caa a22 4500 475784227 CHVBK 20180406123653.0 cr unu---uuuuu 170329e20000501xx s 000 0 eng 10.1007/s004310051301 doi (NATIONALLICENCE)springer-10.1007/s004310051301 Enalapril in paediatric patients with Alport syndrome: 2 years' experience [Elektronische Daten] [Willem Proesmans, Hilde Knockaert, Dominique Trouet] 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 m2 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. The 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 m2 at 18 months and an increase again to 95 ml/min per 1.73 m2 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. Springer-Verlag Berlin Heidelberg, 2000 Key words Alport syndrome nationallicence Angiotensin-converting enzyme inhibitors nationallicence Proteinuria nationallicence Children nationallicence AbbreviationACEi angiotensin-converting enzyme inhibitor nationallicence Proesmans Willem Department of Paediatrics, University Hospital Gasthuisberg, Herestraat 49, 3000 Leuven, Belgium Tel.: +32-16-343841; Fax: +32-16-343842, BE aut Knockaert Hilde Department of Paediatrics, University Hospital Gasthuisberg, Herestraat 49, 3000 Leuven, Belgium Tel.: +32-16-343841; Fax: +32-16-343842, BE aut Trouet Dominique Department of Paediatrics, University Hospital Gasthuisberg, Herestraat 49, 3000 Leuven, Belgium Tel.: +32-16-343841; Fax: +32-16-343842, BE aut https://doi.org/10.1007/s004310051301 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s004310051301 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Proesmans Willem Department of Paediatrics, University Hospital Gasthuisberg, Herestraat 49, 3000 Leuven, Belgium Tel.: +32-16-343841; Fax: +32-16-343842, BE aut NATIONALLICENCE 700 1- Knockaert Hilde Department of Paediatrics, University Hospital Gasthuisberg, Herestraat 49, 3000 Leuven, Belgium Tel.: +32-16-343841; Fax: +32-16-343842, BE aut NATIONALLICENCE 700 1- Trouet Dominique Department of Paediatrics, University Hospital Gasthuisberg, Herestraat 49, 3000 Leuven, Belgium Tel.: +32-16-343841; Fax: +32-16-343842, BE aut Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer