular filtration rate (eGFR), and all estimated parameters correlated positively with albumin/creatinine ratio (p < 0.001). CONCLUSION: Urinary activity of HEX, its isoenzymes HEX A and HEX B, and FUC, GAL, MAN, and GLU is elevated in children with SFK. Long-term follow-up studies in larger groups of children with SFK may help us to better understand their clinical significance.