Correlation between neuroimaging, neurological phenotype, and functional outcomes in Wilson's disease.

Introduction: Wilson's disease (WD) is associated with a variety of movement disorders and progressive neurological dysfunction. The aim of this study was to correlate baseline brain magnetic resonance imaging (MRI) features with clinical phenotype and long-term outcomes in chronically treated WD patients.

Methods: Patients were retrospectively selected from an institutional database.

Brain MRI in the Decision for Liver Transplantation in Pediatric Neurological Wilson's Disease.

Background: Neurological Wilson's disease (WD) presentation in the pediatric population is rare, and liver transplantation (LT) in these patients remains controversial. The aim of the present study was to assess the role of brain magnetic resonance imaging (MRI) in predicting reversion of brain lesions and neurological outcomes in pediatric WD patients after LT.

Methods: Patients with confirmed WD (Leipzig score ≥4), disease onset in pediatric age (<18 years), neurological involvement, and submitted to LT were selected.

Budd-Chiari Syndrome and Acute Liver Failure: An Uncommon Presentation of Acute Myeloid Leukaemia.

Acute liver failure (ALF) is a rare entity, particularly in the context of Budd-Chiari syndrome (BCS). BCS is an uncommon disorder with multiple risk factors, most commonly myeloproliferative disorders. In BCS, active search and exclusion of underlying malignancy is mandatory, particularly in the context of ALF, as it may contraindicate liver transplantation (LT).

Systemic inflammation as a risk factor for portal vein thrombosis in cirrhosis: a prospective longitudinal study.

Background And Aims: Various risk factors for portal vein thrombosis (PVT) development in patients with cirrhosis have been identified, but the role of systemic inflammatory reaction is unknown. The study aims to assess the association between markers of systemic inflammation and PVT in cirrhosis.

Methods: Between January 2014 and October 2015, 107 outpatients with cirrhosis and no PVT were recruited, and followed till February 2017.

Nonselective beta-blockers and the risk of portal vein thrombosis in patients with cirrhosis: results of a prospective longitudinal study.

Background: Nonmalignant portal vein thrombosis is a significant event in the course of cirrhosis that can contraindicate liver transplantation and even impact survival after the surgical procedure. Risk factors are not completely known or validated and are still debated.

Aim: To identify in patients with cirrhosis the risk factors for portal vein thrombosis that are assessable in clinical practice.

Evaluation of fibrosis and inflammation in diffuse liver diseases using intravoxel incoherent motion diffusion-weighted MR imaging.

Purpose: The purpose of the study was to evaluate the role of intravoxel incoherent motion (IVIM) diffusion model for the assessment of liver fibrosis and inflammation in diffuse liver disorders, also considering the presence of liver steatosis and iron deposits.

Methods: Seventy-four patients were included, with liver biopsy and a 3 Tesla abdominal magnetic resonance imaging examination, with an IVIM diffusion-weighted sequence (single-shot spin-echo echo-planar sequence, with gradient reversal fat suppression; 6 b-values: 0, 50, 200, 400, 600, and 800 s/mm). Histological evaluation comprised the Ishak modified scale, for grading inflammation and fibrosis, plus steatosis and iron loading classification.

Wilson's Disease and Ulcerative Colitis in the Same Patient: Just A Coincidence? A Case Report and Literature Review.

Ulcerative Colitis (UC) is a chronic relapsing inflammatory bowel disease (IBD). Wilson's disease (WD) is a disorder of copper (Cu) metabolism due to inherited mutations in a gene encoding a putative Cu-transporting P-type ATPase, with a heterogeneous clinical presentation that includes hepatic, neurological, or psychiatric symptoms. The case of a 17-year-old female that presented with severe liver failure, three years after UC onset, and in which diagnosis of WD was established is reported.

Sorafenib Improves Survival in Metastatic Hepatocellular Carcinoma: A Case Report.

Hepatocellular carcinoma (HCC) is a very common cancer. Curative treatments and local ones are well validated. Sorafenib, a multi-kinase receptor inhibitor was introduced in 2007 for advanced HCC in patients with preserved liver function.

Latent schistosomiasis in Portuguese soldiers.

Schistosomiasis was diagnosed in two Portuguese soldiers who had been deployed to Portuguese colonies in Africa. The first veteran was diagnosed as having schistosomiasis 34 years after returning from Angola, and the second veteran was found with Schistosoma haematobium infection 40 years after returning from Mozambique. The patient with Schistosoma mansoni had an active infection, because eggs were recovered with living miracidia.

Growth hormone (GH)-induced reconstitution of CD8+ CD28+ T lymphocytes in a rare case of severe lymphopenia associated with Juvenile Haemochromatosis and Turner's syndrome.

This paper describes a rare case of Turner's syndrome associated with Juvenile Haemochromatosis and severe lymphopenia, followed-up for a period of 5 years. Because of the indication for treatment with growth hormone (GH), this case was observed as a model to analyse the effects of GH on growth, iron mobilization and lymphocyte reconstitution. For this purpose, a serial study of the T lymphocyte subpopulations CD4+, CD8+, CD8+ CD28+ and CD8+ CD28- was performed by immunophenotyping during the follow-up period.