title: Elimination of pain and improvement of exercise capacity in Camurati-Engelmann disease with losartan.
abstract: BACKGROUND
  Camurati-Engelmann disease (CED) is a rare disorder, with approximately 250 described cases in the literature. Treatment options are limited and have been suboptimal so far.
  
  
  METHODS
  A prepubertal girl aged 9 years was diagnosed with CED. Treatment with losartan was initiated at a daily dose of 0.75 mg/kg. Over a period of 12 weeks, the dose was gradually increased to 1.0 mg/kg/d. The patient was reviewed in clinic regularly and underwent thorough clinical assessments 9, 17, and 38 months after treatment initiation.
  
  
  RESULTS
  The patient experienced marked clinical improvements with losartan. In particular, losartan treatment led to the complete elimination of the previously severe and incapacitating pain, with an increased ability to walk and perform physical activities. There was also a considerable improvement in body composition with increased lean and adipose tissue. Notably, the improvement in fat deposition had not been previously observed with other treatments in CED. Hematology, liver, and renal function tests were within normal ranges at presentation and remained so over the course of treatment.
  
  
  CONCLUSIONS
  In light of our findings, losartan may be a useful option in CED management.
authors.affiliation: Liggins Institute (A.A., J.G.B.D., W.S.C., P.L.H.), University of Auckland, Auckland 1142, New Zealand; and Department of Pediatric Endocrinology (W.S.C., P.L.H.), Greenlane Clinical Centre, Auckland District Health Board, Auckland 1051, New Zealand.
authors.forename: Ahila
authors.initials: A
authors.lastname: Ayyavoo
authors.forename: José G B
authors.initials: JG
authors.lastname: Derraik
authors.forename: Wayne S
authors.initials: WS
authors.lastname: Cutfield
authors.forename: Paul L
authors.initials: PL
authors.lastname: Hofman
mesh_terms: D047228:Angiotensin II Type 1 Receptor Blockers / Q000627:therapeutic use*; D003966:Camurati-Engelmann Syndrome / Q000150:complications / Q000188:drug therapy*; D002648:Child; D015444:Exercise; D005260:Female; D006801:Humans; D019808:Losartan / Q000627:therapeutic use*; D010146:Pain / Q000188:drug therapy* / Q000209:etiology; D016896:Treatment Outcome
chemical_list: D047228:Angiotensin II Type 1 Receptor Blockers; D019808:Losartan