Blood pressure level and progression of renal disease in children with Х-linked Alport syndrome

AIM: The aim was to determine the significance of blood pressure level on progression of renal failure in children with X-linked Alport syndrome. PATIENTS AND METHODS. eGFR, urine proteine (mg/m²/day), mean day blood pressure – MBP (normalized for sex, age, height) were assessed in retrospective single center study. RESULTS. A 69 children (age 9.49±4.18 years, М/ F=47/22, eGFR=109±17.36 ml/min/1,73m²) were followed for 4[3;5] years. The 27 pts (М/F=13/14) had MBP<50‰, the 33 children (М/F=25/8) – MBP 50-90‰ and pts (М/F=9/0) – MBP≥90‰. Rate of eGFR decline was higher in male (-3[-0.8;-4.7] vs 0.2[-0.4;2]; χ² = 21.15587, p = 0.0067), in pts with MBP ≥90‰ (0.65[-2.65;2] vs -2.3[-6;2.3] vs -3[-7.5;-1.8] in 1st, 2 nd and 3 groups, respectively; р_1,3 =0.0091) and proteinuria (-2.5[-7;0.5] vs 0.2[-4;3]; p=0.0018). Patients with blood hypertension had high risk of renal failure (OR=5.33, 95 % CI 1.75;16.22), especially in cases of proteinuria (OR=7.08, 95 % CI 2.46;20.39). MBP<50‰ associated with low risk of renal disease progression (OR=0.83, 95 % CI 0.31;2.2), especially in boys and proteinuric patients. The male gender and proteinuria are independent risk factors for eGFR decline. CONCLUSIONS. MBP≥90‰ is associated with renal disease progression, especially in case of proteinuria; the MBP<50‰ reduced the risk of eGFR decline in male and proteinuric patients with Alport syndrome; the male gender and proteinuria are independent risk factors for renal disease progression in children with Alport syndrome.

children, Alport syndrome X-linked, blood pressure, blood pressure monitoring ambulatory, blood hypertension, proteinuria, glomerular filtration rate, chronic renal failure

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