重症监护室长期治疗后重症患者的胆道铸型综合征和继发性硬化性胆管炎

IF 0.5 Q4 GASTROENTEROLOGY & HEPATOLOGY Case Reports in Gastroenterology Pub Date : 2024-05-10 eCollection Date: 2024-01-01 DOI:10.1159/000537957
Adnan Alkurdi, Johannes Herrmann, Damir Bikmukhametov, Rebecca Tschöpe
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引用次数: 0

摘要

简介:重症患者继发性硬化性胆管炎(SSC-CIP)是一种罕见但诊断不足的疾病,多发生在危及生命的事件和重症监护室(ICU)的治疗之后。SSC-CIP 的病因尚不完全清楚,但可能是由缺血性胆管损伤引起的。SSC-CIP 是一种胆汁淤积性肝病,会迅速发展为肝硬化,第一年的死亡率高达 50%。内镜逆行胰胆管造影术(ERCP)是诊断 SSC-CIP 的金标准,可显示原发性 SC 样改变,通常发生在肝内胆管。胆管铸型形成是 SSC-CIP 的病理标志。SSC-CIP 目前尚无行之有效的保守治疗方法,当出现肝硬化或复发性胆管炎时,肝移植是唯一可治愈的治疗方法:我们报告了一例 47 岁男性患者的病例,他因重症肺炎在重症监护室接受长期治疗后出现胆汁淤积。ERCP 显示肝内胆管稀疏化和多节段狭窄。我们从胆管中取出了多个胆汁栓:结论:对于胆汁淤积不清的 ICU 患者,尤其是胆汁淤积在基础疾病康复后仍持续存在时,应考虑使用 SSC-CIP。早期诊断对取得更好的治疗效果非常重要;如果不进行肝移植,预后一般较差。
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Biliary Cast Syndrome and Secondary Sclerosing Cholangitis in Critically Ill Patient after Long-Term Treatment in the Intensive Care Unit.

Introduction: Secondary sclerosing cholangitis in critically ill patients (SSC-CIP) is a rare but underdiagnosed entity that occurs after life-threatening events and treatment in the intensive care unit (ICU). The etiology of SSC-CIP is not fully understood but may be caused by ischemic bile duct injury. SSC-CIP is a cholestatic liver disease that rapidly progresses to liver cirrhosis, with a high mortality rate in the first year of 50%. Endoscopic retrograde cholangiopancreatography (ERCP), which is the gold standard for diagnosing SSC-CIP, shows primary SC-like changes, usually in the intrahepatic bile ducts. Biliary cast formation is pathognomonic for SSC-CIP. No proven effective conservative treatment is available for SSC-CIP, and liver transplantation is the only curative therapy when liver cirrhosis or recurrent cholangitis occurs.

Case presentation: We report the case of a 47-year-old male patient who developed cholestasis after a long treatment in the ICU for severe pneumonia. ERCP showed characteristic findings with rarefication and multiple segmental stenosis in the intrahepatic bile ducts. We removed multiple biliary casts from the bile ducts.

Conclusion: SSC-CIP should be considered for ICU patients with unclear cholestasis, especially when the cholestasis persists after recovery from the underlying disease. Early diagnosis is important to achieve better outcomes; without liver transplantation, the prognosis is generally poor.

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来源期刊
Case Reports in Gastroenterology
Case Reports in Gastroenterology Medicine-Gastroenterology
CiteScore
1.10
自引率
0.00%
发文量
99
审稿时长
7 weeks
期刊介绍:
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