Mitochondrial Complex I Deficiency among Egyptian Pediatric Patients with Steroid-Resistant Nephrotic Syndrome.

RESULTS: Positive consanguinity was a remarkable finding in 44 patients among the SRNS group (73%), compared with 33 patients among the SSNS group (55%). Complex I activity was significantly lower in the SRNS group (0.2657 ± 0.1831 nmol/ml/min), than in the SSNS group (0.4773 ± 0.1290 nmol/ml/min) (p < 0.001). There was a significant positive correlation between complex I activity and the heaviness of proteinuria among the SRNS group (r 0.344, p < 0.001). There were statistically significant differences in serum C3 and C4 levels between both groups (p < 0.001, 0.053, respectively).

CONCLUSION: Mitochondrial complex I deficiency in patients who have a nephrotic syndrome complaint may play a role in their responsiveness to steroid therapy and the development of SRNS and even the prognosis of their illness.