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Situs inversus totalis and secondary biliary cirrhosis: a case report.

作者信息

Sökmen Hacı Mehmet, Ozdil Kamil, Calhan Turan, Sahin Abdurrahman, Senateş Ebubekir, Kahraman Resul, Niğdelioğlu Adil, Zemheri Ebru

机构信息

Ümraniye Education and Research Hospital, Department of Gastroenterology, Istanbul, Turkey.

出版信息

Comp Hepatol. 2011 Aug 3;10:5. doi: 10.1186/1476-5926-10-5.

DOI:10.1186/1476-5926-10-5
PMID:21813017
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3161837/
Abstract

Situs inversus totalis is is a congenital anomaly associated with various visceral abnormalities, but there is no data about the relationship between secondary biliary cirrhosis and that condition. We here present a case of a 58 year-old female with situs inversus totalis who was admitted to our clinic with extrahepatic cholestasis. After excluding all potential causes of biliary cirrhosis, secondary biliary cirrhosis was diagnosed based on the patient's history, imaging techniques, clinical and laboratory findings, besides histolopathological findings. After treatment with tauroursodeoxycholic acid, all biochemical parameters, including total/direct bilirubin, alanine aminotransferase, aspartate aminotransferase, alkaline phosphatase and gama glutamyl transferase, returned to normal ranges at the second month of the treatment. We think that this is the first case in literature that may indicate the development of secondary biliary cirrhosis in a patient with situs inversus totalis. In conclusion, situs inversus should be considered as a rare cause of biliary cirrhosis in patients with situs inversus totalis which is presented with extrahepatic cholestasis.

摘要
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e4dc/3161837/8851b00d7567/1476-5926-10-5-6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e4dc/3161837/7d6030bcc401/1476-5926-10-5-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e4dc/3161837/b69d1d8abeaf/1476-5926-10-5-2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e4dc/3161837/dad8d0e371c7/1476-5926-10-5-3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e4dc/3161837/b8d819471cea/1476-5926-10-5-4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e4dc/3161837/461ece7550bd/1476-5926-10-5-5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e4dc/3161837/8851b00d7567/1476-5926-10-5-6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e4dc/3161837/7d6030bcc401/1476-5926-10-5-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e4dc/3161837/b69d1d8abeaf/1476-5926-10-5-2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e4dc/3161837/dad8d0e371c7/1476-5926-10-5-3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e4dc/3161837/b8d819471cea/1476-5926-10-5-4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e4dc/3161837/461ece7550bd/1476-5926-10-5-5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e4dc/3161837/8851b00d7567/1476-5926-10-5-6.jpg

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本文引用的文献

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Appendicular peritonitis in situs inversus totalis: a case report.全内脏转位患者的阑尾性腹膜炎:一例报告
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Mutations in INVS encoding inversin cause nephronophthisis type 2, linking renal cystic disease to the function of primary cilia and left-right axis determination.编码倒转蛋白的INVS基因突变会导致2型肾单位肾痨,将肾囊性疾病与初级纤毛的功能及左右轴的确定联系起来。
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