title: Proteinuria in Frasier syndrome.
abstract: BACKGROUND
  Frasier syndrome (FS) is a genetic form of glomerulopathy, which results from mutations in the Wilms'tumour suppressor gene (WT1). Proteinuria in FS has been traditionally considered unresponsive to any medication and FS inevitably progresses to end stage renal failure.
  
  
  METHODS
  We present a patient with FS who had atypical clinical manifestation and unusual beneficial antiproteinuric response to renin-angiotensin system (RAS) inhibitors given in combination with indomethacin. After 13 years of follow-up, the patient is now 17-year old with normal renal functions and no proteinuria.
  
  
  CONCLUSIONS
  RAS inhibitors combined with indomethacin showed beneficial effect in our patient. Thus, this combination might be the initial treatment of patients with FS. If this treatment strategy was not satisfied for at least 3 months, then CsA would be considered to be administered taking account of the nephrotoxicity and the increased risk of malignancy. Further prospective study is required to clarify this issue.
authors.affiliation: School of Medicine, University of Belgrade, Belgrade, Serbia. amira@udk.bg.ac.rs
authors.forename: Amira
authors.initials: A
authors.lastname: Peco-Antić
authors.affiliation: Pediatric Nephrology Unit, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.
authors.forename: Fatih
authors.initials: F
authors.lastname: Ozaltin
authors.affiliation: School of Medicine, University of Belgrade, Belgrade, Serbia.
authors.forename: Vojislav
authors.initials: V
authors.lastname: Parezanović
authors.affiliation: Nephrology Unit, University Children's Hospital, Belgrade, Serbia.
authors.forename: Gordana
authors.initials: G
authors.lastname: Milosevski-Lomić
authors.affiliation: School of Medicine, University of Belgrade, Belgrade, Serbia.
authors.forename: Verica
authors.initials: V
authors.lastname: Zdravković
mesh_terms: D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use*; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use*; D002675:Child, Preschool; D005260:Female; D052159:Frasier Syndrome / Q000150:complications*; D006801:Humans; D011507:Proteinuria / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology
chemical_list: D000806:Angiotensin-Converting Enzyme Inhibitors; D000894:Anti-Inflammatory Agents, Non-Steroidal