The use of recombinant factor VIIa in a patient with Noonan syndrome and life-threatening bleeding.
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.
Tofil
Nancy M
NM
Department of Pediatrics, Division of Critical Care, University of Alabama at Birmingham, Birmingham, AL, USA.
Winkler
Margaret K
MK
Watts
Raymond G
RG
Noonan
Jacqueline
J
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
D006490:Hemostatics; D011994:Recombinant Proteins; D005167:Factor VII; C103587:recombinant FVIIa; D015942:Factor VIIa