ESPN 54th Annual Meeting

ESPN 2022


 
EFFICACY OF ANGIOTENSIN-CONVERTING ENZYME INHIBITORS IN CHILDREN WITH DENT DISEASE
LARISA PRIKHODINA 1 SVETLANA PAPIZH 1 ZILYA BASHIROVA 1 VARVARA OBUKHOVA 1 TATYANA LEPAEVA 1 TATYANA NIKISHINA 1

1- RESEARCH & CLINICAL INSTITUTE FOR PEDIATRICS PIROGOV RUSSIAN NATIONAL RESEARCH MEDICAL UNIVERSITY
 
Introduction:

Dent disease (DD) is an X-linked proximal tubulopathy characterized by low molecular weight proteinuria, hypercalciuria and nephrocalcinosis with progression to end-stage kidney disease in males. Data on efficacy of angiotensin-converting enzyme (ACE) inhibitors in children with DD are sparse. The aim of the study was to investigate the efficacy of ACE inhibitors in boys with DD.

Material and methods:

We conducted retrospective longitudinal study of 15 boys aged 12.0 (IQR: 9.0; 15.5) years at the last follow up including 12 patients with DD1 and 3 with DD2 types confirmed by direct Sanger sequencing. The median proteinuria before treatment with ACE inhibitors was 0.92 (0.67; 1.42) g/m2/day. The median eGFR before ACE inhibitors was 82.1 (70.2; 89.5) ml/min/1.73m2. The median age of starting ACE inhibitors was 7.5 (6.0; 9.5) years. The initial ACE inhibitors dosage was 0.13 (0.11; 0.2) mg/kg/day. The median time of treatment with ACE inhibitors was 35.0 (23.0; 85.0) months.

Results:

Treatment with ACE inhibitors led to reduction (>50%) in proteinuria in 9/15 (60%) children with DD including all 3 boys with DD2 type. 2/15 (13.3%) and 4/15 (26.7%) boys with DD treated with ACE inhibitors had the same or increased proteinuria, respectively. Increased eGFR at the last follow-up was found in 8/15 (53.3%) children with DD including all 3 boys with DD2 type. The median rate of increasing eGFR from baseline level on the treatment with ACE inhibitors was 11% (3.4%; 26.4%). 7/15 (46.7%) boys with DD had declined eGFR at the last follow up by 10.6% (-30.1%; -6.2%). All of them had DD 1 type.

Conclusions:

We conclude that treatment with ACE inhibitors lead to decreasing of proteinuria in 60% of patients and increasing of eGFR in 53.3% of boys with DD 1 and 2 types.