본문 바로가기
KERD
논문KCI 등재

Hereditary protein S deficiency presenting acute pulmonary embolism

1

조회

0

다운로드

0

인용

0

참고문헌

키워드

주제분류의학일반

초록

Protein S deficiency is one of the several risk factors for thrombophilia and can cause blood clotting disorders such as deep vein thrombosis and pulmonary embolism. A 54-year-old man was admitted with the complaint of dyspnea and was diagnosed with pulmonary embolism. The patient had very low level of free protein S, total protein S antigen, and protein S activity (type I protein S deficiency). In history taking, we found that his mother, 78 year old, had a history of same disease 10 years ago, and confirmed the pronounced low level of protein S. The patient’s son also had very low level of protein S, however there had not been any history of pulmonary embolism yet. This case study suggests that asymptomatic persons with a family history of protein S deficiency and pulmonary embolism should be checked regularly for early detection of the disease, as protein S deficiency can be suspected.

Abstract

Protein S deficiency is one of the several risk factors for thrombophilia and can cause blood clotting disorders such as deep vein thrombosis and pulmonary embolism. A 54-year-old man was admitted with the complaint of dyspnea and was diagnosed with pulmonary embolism. The patient had very low level of free protein S, total protein S antigen, and protein S activity (type I protein S deficiency). In history taking, we found that his mother, 78 year old, had a history of same disease 10 years ago, and confirmed the pronounced low level of protein S. The patient’s son also had very low level of protein S, however there had not been any history of pulmonary embolism yet. This case study suggests that asymptomatic persons with a family history of protein S deficiency and pulmonary embolism should be checked regularly for early detection of the disease, as protein S deficiency can be suspected.

참고문헌 (13)

  1. Goodwin AJ, "A review of the technical, diagnostic, and epidemiologic considerations for protein S assays", Arch Pathol Lab Med, 126, pp.1349~1366 (2002)
  2. Comp PC, "Familial protein S deficiency is associated with recurrent thrombosis", J Clin Invest, 74, pp.2082~2088 (1984)
  3. Walker FJ, "Regulation of activated protein C by a new protein. a possible function for bovine protein S", J Biol Chem, 255, pp.5521~5524 (1980)
  4. Dahlbäck B, "The tale of protein S and C4b-binding protein, a story of affection", Thromb Haemost, 98, pp.90~96 (2007)
  5. Dykes AC, "A study of Protein S antigen levels in 3788 healthy volunteers:influence of age, sex and hormone use, and estimate for prevalence of deficiency state", Br J Haematol, 113, pp.636~641 (2001)
  6. Rezende SM, "Coagulation, inflammation, and apoptosis: different roles for protein S and the protein S-C4b binding protein complex", Blood, 103, pp.1192~1201 (2004)
  7. D’Angelo A, "Protein S deficiency", Haematologica, 93, pp.498~501 (2008)
  8. ten Kate MK, "Protein S deficiency: a clinical perspective", Haemophilia, 14, pp.1222~1228 (2008)
  9. Lijfering WM, "Clinical relevance of decreased free protein S levels: results from a retrospective family cohort study involving 1143 relatives", Blood, 113, pp.1225~1230 (2009)
  10. Yoo BH, "A case of isolated protein S deficiency, complicated by acute pulmonary thromboembolism after coronary angiography", Korean Circ J, 30, pp.876~880 (2000)
  11. 박정화, "천식환자에서 병발된 S 단백 결핍과 연관된 폐동맥 색전증 1예", 천식 및 알레르기, 30(2), pp.135~139 (2010), 대한천식알레르기학회
  12. 박성민, "심첨부 비후형 심근증에서 C, S 단백이 동반 결핍된 폐동맥 색전증 1예", 고신대학교 의과대학 학술지, 26(1), pp.84~88 (2011), 고신대학교 의과대학 학술지
  13. 김선광, "노인 S단백 결핍 환자에게서 발생한 재발성 폐색전증", Annals of geriatric medicine and research, 17(1), pp.55~58 (2013), 대한노인병학회[DOI]

관련 논문AI추천

이 간행물의 다른 논문 (14건)