A Case of Pulmonary Thromboembolism Due to Congenital Antithrombin III Deficiency

선천성 Antithrombin III 결핍증에서 발생한 폐색전증 1 예

  • Park, Hyeong-Kwan (Pulmonary Department of Internal Medicine, Chonnam University Medical School) ;
  • Park, Chang-Min (Pulmonary Department of Internal Medicine, Chonnam University Medical School) ;
  • Ko, Kyoung-Haeng (Pulmonary Department of Internal Medicine, Chonnam University Medical School) ;
  • Rim, Myung-Soo (Pulmonary Department of Internal Medicine, Chonnam University Medical School) ;
  • Kim, Yu-Il (Pulmonary Department of Internal Medicine, Chonnam University Medical School) ;
  • Hwang, Jun-Hwa (Pulmonary Department of Internal Medicine, Chonnam University Medical School) ;
  • Lim, Sung-Chul (Pulmonary Department of Internal Medicine, Chonnam University Medical School) ;
  • Kim, Young-Chul (Pulmonary Department of Internal Medicine, Chonnam University Medical School) ;
  • Park, Kyung-Ok (Pulmonary Department of Internal Medicine, Chonnam University Medical School)
  • 박형관 (전남대학교 의과대학 내과학교실) ;
  • 박창민 (전남대학교 의과대학 내과학교실) ;
  • 고경행 (전남대학교 의과대학 내과학교실) ;
  • 임명수 (전남대학교 의과대학 내과학교실) ;
  • 김유일 (전남대학교 의과대학 내과학교실) ;
  • 황준화 (전남대학교 의과대학 내과학교실) ;
  • 임성철 (전남대학교 의과대학 내과학교실) ;
  • 김영철 (전남대학교 의과대학 내과학교실) ;
  • 박경옥 (전남대학교 의과대학 내과학교실)
  • Published : 1999.09.30

Abstract

We report a case of congenital and familial antithrombin III deficiency developing massive pulmonary thromboembolism. A 44-year-old man was admitted to our hospital because of sudden chest pain and severe dyspnea. Five years ago, he was operated due to a mesenteric vein thrombosis of unknown cause. On admission, radioisotopic venogram showed deep vein thrombosis and lung scintigram showed multiple segmental perfusion defects. His plasma antithrombin III level was 10.5 mg/dL which was less than 50% of normal and those of a son and two daughters were also decreased. After treatment with tissue plasminogen activator, heparin and coumadin, his symptom and lung scintigram were significantly improved. As far as we reviewed, there were very rare reports with congenital antithrombin III deficiency presenting as pulmonary thromboembolism in Korea.

저자들은 갑작스런 흉통을 주소로 내원한 젊은 남자에서 AT III 결핍증에 의한 폐색전증을 진단하고 세 자녀 모두에서도 AT III 결핍이 확인됨으로써 폐색전증을 동반한 선천성, 가족성 AT III 결핍증을 경험하였기에 문헌고찰과 함께 보고한다.

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