Search results for " FIBROSIS"

BIOCOMPATIBLE POLYAMINOACID-BASED POLYCATIONS AS NON-VIRAL VECTORS FOR GENE THERAPY OF CYSTIC FIBROSIS.

2009

PREVALENCE OF LIVER STEATOSIS AND FIBROSIS AND RELIABILITY OF ULTRASOUND IN DETECTING NAFLD IN OBESE PATIENTS

2018

Background: Non-alcoholic fatty liver disease (NAFLD) is the most frequent chronic liver disease in Western countries, and in particular in obese patients the prevalence is higher than in general population. It has a large clinical spectrum, ranging from simple steatosis to steatohepatitis (NASH) and cirrhosis. Aim of the study: To evaluate in obese patients who underwent bariatric surgery in the last 3 years: 1) the histological prevalence and severity of liver steatosis (LS) and fibrosis; 2) the reliability of ultrasound (US) in diagnosing steatosis. Patients and methods: We reviewed preoperative liver US and intraoperative liver biopsy results in 57 bariatric surgery patients (age 42 ± 1…

Long-term hepatic safety of lomitapide in homozygous familial hypercholesterolaemia

2023

Introduction: Lomitapide is a microsomal triglyceride transfer protein inhibitor for patients with homozygous familial hypercholesterolaemia. Due to its mechanism of action, potential hepatic effects of lomitapide are of clinical interest. This study aimed to determine the long-term hepatic safety of lomitapide. Methods: Data were aggregated from the pivotal phase 3 and extension phase clinical trial with lomitapide (median 5.1 years; serum total bilirubin, transaminases, cytokeratin-18 [CK-18] and enhanced liver fibrosis [ELF] score, fat-soluble vitamins and essential fatty acids), 8-year data from the Lomitapide Observational Worldwide Evaluation Registry (LOWER) and real-world evidence f…

The severity of steatosis influences liver stiffness measurement in patients with nonalcoholic fatty liver disease

2015

In nonalcoholic fatty liver disease, the influence of severity of steatosis on liver stiffness measurement (LSM) is poorly studied and still debated. We assessed the impact of steatosis severity and its ultrasonographic (US) sign, severe bright liver echo pattern, on LSM values and on transient elastography accuracy for the diagnosis of liver fibrosis in a cohort of consecutive patients with nonalcoholic fatty liver disease. Patients (n = 253) were assessed by clinical, US, and histological (Kleiner score) features. Transient elastography was performed using the M probe. Among patients with low amounts of fibrosis (F0-F1 and F0-F2), median LSM values, expressed in kilopascals, were signific…

Visceral adiposity index is associated with significant fibrosis in patients with non-alcoholic fatty liver disease

2012

Background: Metabolic factors have been associated with liver damage in patients with non-alcoholic fatty liver disease (NAFLD). Aims To test a new marker of adipose dysfunction, the visceral adiposity index (VAI), in NAFLD patients to assess whether or not it is associated with host factors, and to investigate a potential correlation with histological findings. Methods One hundred and forty-two consecutive NAFLD patients were evaluated by liver biopsy, and clinical and metabolic measurements, including insulin resistance with the homeostasis model assessment (HOMA), and VAI by using waist circumference, body mass index, triglycerides and HDL. Serum levels of TNFα, IL-6, adiponectin and lep…

ADIPONECTIN, LEPTIN, RESISTIN LEVELS IN CYSTIC FIBROSIS ADOLESCENTS

2012

INTRODUCTION: Patients with Cystic Fibrosis, especially in adolescence, could develop endocrine and metabolic complications, related to nutritional state and chronic inflammation. They develop a progressive decrease in lean body mass correlated with the progression of lung disease. Adipose tissue is involved as well and adipocytokines are a possible link between malnutrition and long term complications. PATIENTS AND METHODS: In 24 Cystic Fibrosis adolescents we studied auxological, nutritional, glycometabolic, endocrine patterns, together with leptin, adiponectin and resistin levels. We selected patients not affected by diabetes, insulin resistance, malnutrition, acute inflammatory states s…

Efficacy of a 12-Week Simeprevir Plus Peginterferon/Ribavirin (PR) Regimen in Treatment-Naïve Patients with Hepatitis C Virus (HCV) Genotype 4 (GT4) …

2017

Background HCV GT4 accounts for up to 20% of HCV infections worldwide. Simeprevir, given for 12 weeks as part of a 24- or 48-week combination regimen with PR is approved for the treatment of chronic HCV GT4 infection. Primary study objectives were assessment of efficacy and safety of simeprevir plus PR in treatment-naïve patients with HCV GT4 treated for 12 weeks. Primary efficacy outcome was sustained virologic response 12 weeks post-treatment (SVR12). Additional objectives included investigation of potential associations of rapid virologic response and baseline factors with SVR12. Methods This multicentre, open-label, single-arm study (NCT01846832) evaluated efficacy and safety of simepre…

Physiotherapy adherence and perception in patients with cystic fibrosis: A descriptive study

2015

Background: Cystic fibrosis (CF) is a chronic and multisystem disease in which respiratory physiotherapy (RP) has revealed as one of the main pillars of treatment. Aim: This study was aimed at assessing the convergent validity and the internal consistency of a new instrument (CAP-FISIO questionnaire) to measure the adherence and the perception of RP in patients with CF. In addition, we inquired into the correlation of the CAP-FISIO questionnaire outcomes with degrees of self-efficacy and coping of the patients. Methods: This is a descriptive cross-sectional pilot study. Patients with CF were assessed in one temporal moment: 1) anthropometrics; 2) forced Spirometry; 3) adherence and percepti…

Genome-wide association analysis identifies six new loci associated with forced vital capacity

2014

Forced vital capacity (FVC), a spirometric measure of pulmonary function, reflects lung volume and is used to diagnose and monitor lung diseases. We performed genome-wide association study meta-analysis of FVC in 52,253 individuals from 26 studies and followed up the top associations in 32,917 additional individuals of European ancestry. We found six new regions associated at genome-wide significance (P <5 x 10(-8)) with FVC in or near EFEMP1, BMP6, MIR129-2-HSD17B12, PRDM11, WWOX and KCNJ2. Two loci previously associated with spirometric measures (GSTCD and PTCH1) were related to FVC. Newly implicated regions were followed up in samples from African-American, Korean, Chinese and Hispani…

3 He-MRI-based vs. conventional determination of lung volumes in patients after unilateral lung transplantation: a new approach to regional spirometry

2002

Background: To use 3Helium (3He)-MRI in patients with unilateral lung grafts to assess the contributions of graft and native lung to total ventilated lung volume, and second to compare conventional measurements of intrapulmonary gas volume (spirometry, body plethysmography) with image-based volumetry of ventilated lung parenchyma visualized by hyperpolarized 3He-MRI. Methods: With Ethics Committee approval, five patients with single lung transplantation (SLTX) for idiopathic pulmonary fibrosis (IPF) underwent both conventional pulmonary function testing (PFT) and 3He-MRI of the lung. Intrapulmonary gas volume (GV) during the inspiratory breathhold for 3He-MRI was calculated from measured fu…