title: Long-term treatment by ACE inhibitors and angiotensin receptor blockers in children with Alport syndrome.
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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Pediatrics, Peking University First Hospital, No. 1, Xi An Men Da Jie, Beijing, 100034, People's Republic of China.
authors.forename: Yanqin
authors.initials: Y
authors.lastname: Zhang
authors.affiliation: Department of Pediatrics, Peking University First Hospital, No. 1, Xi An Men Da Jie, Beijing, 100034, People's Republic of China.
authors.forename: Fang
authors.initials: F
authors.lastname: Wang
authors.affiliation: Department of Pediatrics, Peking University First Hospital, No. 1, Xi An Men Da Jie, Beijing, 100034, People's Republic of China. djnc_5855@126.com.
authors.forename: Jie
authors.initials: J
authors.lastname: Ding
authors.affiliation: Department of Pediatrics, Peking University First Hospital, No. 1, Xi An Men Da Jie, Beijing, 100034, People's Republic of China.
authors.forename: Hongwen
authors.initials: H
authors.lastname: Zhang
authors.affiliation: Department of Pediatrics, Peking University First Hospital, No. 1, Xi An Men Da Jie, Beijing, 100034, People's Republic of China.
authors.forename: Xiaoyu
authors.initials: X
authors.lastname: Liu
authors.affiliation: Department of Electron Microscopy, Peking University First Hospital, Beijing, China.
authors.forename: Suxia
authors.initials: S
authors.lastname: Wang
authors.affiliation: Department of Pediatrics, Peking University First Hospital, No. 1, Xi An Men Da Jie, Beijing, 100034, People's Republic of China.
authors.forename: Huijie
authors.initials: H
authors.lastname: Xiao
authors.affiliation: Department of Pediatrics, Peking University First Hospital, No. 1, Xi An Men Da Jie, Beijing, 100034, People's Republic of China.
authors.forename: Yong
authors.initials: Y
authors.lastname: Yao
authors.affiliation: Department of Pediatrics, Peking University First Hospital, No. 1, Xi An Men Da Jie, Beijing, 100034, People's Republic of China.
authors.forename: Jingcheng
authors.initials: J
authors.lastname: Liu
authors.affiliation: Department of Pediatrics, Peking University First Hospital, No. 1, Xi An Men Da Jie, Beijing, 100034, People's Republic of China.
authors.forename: Xuhui
authors.initials: X
authors.lastname: Zhong
authors.affiliation: Department of Pediatrics, Peking University First Hospital, No. 1, Xi An Men Da Jie, Beijing, 100034, People's Republic of China.
authors.forename: Na
authors.initials: N
authors.lastname: Guan
authors.affiliation: Department of Pediatrics, Peking University First Hospital, No. 1, Xi An Men Da Jie, Beijing, 100034, People's Republic of China.
authors.forename: Baige
authors.initials: B
authors.lastname: Su
authors.affiliation: Department of Pediatrics, Peking University First Hospital, No. 1, Xi An Men Da Jie, Beijing, 100034, People's Republic of China.
authors.forename: Guohong
authors.initials: G
authors.lastname: Wu
authors.affiliation: Department of Pediatrics, Peking University First Hospital, No. 1, Xi An Men Da Jie, Beijing, 100034, People's Republic of China.
authors.forename: Lixia
authors.initials: L
authors.lastname: Yu
mesh_terms: D000293:Adolescent; D000367:Age Factors; D047228:Angiotensin II Type 1 Receptor Blockers / Q000009:adverse effects / Q000627:therapeutic use*; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000009:adverse effects / Q000627:therapeutic use*; D015415:Biomarkers / Q000097:blood / Q000652:urine; D002648:Child; D002675:Child, Preschool; D002681:China; D024141:Collagen Type IV / Q000235:genetics; D003404:Creatinine / Q000097:blood / Q000652:urine; D018450:Disease Progression; D004359:Drug Therapy, Combination; D005260:Female; D020022:Genetic Predisposition to Disease; D006579:Heterozygote; D006801:Humans; D007223:Infant; D007668:Kidney / Q000187:drug effects* / Q000503:physiopathology; D007676:Kidney Failure, Chronic / Q000188:drug therapy / Q000235:genetics / Q000503:physiopathology; D008297:Male; D009154:Mutation; D009394:Nephritis, Hereditary / Q000175:diagnosis / Q000188:drug therapy* / Q000235:genetics / Q000503:physiopathology; D010641:Phenotype; D011507:Proteinuria / Q000175:diagnosis / Q000188:drug therapy* / Q000235:genetics / Q000503:physiopathology; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D047228:Angiotensin II Type 1 Receptor Blockers; D000806:Angiotensin-Converting Enzyme Inhibitors; D015415:Biomarkers; D024141:Collagen Type IV; D003404:Creatinine
keywords: ACE inhibitor; Alport syndrome; Angiotensin receptor blocker; Children; Proteinuria