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原发性胆汁性肝硬化合并严重低氧血症。

Primary biliary cirrhosis complicated by severe hypoxemia.

作者信息

Shiomi S, Kuroki T, Fujimoto S, Yamaoka M, Sakaguchi H, Takeda T, Nishiguchi S, Nakajima S, Kobayashi K, Ochi H

机构信息

Third Department of Internal Medicine, Osaka City University Medical School, Japan.

出版信息

J Gastroenterol. 1995 Dec;30(6):790-4. doi: 10.1007/BF02349650.

DOI:10.1007/BF02349650
PMID:8963401
Abstract

The case reported here is of a 61-year-old woman diagnosed with hepatopulmonary syndrome. She had had severe hypoxemia for 5 years. In room air, her arterial oxygen tension was low, and although we anticipated that treatment with oxygen would increase it, the treatment failed to do so. Pulmonary perfusion imaging with Tc-99m-labelled macroaggregated albumin showed an arterio-venous shunt in the lungs. Blood tests and liver scanning with Tc-99m-labelled galactosyl human serum albumin showed abnormalities of liver function, and per-rectal portal scintigraphy with Tc-99m pertechnetate showed severe portal hypertension. Inspection of a biopsy specimen taken under ultrasonographic guidance showed primary biliary cirrhosis of Scheuer stage I. The causes of hepatopulmonary syndrome are unknown, but seem to include a decrease in hepatic functional reserve, portal hypertension, or other factors. The development of hepatopulmonary syndrome due to portal hypertension in this patient is of interest.

摘要

本文报道的病例为一名61岁女性,被诊断为肝肺综合征。她患有严重低氧血症已达5年。在室内空气中,她的动脉血氧张力较低,尽管我们预计吸氧治疗会使其升高,但治疗并未达到预期效果。用99m锝标记的大颗粒聚合白蛋白进行肺灌注显像显示肺部存在动静脉分流。血液检查以及用99m锝标记的半乳糖基人血清白蛋白进行肝脏扫描显示肝功能异常,而用99m锝高锝酸盐进行经直肠门静脉闪烁显像显示严重门静脉高压。对在超声引导下获取的活检标本进行检查显示为Scheuer I期原发性胆汁性肝硬化。肝肺综合征的病因尚不清楚,但似乎包括肝功能储备减少、门静脉高压或其他因素。该患者因门静脉高压导致肝肺综合征的情况值得关注。

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1
Primary biliary cirrhosis complicated by severe hypoxemia.原发性胆汁性肝硬化合并严重低氧血症。
J Gastroenterol. 1995 Dec;30(6):790-4. doi: 10.1007/BF02349650.
2
Portal hemodynamics in primary biliary cirrhosis as evaluated by per-rectal portal scintigraphy with Tc-99m pertechnetate.用锝-99m高锝酸盐经直肠门静脉闪烁显像评估原发性胆汁性肝硬化的门静脉血流动力学。
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引用本文的文献

1
Hematologic traits and primary biliary cholangitis: a Mendelian randomization study.血液学特征与原发性胆汁性胆管炎:一项孟德尔随机化研究。
J Hum Genet. 2023 Aug;68(8):559-563. doi: 10.1038/s10038-023-01146-0. Epub 2023 Apr 3.

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