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Abernethy malformation: beware in cases of unexplained hepatic encephalopathy in adults-case report and review of the relevant literature
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Orientador(es)
Resumo(s)
The Abernethy malformation consists of a congenital extrahepatic portosystemic shunt and is believed to be extremely rare in humans. The potential implications of abnormal portovenous shunting and decreased hepatic portal flow are numerous and potentially serious. Although congenital extrahepatic portosystemic shunts are increasingly suspected and diagnosed in specialized centres, much of their clinical presentation and natural history is not fully understood. Symptoms of portosystemic shunt are mainly caused by increased levels of ammonia, which lead to signs of encephalopathy. Therapeutic options depend on the type of shunt and its clinical course, so the classification of the congenital portosystemic shunt is a key finding in these patients.
Descrição
Palavras-chave
Encefalopatia Hepática Veia Porta/anomalias congénitas
Contexto Educativo
Citação
BJR Case Rep. 2017 Nov 16;4(2):20170054.