Search results for "fibrosis"

High performance of intravoxel incoherent motion diffusion MRI in detecting viral hepatitis-b induced liver fibrosis.

2019

Background: Recently a small cohort study demonstrated that intravoxel incoherent motion (IVIM) diffusion MRI can detect early stage liver fibrosis. Using modified IVIM data acquisition parameters, the current study aims to confirm this finding. Methods: Twenty-six healthy volunteers, three patients of chronic viral hepatitis-b but without fibrosis and one mild liver steatosis subject, and 12 viral hepatitis-b patients with fibrosis (stage 1–2=7, stage 3–4=5) were included in this study. With a 1.5-T MR scanner and respiration-gating, IVIM diffusion imaging was acquired using a single-shot echo-planar sequence with a b -value series of 2, 0, 1, 15, 20, 30, 45, 50, 60, 80, 100, 200, 300, 600…

Wild-type JAK2 secondary acute erythroleukemia developing after JAK2-V617F-mutated primary myelofibrosis.

2009

A 54-year-old female patient developed acute erythroleukemia after an 8-year course of primary myelofibrosis. The latter harbors the JAK2-V617F mutation and was treated with hydroxyurea and anagrelide. A bone marrow trephine biopsy disclosed 2 morphologically distinct areas of chronic primary myelofibrosis and acute erythroleukemia. Microdissection and a separate molecular pathological analysis was performed. Although the activating JAK2-V617F mutation was not maintained in blasts of acute erythroleukemia, it was detectable in the chronic phase of primary myelofibrosis, indicating that this mutation did not play a role in the leukemic transformation of erythroid cells.

Fibrosis Quística: comparativa de tres cuestionarios de calidad de vida

2015

Comprender cuáles son los determinantes de la calidad de vida relacionada con la salud (CVRS) en fibrosis quística (FQ) podría ayudar al desarrollo de intervenciones para mejorarla. Cómo refiera una persona cuál es su CVRS puede estar influido por muchas variables: demográficas, clínicas, psicológicas y sociales. Algunos de estos factores probablemente estén interrelacionados, pero es útil tenerlos en cuenta cuando se diseña, analiza e interpretan estudios que midan la CVRS, ya que pueden influir sobre los hallazgos. La medición de la CVRS en FQ permite valorar la enfermedad desde la perspectiva del paciente, aportando información valiosa tanto para la clínica como para la investigación. As…

Noninvasive assessment of liver fibrosis in thalassaemia major patients by transient elastography (TE) - lack of interference by iron deposition.

2009

The correlation between liver stiffness, measured by transient elastography, liver fibrosis, using the histological METAVIR score, and iron overload, measured by atomic absorption spectrometry was evaluated in 56 homozygous-b-thalassaemics. Liver stiffness increased proportionally to liver fibrosis staging (r = 0Æ70; P > 0Æ001) independently of liver iron concentration (r = 0Æ01; P = 0Æ932). The area under the receiver-operating characteristic curve for prediction of cirrhosis was 0Æ997 (95% confidence interval [CI]: 0Æ925–1Æ000) with cut-off of 13 kPa with 100% sensitivity (95% CI: 69Æ0–100Æ0) and 95% specificity (95% CI: 84Æ2–99Æ3). Transient elastography is a reliable non-invasive tool f…

Endotrophin, a pro-peptide of Type VI collagen, is a biomarker of survival in cirrhotic patients with hepatocellular carcinoma

2021

Aim: Type VI collagen, is emerging as a signaling collagen originating from different types of fibroblasts. A specific fragment of Type VI collagen, the pro-peptide, is also known as the hormone endotrophin. We hypothesized that this fibroblast hormone would be of particular relevance in cancer types with a high amount of fibrosis activity, namely for outcome in hepatocellular carcinoma (HCC) cirrhotic patients. Patients & methods: Plasma C6M, PRO-C6 and alphafeto-protein (AFP) were assessed in 309 patients with mixed etiologies (hepatitis C, hepatitis B, alcohol and nonalcoholic fatty liver) diagnosed as cirrhotics, cirrhotics with HCC, noncirrhotics and healthy controls. Progression-f…

In rat fibrotic colon TGF-beta/SMAD signalling is modulated by cyclooxygenases inihibitors

2013

Current development of CFTR potentiators in the last decade

2020

Cystic fibrosis (CF) is a genetic disorder produced by the loss of function of CFTR, a main chloride channel involved in transepithelial salt and water transport. CFTR function can be rescued by small molecules called "potentiators" which increase gating activity of CFTR on epithelial surfaces. High throughput screening (HTS) assays allowed the identification of new chemical entities endowed with potentiator properties, further improved through medicinal chemistry optimization. In this review, the most relevant classes of CFTR potentiators developed in the last decade were explored, focusing on structure-activity relationships (SAR) of the different chemical entities, as a useful tool for t…

Investigating REPAIRv2 as a Tool to Edit CFTR mRNA with Premature Stop Codons

2020

Cystic fibrosis (CF) is caused by mutations in the gene encoding the transmembrane conductance regulator (CFTR) protein. Some CF patients are compound heterozygous or homozygous for nonsense mutations in the CFTR gene. This implies the presence in the transcript of premature termination codons (PTCs) responsible for a truncated CFTR protein and a more severe form of the disease. Aminoglycoside and PTC124 derivatives have been used for the read-through of PTCs to restore the full-length CFTR protein. However, in a precision medicine framework, the CRISPR/dCas13b-based molecular tool &ldquo

Cyclooxygenase inhibitors counteract pro-fibrotic signalling in experimental colitis through modulation of TGF-beta/SMAD network

2013

Aims. Cyclooxygenase isoforms (COX-1, COX-2) have been implicated in the development of fibrosis at gastrointestinal sites. Under bowel inflammation, transforming growth factor beta (TGF-beta) has been identified as the main regulator of fibrotic remodelling. The present study investigated the effects of cyclooxygenase inhibitors on pro-fibrotic signalling mediated by the TGF-beta/SMAD pathway in experimental colitis. Methods. Colitis was induced in rats by intrarectal 2,4-dinitrobenzenesulfonic acid (DNBS, 30 mg/rat in 0.25 ml ethanol 50%). After 6 days, systemic [body and spleen weight] and tissue inflammatory parameters [macroscopic and microscopic damage] were assessed. Three days befor…

Estudio del perfil farmacológico de inhibidores de PDE4 en modelos de fibrosis cutánea

2021

La fibrosis es un mecanismo fisiológico que se define como el aumento de la proliferación de fibroblastos y deposición de matriz extracelular (MEC), que, cuando adquiere un carácter patológico, puede destruir la arquitectura normal y producir la disfunción del órgano. La fibrosis cutánea ocurre en una variedad de enfermedades tales como la esclerosis sistémica (ES) o esclerodermia, queloides, cicatrices hipertróficas y otras afecciones. Se considera que es el resultado de una reparación anormal en respuesta al daño tisular. Roflumilast, un inhibidor de la fosfodiesterasa (PDE) 4 aprobado para el tratamiento de la enfermedad pulmonar obstructiva crónica (EPOC), ha demostrado tener efectos an…