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Hereditary haemorrhagic telangiectasia (HHT) is a rare genetic disease characterised by mucocutaneous telangiectasias and arteriovenous malformations that can affect multiple organs. Although rare, ischaemic cholangiopathy can occur, a serious complication that can even lead to death. We present the case of a patient with HHT disease with previous mucocutaneous and gastrointestinal manifestations in whom 8 weeks after cholecystectomy a saccular dilatation of the intrahepatic bile duct was observed.

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Article Synopsis
  • - The study focused on creating a CT-enhanced scanning radiomics nomogram to differentiate between early hepatic abscess (EHA) and intrahepatic cholangiocarcinoma (ICC) and to test its effectiveness in diagnosis.
  • - Researchers analyzed data from 112 patients using 3D Slicer software for imaging, and various modeling techniques, including LASSO regression and ROC analysis, helped develop a predictive model and a joint nomogram combining radiomics and clinical scores.
  • - Results showed significant differences in various clinical and imaging features between EHA and ICC patients, indicating that the nomogram can potentially enhance diagnostic accuracy for these conditions.
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An 87-year-old woman was hospitalized for liver abscesses and cholangitis due to common bile duct stones. She developed worsening anemia and abdominal pain. Contrast-enhanced computed tomography revealed an intrahepatic pseudoaneurysm and an arteriovenous fistula between the hepatic arteries and inferior vena cava.

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Ischemic cholangitis: Lethal complication of Osler-Weber-Rendu disease.

Radiol Case Rep

September 2024

Digestive Disease Research Laboratory, Medical School, Mohammed First University, Faculty of Medicine and Pharmacy Oujda, Morocco.

Osler-Weber-Rendu disease (OWRD), also known as hereditary haemorrhagic telangiectasia (HHT), is an autosomal dominant genetic disorder characterised by arteriovenous malformations in several organs. Ischemic cholangitis is a rare life-threatening complication of OWRD, with only a few documented cases in the literature. A liver transplant is the main curative treatment.

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