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Abstract
The present study aimed to assess whether urinary profiles of the following lysosomal exoglycosidases: N-acethyl-β-hexosaminidase (HEX), its isoenzymes A (HEX A) and B (HEX B), α – fucosidase (FUC), β -galactosidase (GAL), α – mannosidase (MAN), and β – glucuronidase (GLU) are useful biomarkers of tubular dysfunction in children with solitary functioning kidney (SFK).
Methods We measured HEX, its isoenzymes HEX A, HEX B and FUC, GAL, MAN, GLU urinary activity in 52 patients with SFK. Patients were subdivided in two groups: congenital SFK (cSFK) - unilateral renal agenesis and acquired SFK (aSFK) - unilateral nephrectomy. The reference group (RG) contained 60 healthy children sex and age matched.
Results Urinary activity of all exoglycosidases in SFK was significantly higher than in RG (p < 0.05). There was no difference in exoglycosidases activity between cSFK and aSFK (p > 0.05). HEX, its isoenzymes HEX A, HEX B negatively correlated with eGFR, and all estimated parameters correlated positively with albumin/ creatinine ratio (p < 0.001).
Conclusion Urinary activity of HEX, its isoenzymes HEX A, HEX B, 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 better understand their clinical significance.