Long-term Treatment by ACE Inhibitors and Angiotensin Receptor Blockers in Children with Alport Syndrome

Overview

Journal Pediatr Nephrol

Specialties Nephrology
Pediatrics

Date 2015 Aug 8

PMID 26248473

Citations 11

Authors

Yanqin Zhang

Fang Wang

Jie Ding

Hongwen Zhang

Xiaoyu Liu

SuXia Wang

Huijie Xiao

Yong Yao

Jingcheng Liu

Xuhui Zhong

Na Guan

Baige Su

Guohong Wu

Lixia Yu

Affiliations

Soon will be listed here.

Abstract

Background: The aim of this study was to analyze the long-term efficacy and safety of angiotensin-converting enzyme inhibitor (ACEi) and ACEi + angiotensin receptor blocker (ARB) treatments in a cohort of children with Alport syndrome (AS).

Methods: This was a respective review of 79 Chinese children with AS who received ACEi alone or combined ACEi + ARB therapy.

Results: The mean age of the pediatric patients with AS at onset of treatment was 8.6 ± 4.1 (range 1.5-16.3) years. The mean duration of follow-up was 2.5 ± 1.8 (range 0.5-7.8) years. For analysis, we separated the children into three groups according to proteinuria level before treatment, namely, <25, 25-50, and ≥50 mg/kg/day, respectively; after 1 year of treatment the proteinuria had decreased from 11.0 to 9.7 mg/kg/day, from 34.6 to 15.2 mg/kg/day, and from 73.0 to 50.0 mg/kg/day in each group, respectively. Proteinuria decreased significantly during the first 2 years of treatment and was stable from the third to fifth years of treatment. There was no statistically significant difference in the antiproteinuric effect of the ACEi and ACEi + ARB treatments in patients with severe or less severe mutations after 1 year of therapy. Five children stopped the ACEi + ARB treatment due to a decline in creatinine clearance.

Conclusion: Our findings demonstrate that early and long-term ACEi and ARB treatments in children with AS is efficient and well tolerated. The antiproteinuric effect of ACEi and ARB is of equal value in children with severe and less severe mutations in the COL4An gene.

Citing Articles

Case report: A novel compound heterozygous variant in the gene was identified in a patient with autosomal recessive Alport syndrome.

Chen S, Zhang Y, He J, Yang D Front Genet. 2024; 15:1426806.

PMID: 39071776 PMC: 11272558. DOI: 10.3389/fgene.2024.1426806.

Single, Double and Triple Blockade of RAAS in Alport Syndrome: Different Tools to Freeze the Evolution of the Disease.

Mastrangelo A, Brambilla M, Romano G, Serafinelli J, Puccio G, Giani M J Clin Med. 2021; 10(21).

PMID: 34768466 PMC: 8584724. DOI: 10.3390/jcm10214946.

Organoprotective Effects of Spironolactone on Top of Ramipril Therapy in a Mouse Model for Alport Syndrome.

Rubel D, Zhang Y, Sowa N, Girgert R, Gross O J Clin Med. 2021; 10(13).

PMID: 34209341 PMC: 8268845. DOI: 10.3390/jcm10132958.

Genotype-phenotype correlations and nephroprotective effects of RAAS inhibition in patients with autosomal recessive Alport syndrome.

Zhang Y, Bockhaus J, Wang F, Wang S, Rubel D, Gross O Pediatr Nephrol. 2021; 36(9):2719-2730.

PMID: 33772369 PMC: 8370956. DOI: 10.1007/s00467-021-05040-9.

An overview of the multi-pronged approach in the diagnosis of Alport syndrome for 22 children in Northeast China.

Zhang L, Sun B, Zhao B, Ma Q BMC Nephrol. 2020; 21(1):294.

PMID: 32703181 PMC: 7379802. DOI: 10.1186/s12882-020-01962-y.

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