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妊娠期难治性系统性毛细血管渗漏综合征(克拉克森病)1例。

A Case of Refractory Systemic Capillary Leak Syndrome (Clarkson's Disease) during Pregnancy.

作者信息

Hikasa Yukiko, Hayashi Masao, Suzuki Satoshi, Morimatsu Hiroshi

机构信息

Department of Anesthesiology and Resuscitology, Okayama University Hospital, Okayama 700-8558,

出版信息

Acta Med Okayama. 2016 Dec;70(6):497-501. doi: 10.18926/AMO/54814.

DOI:10.18926/AMO/54814
PMID:28003676
Abstract

A 32-year-old woman, pregnant with twins, presented with a chief complaint of general fatigue. Her general condition had rapidly deteriorated since her last visit to the primary obstetrician; the patient was then referred to our hospital because of suspected fetal death. She underwent emergency cesarean section because fetal death had indeed occurred, and she was then admitted to the intensive care unit (ICU). On ICU admission, she was found to be in shock. Laboratory analysis revealed extreme hemoconcentration and a low albumin level, and initially, septic shock with obstetric complications was suspected. However, because she did not respond to conventional therapy but instead, rapidly developed severe generalized edema, systemic capillary leak syndrome (SCLS) was diagnosed. The patient remained in shock for several days until undergoing plasma exchange (PE), despite some earlier empirical treatments. She eventually recovered from profound shock status and was discharged from the ICU without sequelae. Among potentially effective treatments, PE seemed to be the most reasonable choice for the treatment of her SCLS.

摘要

一名怀有双胞胎的32岁女性,以全身乏力为主诉前来就诊。自她上次就诊于初级产科医生后,其一般状况迅速恶化;随后,该患者因怀疑胎儿死亡而被转诊至我院。由于胎儿确实已死亡,她接受了紧急剖宫产手术,随后被收入重症监护病房(ICU)。入住ICU时,发现她处于休克状态。实验室分析显示有极度血液浓缩和低白蛋白水平,最初怀疑是伴有产科并发症的感染性休克。然而,由于她对常规治疗无反应,反而迅速出现严重的全身性水肿,故诊断为系统性毛细血管渗漏综合征(SCLS)。尽管早期进行了一些经验性治疗,但该患者在接受血浆置换(PE)之前,休克状态持续了数天。她最终从深度休克状态中康复,从ICU出院,无后遗症。在可能有效的治疗方法中,PE似乎是治疗她的SCLS最合理的选择。

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