title: Medical management of neurofibromatosis 1: a cross-sectional study of 383 patients.
abstract: BACKGROUND
  The morbidity and mortality caused by neurofibromatosis 1 are a result of complications that may involve any of the body systems. Two models of management have been proposed for the detection of various complications in specialized neurofibromatosis clinics: investigation protocols (including extensive imaging and analysis of 24-hour urinary catecholamine levels); or clinical follow-up without imaging.
  
  
  OBJECTIVE
  Our purpose was to validate the strategy of clinical follow-up (without routine imaging and 24-hour urinary catecholamine levels).
  
  
  METHODS
  We retrospectively compared the number of treated complications during 2 successive periods from our database: screening investigations from November 1988 to June 1995 and clinical examination from July 1995 to June 2000.
  
  
  RESULTS
  The number of treated complications during the 2 periods was not statistically different (27/166 vs 28/217; Fisher's exact test, P =.39).
  
  
  CONCLUSIONS
  Screening investigations added little to clinical follow-up. Indeed, routine clinical examination can easily identify complications that require treatment in adult patients with neurofibromatosis 1.
authors.affiliation: Department of Dermatology, Henri-Mondor Hospital, AP-HP, Paris XII University, Créteil, France.
authors.forename: Jean-Christophe
authors.initials: JC
authors.lastname: Drappier
authors.forename: Kiarash
authors.initials: K
authors.lastname: Khosrotehrani
authors.forename: Jacques
authors.initials: J
authors.lastname: Zeller
authors.forename: Jean
authors.initials: J
authors.lastname: Revuz
authors.forename: Pierre
authors.initials: P
authors.lastname: Wolkenstein
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D002395:Catecholamines / Q000032:analysis*; D003430:Cross-Sectional Studies; D003952:Diagnostic Imaging / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D008403:Mass Screening / Q000379:methods*; D008875:Middle Aged; D009456:Neurofibromatosis 1 / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D018570:Risk Assessment; D012680:Sensitivity and Specificity; D012720:Severity of Illness Index; D017678:Sex Distribution; D015996:Survival Rate
chemical_list: D002395:Catecholamines