title: ACE inhibitorenalapril diminishes albuminuria in patients with cystinosis.
abstract: OBJECTIVE
  Cystinosis, a rare autosomal recessive disease, manifests with renal Fanconi syndrome during the first year of life. Interstitial damage is a major cause of renal failure in patients with cystinosis. We presume that albuminuria contributes to the development of renal failure in these patients. The aim of this study was to examine whether the administration of ACE inhibitor enalapril diminishes albuminuria in patients with cystinosis.
  
  
  METHODS
  Five patients with cystinosis aged 4 - 9 years were studied. All patients had Fanconi syndrome and were treated with cysteamine. Median creatinine clearance was 48 ml/min/1.73 m2 (range 21 - 61). The excretion of albumin and alpha1 microglobulin as well as arterial blood pressure and serum creatinine were evaluated before and at 3 months on oral administration of enalapril (0.15 mg/kg once daily).
  
  
  RESULTS
  At 3 months on enalapril, albuminuria decreased in all patients (1,042 vs 629 mg per 24 h, p < 0.05). The median reduction of albuminuria was 43% (range: 4 - 72%, p < 0.05). Urinary excretion of alpha1 microglobulin remained constant. Systolic blood pressure decreased from median 110 - 100 mmHg (p < 0.05), while diastolic blood pressure remained stable (median 60 mmHg). Creatinine clearance decreased from median 48 - 45 ml/min/1.73 m2 (p < 0.05) and returned to previous values after discontinuation of enalapril.
  
  
  CONCLUSIONS
  ACE inhibitor enalapril diminishes albuminuria in patients with cystinosis and might be used in these patients in order to slow the progression of renal insufficiency attributed to proteinuria.
authors.affiliation: Department of Paediatric Nephrology, University Medical Centre, Nijmegen, The Netherlands. e.levtchenko@cukz.umcn.nl
authors.forename: E
authors.initials: E
authors.lastname: Levtchenko
authors.forename: H
authors.initials: H
authors.lastname: Blom
authors.forename: M
authors.initials: M
authors.lastname: Wilmer
authors.forename: L
authors.initials: L
authors.lastname: van den Heuvel
authors.forename: L
authors.initials: L
authors.lastname: Monnens
mesh_terms: D000419:Albuminuria / Q000188:drug therapy* / Q000209:etiology; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use*; D001794:Blood Pressure; D002648:Child; D002675:Child, Preschool; D003404:Creatinine / Q000097:blood / Q000652:urine; D003554:Cystinosis / Q000150:complications*; D004656:Enalapril / Q000627:therapeutic use*; D006801:Humans; D011507:Proteinuria / Q000150:complications; D018709:Statistics, Nonparametric; D016896:Treatment Outcome
chemical_list: D000806:Angiotensin-Converting Enzyme Inhibitors; D004656:Enalapril; D003404:Creatinine