Enalapril in paediatric patients with Alport syndrome: 2 years' experience

被引:22
作者
Proesmans, W [1 ]
Knockaert, H [1 ]
Trouet, D [1 ]
机构
[1] Univ Hosp Gasthuisberg, Dept Paediat, B-3000 Louvain, Belgium
关键词
Alport syndrome; angiotensin-converting enzyme inhibitors; proteinuria; children;
D O I
10.1007/s004310051301
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
Enalapril, a long-acting inhibitor of angiotensin-converting enzyme, was given for 2 years to seven children with Alport syndrome. Five patients had a classical X-linked form of the disease; two siblings had the autosomal recessive variant. Their age was between 5.15 and 13.75 years when enalapril was started. All patients had haematuria and proteinuria, creatinine clearance was >80 ml/min per 1.73 m(2) in all. and only one patient was hypertensive. The starting dose of enalapril (0.1 mg/kg body weight per day) was increased progressively according to individual clinical tolerance. Thc median doses were 0.13, 0.12, 0.21 and 0.29 mg/kg at 6, 12, 18 and 24 months, respectively. Median values of mean blood pressure were 95 mmHg at the start and 84 mmHg after 24 months. Median daily proteinuria decreased from 52 mg/kg to 18 mg/kg at 6 months, 21 mg/kg at 12 months, 12 mg/kg at 18 months and 30 mg/kg at 24 months. Serum creatinine increased over time from a median of 0.64 mg/dl at baseline to 0.77 mg/dl at 24 months. Concomitantly, there was a decrease in GFR from 104 to 83 ml/min per 1.73 mt at 18 months and an increase again to 95 ml/min per 1.73 m(2) at 24 months. Analysis of the individual data showed three patterns: no response (n = 2), temporary response (n = 2) and sustained response (n = 3). Conclusion When given enalapril at the dosages mentioned, Alport patients as a group display a marked reduction in urinary protein excretion with a nadir of 23% of the baseline figure at 18 months, a decrease that cannot be accounted for by the slight decrease in glomerular filtration rate. Although these are preliminary data, it is recommended to try an angiotensin-converting enzyme inhibitor in every paediatric Alport patient with proteinuria.
引用
收藏
页码:430 / 433
页数:4
相关论文
共 10 条
  • [1] THERAPEUTIC ADVANTAGE OF CONVERTING ENZYME-INHIBITORS IN ARRESTING PROGRESSIVE RENAL-DISEASE ASSOCIATED WITH SYSTEMIC HYPERTENSION IN THE RAT
    ANDERSON, S
    RENNKE, HG
    BRENNER, BM
    [J]. JOURNAL OF CLINICAL INVESTIGATION, 1986, 77 (06) : 1993 - 2000
  • [2] GENETIC-HETEROGENEITY OF ALPORT SYNDROME
    FEINGOLD, J
    BOIS, E
    CHOMPRET, A
    BROYER, M
    GUBLER, MC
    GRUNFELD, JP
    [J]. KIDNEY INTERNATIONAL, 1985, 27 (04) : 672 - 677
  • [3] Treatment of X-linked hereditary nephritis in Samoyed dogs with angiotensin converting enzyme (ACE) inhibitor
    Grodecki, KM
    Gains, MJ
    Baumal, R
    Osmond, DH
    Cotter, B
    Valli, VEO
    Jacobs, RM
    [J]. JOURNAL OF COMPARATIVE PATHOLOGY, 1997, 117 (03) : 209 - 225
  • [4] MASHIO G, 1996, NEW ENGL J MED, V334, P939
  • [5] IDENTIFICATION OF MUTATIONS IN THE ALPHA-3(IV) AND ALPHA-4(IV) COLLAGEN GENES IN AUTOSOMAL RECESSIVE ALPORT SYNDROME
    MOCHIZUKI, T
    LEMMINK, HH
    MARIYAMA, M
    ANTIGNAC, C
    GUBLER, MC
    PIRSON, Y
    VERELLENDUMOULIN, C
    CHAN, B
    SCHRODER, CH
    SMEETS, HJ
    REEDERS, ST
    [J]. NATURE GENETICS, 1994, 8 (01) : 77 - 82
  • [6] PROTECTION OF KIDNEY-FUNCTION AND DECREASE IN ALBUMINURIA BY CAPTOPRIL IN INSULIN DEPENDENT DIABETICS WITH NEPHROPATHY
    PARVING, HH
    HOMMEL, E
    SMIDT, UM
    [J]. BRITISH MEDICAL JOURNAL, 1988, 297 (6656) : 1086 - 1091
  • [7] Long-term therapy with enalapril in patients with nephrotic-range proteinuria
    Proesmans, W
    VanWambeke, I
    VanDyck, M
    [J]. PEDIATRIC NEPHROLOGY, 1996, 10 (05) : 587 - 589
  • [8] LONG-TERM STABILIZING EFFECT OF ANGIOTENSIN-CONVERTING ENZYME-INHIBITION ON PLASMA CREATININE AND ON PROTEINURIA IN NORMOTENSIVE TYPE-II DIABETIC-PATIENTS
    RAVID, M
    SAVIN, H
    JUTRIN, I
    BENTAL, T
    KATZ, B
    LISHNER, M
    [J]. ANNALS OF INTERNAL MEDICINE, 1993, 118 (08) : 577 - 581
  • [9] EFFECT OF ANGIOTENSIN-CONVERTING ENZYME-INHIBITOR THERAPY ON PROTEINURIA IN CHILDREN WITH RENAL-DISEASE
    TRACHTMAN, H
    GAUTHIER, B
    [J]. JOURNAL OF PEDIATRICS, 1988, 112 (02) : 295 - 298
  • [10] MOLECULAR-GENETICS OF ALPORT SYNDROME
    TRYGGVASON, K
    JING, Z
    HOSTIKKA, SL
    SHOWS, TB
    [J]. KIDNEY INTERNATIONAL, 1993, 43 (01) : 38 - 44