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Practical and Multidisciplinary Review on Wilson Disease: The Portuguese Perspe...

Calinas, Filipe; Cardoso, Hélder; Carvalhana, Sofia; Ferreira, José; Gonçalves, Cristina; Magalhães, Marina; Miranda, Helena Pessegueiro; Presa, José

Wilson disease (WD) is a genetic disorder of copper metabolism caused by mutations in the ATP7B gene resulting in toxic copper accumulation in several organs. WD can manifest as liver disease, a progressive neurological disorder, a psychiatric illness, or a combination of these. Other clinical manifestations can also occur. Diagnosis is challenging and typically requires a range of biochemical tests, imaging, g...


Prediction of Hepatocellular Carcinoma in a Portuguese Population after Hepatit...

Mascarenhas, André; Serrazina, Juliana; Bronze, Sérgio; Cortez-Pinto, Helena; Presa, José; Barreira, Ana; Carrola, Paulo; Vara-Luiz, Francisco

Introduction: Chronic infection with hepatitis C virus (HCV) causes 25% of hepatocellular carcinoma (HCC) cases worldwide, a major cause of morbimortality even after sustained virologic response (SVR). Universal screening to all patients with advanced liver fibrosis is currently recommended. A risk-based strategy could improve the detection rate of early HCC and diminish the surveillance burden. Although severa...


Predictors for incomplete response to ursodeoxycholic acid in primary biliary c...

Cortez-Pinto, Helena; Liberal, Rodrigo; Lopes, Susana; Machado, Mariana; Carvalho, Joana; Dias, Teresa; Santos, Arsénio; Agostinho, Cláudia

Background: The current standard of treatment in primary biliary cholangitis (PBC) is ursodeoxycholic acid (UDCA), although a considerable proportion of patients show incomplete response resulting in disease progression. Objective: This study aimed to assess the prevalence of incomplete response to UDCA and determine associated patients' characteristics. Methods: Patients with PBC as main diagnosis were include...


Transient Hepatic Elastography and Detection of Clinically Significant Esophage...

Catarino, Ana; Pais, Carmen; Silva, Renata; Silva, Nuno; Carrola, Paulo; Presa, José

Introduction: Esophageal varices (EV) are a complicationof portal hypertension, and its rupture is associated witha high mortality rate. The use of non-invasive diagnosticmethods, including hepatic elastography, have been underinvestigation.Aim: Evaluate the usefulness of transient hepatic elastographyas a non-invasive test for the prediction of presenceof EV, to avoid systematic screening with esophagogastrodu...


Bleeding oesophageal varices

Presa, José; Próspero, Fernando

Upper gastrointestinal bleeding, is serious complication of portal hypertension, associated with high mortality. Several factors are associated with prognosis and treatment, such as hepatocelular function expressed by Child-Pugh classification. The authors review several aspects of phisiopathology and farmacology. Also review different surgical aproach to bleeding patient, including livrer transplatation.; .


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