PMID: 8590149Oct 1, 1995Paper

Hereditary haemorrhagic telangiectasia and secondary biliary cirrhosis

European Journal of Gastroenterology & Hepatology
A MendozaE Elias

Abstract

We report the case of a 65-year-old woman with a 10-year history of biliary disease and a 3-year history of anaemia, lethargy, angina and recurrent ascending cholangitis; she had hereditary haemorrhagic telangiectasia (HHT) with aneurysms in the splanchnic circulation, and was assumed to have secondary biliary cirrhosis with portal hypertension and hypertrophic obstructive cardiomyopathy. Her portal hypertension was caused by secondary biliary cirrhosis induced by intrahepatic gallstones; this was associated with left ventricular hypertrophy and a high cardiac output, caused by increased splanchnic circulation, and with aneurysms in the superior mesenteric and pancreaticoduodenal arteries, which were visualized by angiography. We conclude that HHT can produce abnormal abdominal vascular anatomy, which affects haemodynamic performance and makes it difficult to manage a patient with portal hypertension caused by secondary biliary cirrhosis. HHT has been shown to be associated with intrahepatic biliary stones and might have a causal role. When assessing patients with this type of condition for liver transplantation, particular attention should be paid to haemodynamic and circulatory abnormalities.

Citations

Apr 13, 2011·Hepatology International·Murat CagPhilippe Wolf
Jun 1, 2005·Digestive and Liver Disease : Official Journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver·E BuscariniA Zambelli
Mar 21, 1998·The American Journal of Surgical Pathology·R SaxenaS N Thung
Dec 28, 2010·Digestive and Liver Disease : Official Journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver·Marco SenzoloUNKNOWN Italian Association for the Study of the Liver (AISF) ad hoc
May 25, 2005·Liver International : Official Journal of the International Association for the Study of the Liver·I Bueres DominguezA Geubel
May 30, 2013·World Journal of Hepatology·Fabio Salvatore MacalusoDaniela Cabibi
Jun 17, 2006·Journal of Computer Assisted Tomography·Laurent MilotFrank Pilleul
Jan 24, 2007·Journal of Hepatology·Guadalupe Garcia-Tsao

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