Tofil Nancy M, Winkler Margaret K, Watts Raymond G, Noonan Jacqueline
Department of Pediatrics, Division of Critical Care, University of Alabama at Birmingham, Birmingham, AL, USA.
Pediatr Crit Care Med. 2005 May;6(3):352-4. doi: 10.1097/01.PCC.0000160656.71424.D1.
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.
Case report.
Pediatric intensive care unit of a children's hospital.
Ten-month-old with Noonan syndrome and massive gastrointestinal bleeding resulting in severe hypovolemic shock.
Recombinant factor VIIa was used in this patient's severe bleeding associated with Noonan syndrome after no other supportive measures were successful.
Recombinant Factor VIIa significantly decreased the patient's bleeding and allowed his hypovolemic shock to improve. Ultimately, the patient made a complete recovery.
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.
报告1例努南综合征患者，该患者在心脏手术后不久发生危及生命的胃肠道出血，经重组凝血因子VIIa成功治疗。
病例报告。
儿童医院的儿科重症监护病房。
一名10个月大的努南综合征患儿，发生大量胃肠道出血，导致严重低血容量性休克。
在其他支持措施均未成功的情况下，该患者因努南综合征相关的严重出血接受了重组凝血因子VIIa治疗。
重组凝血因子VIIa显著减少了患者的出血，并使其低血容量性休克得到改善。最终，患者完全康复。
努南综合征具有一系列心脏和非心脏畸形，包括出血风险增加，重组凝血因子VIIa是治疗严重出血的重要药物。
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