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产后急性布加综合征:一例漏诊导致病情迅速恶化的病例

Acute Onset Budd-Chiari Syndrome in the Postpartum Period: A Case of Missed Diagnosis Leading to Rapid Deterioration.

作者信息

Alami Malalai, Yousofzai Bibi Sarah, Shafiq Salman, Mehmood Rida, Subhan Muhammad, Bibi Ruqiya

机构信息

Obstetrics and Gynaecology, Rabia Balkhi Hospital, Kabul, AFG.

Obstetrics and Gynaecology, Be Team Cure International Hospital, Kabul, AFG.

出版信息

Cureus. 2024 Nov 8;16(11):e73284. doi: 10.7759/cureus.73284. eCollection 2024 Nov.

DOI:10.7759/cureus.73284
PMID:39650885
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11625450/
Abstract

Budd-Chiari syndrome (BCS), a rare hepatic vein obstruction condition, poses significant risks during gestation and the postpartum period. We present the case of a 30-year-old primigravida at 32 weeks gestation admitted with weakness and lethargy, which was diagnosed with impending uterine rupture, HELLP syndrome, and intrauterine fetal demise. An emergency cesarean section was performed, delivering a stillborn baby and uncovering 700 milliliters of blood clots in her abdominal cavity. Postoperatively, she experienced severe headaches, chest tightness, epigastric pain, nausea, vomiting, persistent fever, and abdominal distension, all of which were severe and persistent. Although treatment for sepsis and HELLP syndrome was initiated, BCS was eventually confirmed through ultrasound and Doppler sonography, though advanced imaging techniques were limited. Aggressive treatment with antibiotics and supportive care could not prevent worsening liver function, leading to severe bradycardia and death within five days. This case underscores the necessity of prompt recognition and multidisciplinary management of BCS among pregnant and postpartum women to improve outcomes in this rare yet critical condition.

摘要

布加综合征(BCS)是一种罕见的肝静脉阻塞性疾病,在妊娠期和产后具有重大风险。我们报告一例32周妊娠的30岁初产妇,因虚弱和嗜睡入院,诊断为即将发生子宫破裂、HELLP综合征和宫内胎儿死亡。进行了急诊剖宫产,娩出一名死胎,并在其腹腔内发现700毫升血凝块。术后,她出现严重头痛、胸闷、上腹部疼痛、恶心、呕吐、持续发热和腹胀,所有症状均严重且持续存在。尽管开始了败血症和HELLP综合征的治疗,但最终通过超声和多普勒超声检查确诊为BCS,不过先进的成像技术有限。积极使用抗生素治疗和支持性护理未能阻止肝功能恶化,导致严重心动过缓并在五天内死亡。该病例强调了对孕妇和产后妇女中的BCS进行及时识别和多学科管理的必要性,以改善这种罕见但危急情况的治疗结果。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad46/11625450/3ce3e1fed896/cureus-0016-00000073284-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad46/11625450/3ce3e1fed896/cureus-0016-00000073284-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad46/11625450/3ce3e1fed896/cureus-0016-00000073284-i01.jpg

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本文引用的文献

1
[Budd-Chiari syndrome with hepatopulmonary syndrome: a case report and literature review].[布加综合征合并肝肺综合征:一例报告及文献复习]
Zhonghua Er Ke Za Zhi. 2024 Jan 2;62(1):71-75. doi: 10.3760/cma.j.cn112140-20230828-00139.
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Primary Budd-Chiari Syndrome.原发性布加综合征
N Engl J Med. 2023 Aug 24;389(8):769. doi: 10.1056/NEJMc2305391.
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Managing Budd-Chiari Syndrome with Ayurvedic Treatment: A Case Report.采用阿育吠陀治疗布加氏综合征:病例报告。
Altern Ther Health Med. 2023 Jul;29(5):74-77.
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Pregnancy outcomes in women with Budd-Chiari syndrome or portal vein thrombosis - a multicentre retrospective cohort study.布加综合征或门静脉血栓形成女性的妊娠结局:一项多中心回顾性队列研究。
BJOG. 2022 Mar;129(4):608-617. doi: 10.1111/1471-0528.16915. Epub 2021 Oct 4.
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Ultrasonography in pediatric Budd-Chiari syndrome.小儿布加综合征的超声检查。
Pediatr Radiol. 2020 Nov;50(12):1768-1780. doi: 10.1007/s00247-020-04753-7. Epub 2020 Sep 1.
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An Update on the Management of Budd-Chiari Syndrome.布加综合征治疗进展。
Dig Dis Sci. 2021 Jun;66(6):1780-1790. doi: 10.1007/s10620-020-06485-y. Epub 2020 Jul 20.
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Budd-Chiari syndrome.布加综合征
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Chronic Budd-Chiari syndrome in Behçet's disease successfully managed with transjugular intrahepatic portosystemic shunt: a case report and literature review.Behçet 病并发慢性 Budd-Chiari 综合征经经颈静脉肝内门体分流术成功治疗:病例报告及文献复习。
Clin J Gastroenterol. 2020 Aug;13(4):572-578. doi: 10.1007/s12328-020-01106-7. Epub 2020 Feb 17.
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Budd-Chiari syndrome - various etiologies and imagistic findings. A pictorial review.布加综合征——多种病因及影像学表现。图文综述。
Med Ultrason. 2019 Aug 31;21(3):344-348. doi: 10.11152/mu-1921.
10
Epidemiology of Budd-Chiari syndrome: A systematic review and meta-analysis.布加综合征的流行病学:一项系统评价与荟萃分析。
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