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논문 기본 정보

자료유형
학술저널
저자정보
Ha Nuel Chon (Department of Pediatrics Kyung Hee University Hospital at Gangdong Seoul Korea) Ja Hyang Cho (Department of Pediatrics Kyung Hee University Hospital at Gangdong Seoul Korea) 박영실 (Department of Pediatrics Kyung Hee University Hospital at Gangdong Seoul Korea)
저널정보
대한소아혈액종양학회 Clinical Pediatric Hematology-Oncology Clinical Pediatric Hematology-Oncology Vol.27 No.2
발행연도
2020.1
수록면
113 - 119 (7page)

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Background: Establishing hemostasis for surgical procedures in children with hereditary bleeding disorders is challenging. We evaluated the results of surgical procedures in children with hereditary bleeding disorders at our center and reviewed the preoperative management and hemorrhagic complications. Methods: We conducted a retrospective electronic medical record review from October 2006 to September 2019. Children with hereditary bleeding disorders who had elective surgeries or emergency operations were identified by an electronic record search. The primary focus was a review of clotting factor replacement strategies and bleeding complications. Results: In total, 126 elective procedures and 4 emergency surgeries were performed on 95 children at our center. Of the 95 children, hemophilia A, hemophilia B, von Willebrand disease, and factor VII deficiency were 74, 15, 4, and 2, respectively. The main disease distribution of procedures was 99 with hemophilia A, 24 with hemophilia B, and 4 with von Willebrand disease. Procedures included various orthopedic surgeries (87/130, 66.9%), placement or revision of a central venous catheter (8/130, 6.2%), and otolaryngology procedures (7/130, 5.4%). All patients received preoperative clotting factor replacement followed by various postoperative clotting factor replacement regimens. Thirteen procedures (10.0%) in five children were performed in the presence of high titers of clotting factor inhibitors. No deaths or life-threatening bleeding occurred after any procedure. Nine of the 130 procedures (6.9%) were complicated by postoperative bleeding. Tonsillectomy and adenoidectomy were the most common procedures complicated by hemorrhage (3/5, 60%). Conclusion: Surgical procedures are safe in children with hereditary bleeding disorders with adequate preparation and replacement of clotting factors. Bleeding remains a problem in a subset of patients and requires ongoing hematological involvement and oversight. Delayed bleeding following tonsillectomy was particularly common and suggests a need for close follow-up and ongoing factor administration for this group of patients.
#Bleeding disorder #Hemophilia #von Willebrand disease #Surgical procedure #Perioperative

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참고문헌 (18)

참고문헌 신청
Watts RG / 2012 / Operative management and outcomes in children with congenital bleeding disorders : a retrospective review at a single haemophilia treatment centre / Haemophilia 18:421~425 google schola Berntorp E / 1995 / Modern treatment of haemophilia / Bull World Health Organ 73:691~701 google schola Coppola A / 2010 / Treatment of hemophilia : a review of current advances and ongoing issues / J Blood Med 1:183~195 google schola Hermans C / 2009 / Replacement therapy for invasive procedures in patients with haemophilia: literature review, European survey and recommendations / Haemophilia 15:639~658 google schola Chee YL / 2008 / Guidelines on the assessment of bleeding risk prior to surgery or invasive procedures. British Committee for Standards in Haematology / Br J Haematol 140:496~504 google schola

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