Neonatal Medicine

  • 제17권1호
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  • Pages.132-135
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  • 2010
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  • 2287-9412(pISSN)
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  • 2287-9803(eISSN)
대한신생아학회 (The Korean Society of Neonatology)
권호 표지

미숙아에서 진단된 혈우병 A 1례

A Case of Hemophilia A Diagnosed in a Premature Infant

  • 오기원 (울산대학교 의과대학 울산대학교병원 소아청소년과) ;
  • 이경연 (울산대학교 의과대학 울산대학교병원 소아청소년과) ;
  • 김자형 (울산대학교 의과대학 울산대학교병원 소아청소년과) ;
  • 이강원 (울산대학교 의과대학 울산대학교병원 소아청소년과) ;
  • 정진영 (울산대학교 의과대학 울산대학교병원 소아청소년과) ;
  • 박상규 (울산대학교 의과대학 울산대학교병원 소아청소년과)
  • Oh, Ki-Won (Department of Pediatrics, Ulsan University Hospital, University of Ulsan College of Medicine) ;
  • Lee, Kyung-Yeon (Department of Pediatrics, Ulsan University Hospital, University of Ulsan College of Medicine) ;
  • Kim, Ja-Hyeong (Department of Pediatrics, Ulsan University Hospital, University of Ulsan College of Medicine) ;
  • Rhee, Kang-Won (Department of Pediatrics, Ulsan University Hospital, University of Ulsan College of Medicine) ;
  • Jeong, Jin-Young (Department of Pediatrics, Ulsan University Hospital, University of Ulsan College of Medicine) ;
  • Park, Sang-Kyu (Department of Pediatrics, Ulsan University Hospital, University of Ulsan College of Medicine)
  • 발행 : 2010.05.31
⟨ 이전 논문 다음 논문 ⟩

초록

미숙아에서 비정상적인 출혈의 대부분은 감염과 관련되어 발생하는 후천성 응고 장애가 원인인 경우가 많으므로 혈우병과 같은 유전응고장애질환은 간과되기 쉽다. 저자들은 반복되는 출혈 경향을 보인 재태 기간 31주 1일, 1,880 g으로 출생한 미숙아에서 혈우병 A를 진단하고 VIII인자 보충을 통해 치료하였기에 보고하는 바이다.

Although the majority of abnormal bleeding during the neonatal period results from acquired coagulation disorders, inherited coagulation disorders can also manifest at this time. Hemophilia is the most common of inherited coagulation disorder. Although 40-70% of cases with hemophilia are diagnosed in the neonatal period, few cases have been reported in premature infants. We report a case of a premature infant born at 31 weeks of gestation, diagnosed with hemophilia A by blood coagulation test, coagulation factor assay and study of the F8 gene. The baby was treated with recombinant factor VIII (Recombinate$^{(R)}$, USA) because of repeated seizures and intramuscular hematoma.

키워드

참고문헌

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