#4879 PREDICTORS OF KIDNEY DISEASE PROGRESSION IN MALES WITH X-LINKED ALPORT SYNDROME

Abstract Background and Aims Alport's syndrome is the second most common monogenic cause of end-stage renal disease (ESRD). Males with X-linked (XLAS) have a high risk early ESRD development. The aim study was to determine predictors unfavorable prognosis in boys XLAS. Method children genetically confirmed XLAS (n = 84, age 8.7±4.3 yrs, eGFR 102±16.2 ml/min/1.73 m2, 59 pts missense COL4A5 mutations) were included observation single center (FU 6.9±2.4 yrs). Seventy three (q 0.87) treated ACEi (age start 7.6±3.3 dose for Ramipril 2.7±0.8 mg/m2/day). Arterial blood pressure (BP), proteinuria (Pr, mg/m2/day), (Schwartz equation, ml/min/1,73m2), gene mutation type ACEi-treatment data stage at therapy start, dosage, dynamics Pr eGFR) obtained updated each patients. BP >90 perc gender, height defined as uncontrolled (uBP); categorised according its level low (100-<250 moderate (≥250-500 (≥500-1000 mg/m2/day) nephrotic (≥1000 Gene mutations divided into severe (nonsense, deletion, splicing) less (missense) mutations. eGFR<60 ml/min/1,73m2 primary outcome. Results Twenty 0.24, 13.5±2.96 yrs) reached end point during period. Non-missense (HR 4.28, 95% CI 1.47-12.4, p 0.007), uBP 20, 4.68-29.6, p<0.001), persistent >250 mg/m2/day 3.36-21, absence treatment 10.7, 2.45-14.7, 0.02) or proteinuric 2.2-13.8, p<0.001) factors progression. Multiple regression analysis adjusted age, initial revealed that late (β 0.23, 0.036), persistence 0.26, 0.008) 0.17, 0.04) had independent significance predict (R 0.76, R2 0.57, p<0.000). Conclusion Persistent pressure, initiation glomerulopathy are male