Adnan Alkurdi, Johannes Herrmann, Damir Bikmukhametov, Rebecca Tschöpe
{"title":"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","authors":"Adnan Alkurdi, Johannes Herrmann, Damir Bikmukhametov, Rebecca Tschöpe","doi":"10.1159/000537957","DOIUrl":null,"url":null,"abstract":"<p><strong>Introduction: </strong>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.</p><p><strong>Case presentation: </strong>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.</p><p><strong>Conclusion: </strong>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.</p>","PeriodicalId":9614,"journal":{"name":"Case Reports in Gastroenterology","volume":null,"pages":null},"PeriodicalIF":0.5000,"publicationDate":"2024-05-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11087035/pdf/","citationCount":"0","resultStr":"{\"title\":\"Biliary Cast Syndrome and Secondary Sclerosing Cholangitis in Critically Ill Patient after Long-Term Treatment in the Intensive Care Unit.\",\"authors\":\"Adnan Alkurdi, Johannes Herrmann, Damir Bikmukhametov, Rebecca Tschöpe\",\"doi\":\"10.1159/000537957\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Introduction: </strong>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.</p><p><strong>Case presentation: </strong>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.</p><p><strong>Conclusion: </strong>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.</p>\",\"PeriodicalId\":9614,\"journal\":{\"name\":\"Case Reports in Gastroenterology\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":0.5000,\"publicationDate\":\"2024-05-10\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11087035/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Case Reports in Gastroenterology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1159/000537957\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2024/1/1 0:00:00\",\"PubModel\":\"eCollection\",\"JCR\":\"Q4\",\"JCRName\":\"GASTROENTEROLOGY & HEPATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Case Reports in Gastroenterology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1159/000537957","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/1/1 0:00:00","PubModel":"eCollection","JCR":"Q4","JCRName":"GASTROENTEROLOGY & HEPATOLOGY","Score":null,"Total":0}
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.