title: The use of recombinant factor VIIa in a patient with Noonan syndrome and life-threatening bleeding.
abstract: OBJECTIVE
  To present a case report of a patient with Noonan syndrome who developed life-threatening gastrointestinal bleeding shortly after cardiac surgery that was successfully treated with recombinant factor VIIa.
  
  
  METHODS
  Case report.
  
  
  METHODS
  Pediatric intensive care unit of a children's hospital.
  
  
  METHODS
  Ten-month-old with Noonan syndrome and massive gastrointestinal bleeding resulting in severe hypovolemic shock.
  
  
  METHODS
  Recombinant factor VIIa was used in this patient's severe bleeding associated with Noonan syndrome after no other supportive measures were successful.
  
  
  RESULTS
  Recombinant Factor VIIa significantly decreased the patient's bleeding and allowed his hypovolemic shock to improve. Ultimately, the patient made a complete recovery.
  
  
  CONCLUSIONS
  Noonan syndrome has a constellation of both cardiac and noncardiac malformations including an increased risk of bleeding, and recombinant factor VIIa is an important agent in the treatment of significant bleeding.
authors.affiliation: Department of Pediatrics, Division of Critical Care, University of Alabama at Birmingham, Birmingham, AL, USA.
authors.forename: Nancy M
authors.initials: NM
authors.lastname: Tofil
authors.forename: Margaret K
authors.initials: MK
authors.lastname: Winkler
authors.forename: Raymond G
authors.initials: RG
authors.lastname: Watts
authors.forename: Jacqueline
authors.initials: J
authors.lastname: Noonan
mesh_terms: D006348:Cardiac Surgical Procedures / Q000009:adverse effects; D005167:Factor VII / Q000627:therapeutic use*; D015942:Factor VIIa; D006471:Gastrointestinal Hemorrhage / Q000188:drug therapy* / Q000209:etiology; D006490:Hemostatics / Q000627:therapeutic use*; D006801:Humans; D007223:Infant; D015278:Intensive Care Units, Pediatric; D008297:Male; D009634:Noonan Syndrome / Q000150:complications / Q000601:surgery*; D019106:Postoperative Hemorrhage / Q000188:drug therapy*; D011994:Recombinant Proteins / Q000627:therapeutic use
chemical_list: D006490:Hemostatics; D011994:Recombinant Proteins; D005167:Factor VII; C103587:recombinant FVIIa; D015942:Factor VIIa