DOI QR코드

DOI QR Code

패혈쇼크의 임상 양상으로 발현된 전신 모세혈관누출 증후군 1예

A Case of Systemic Capillary Leak Syndrome Presenting as Septic Shock

  • 조세희 (연세대학교 의과대학 강남세브란스병원 내과) ;
  • 최아란 (연세대학교 의과대학 강남세브란스병원 내과) ;
  • 이서희 (연세대학교 의과대학 강남세브란스병원 내과) ;
  • 조미옥 (연세대학교 의과대학 강남세브란스병원 내과) ;
  • 김태훈 (연세대학교 의과대학 강남세브란스병원 내과) ;
  • 진성준 (연세대학교 의과대학 강남세브란스병원 내과) ;
  • 송영구 (연세대학교 의과대학 강남세브란스병원 내과)
  • Jo, Sehee (Department of Internal Medicine, Gangnam Severance Hospital, Yonsei University College of Medicine) ;
  • Choi, Ahran (Department of Internal Medicine, Gangnam Severance Hospital, Yonsei University College of Medicine) ;
  • Lee, Seohui (Department of Internal Medicine, Gangnam Severance Hospital, Yonsei University College of Medicine) ;
  • Jo, Miok (Department of Internal Medicine, Gangnam Severance Hospital, Yonsei University College of Medicine) ;
  • Kim, Tae Hoon (Department of Internal Medicine, Gangnam Severance Hospital, Yonsei University College of Medicine) ;
  • Jin, Sung joon (Department of Internal Medicine, Gangnam Severance Hospital, Yonsei University College of Medicine) ;
  • Song, Young Goo (Department of Internal Medicine, Gangnam Severance Hospital, Yonsei University College of Medicine)
  • 투고 : 2014.02.26
  • 심사 : 2014.05.29
  • 발행 : 2014.12.01

초록

전신 모세혈관누출 증후군은 다발성 장기부전 증상이 나타나는 드문 질환으로, 가역적이고 순간적인 전신 혈관장벽의 장애로 나타나는 저혈압, 혈액 농축, 저알부민혈증으로 정의된다. 본 증례는 발열, 상기도 감염 증상, 저혈압으로 응급실로 온 36세 여자 환자에서 혈액 농축, 저알부민혈증, 급성신부전 소견을 보였고 패혈쇼크와 감별이 어려웠으나 전신 모세혈관누출 증후군을 진단하고 치료에 성공하였던 경우로 문헌 고찰과 함께 보고하는 바이다.

Systemic capillary leak syndrome is a rare disease characterized by life-threatening attacks of reversible plasma extravasation and vascular collapse accompanied by hypotension, hemoconcentration, and hypoalbuminemia. A 36-year-old woman was admitted to this hospital with a fever, along with symptoms consistent with an upper respiratory tract infection and hypotension. Initial laboratory tests revealed several abnormal findings, including an elevated leukocyte count and hematocrit, hypoalbuminemia, and acute renal failure. Here, we report a case of successful treatment of systemic capillary leak syndrome, which can be difficult to distinguish from septic shock.

키워드

참고문헌

  1. Clarkson B, Thompson D, Horwith M, Luckey EH. Cyclical edema and shock due to increased capillary permeability. Am J Med 1960;29:193-216. https://doi.org/10.1016/0002-9343(60)90018-8
  2. Druey KM, Greipp PR. Narrative review: the systemic capillary leak syndrome. Ann Intern Med 2010;153:90-98. https://doi.org/10.7326/0003-4819-153-2-201007200-00005
  3. Cho EJ, Cha IH, Yoon KC, et al. Recurrent systemic capillary leak syndrome responding to the corticosteroid therapy. Korean J Nephrol 2010;29:513-518.
  4. Lee YS, Kim SY, Kwon CW, et al. Two cases of systemic capillary leak syndrome that were treated with pentastarch. Korean J Intern Med 2007;22:130-132. https://doi.org/10.3904/kjim.2007.22.2.130
  5. Amoura Z, Papo T, Ninet J, et al. Systemic capillary leak syndrome: report on 13 patients with special focus on course and treatment. Am J Med 1997;103:514-519. https://doi.org/10.1016/S0002-9343(97)00272-6
  6. Marx G, Pedder S, Smith L, et al. Resuscitation from septic shock with capillary leakage: hydroxyethyl starch (130 kd), but not Ringer's solution maintains plasma volume and systemic oxygenation. Shock 2004;21:336-341. https://doi.org/10.1097/00024382-200404000-00008
  7. Kapoor P, Greipp PT, Schaefer EW, et al. Idiopathic systemic capillary leak syndrome (Clarkson's disease): the Mayo clinic experience. Mayo Clin Proc 2010;85:905-912. https://doi.org/10.4065/mcp.2010.0159
  8. Gousseff M, Arnaud L, Lambert M, et al. The systemic capillary leak syndrome: a case series of 28 patients from a European registry. Ann Intern Med 2011;154:464-471. https://doi.org/10.7326/0003-4819-154-7-201104050-00004
  9. Xie Z, Ghosh CC, Patel R, et al. Vascular endothelial hyperpermeability induces the clinical symptoms of Clarkson disease (the systemic capillary leak syndrome). Blood 2012;119:4321-4332. https://doi.org/10.1182/blood-2011-08-375816
  10. Assaly R, Olson D, Hammersley J, et al. Initial evidence of endothelial cell apoptosis as a mechanism of systemic capillary leak syndrome. Chest 2001;120:1301-1308. https://doi.org/10.1378/chest.120.4.1301