Search results for "fibrosis"

Fibropolycystic Liver Disease: CT and MR Imaging Findings

2005

Fibropolycystic liver disease encompasses a spectrum of related lesions of the liver and biliary tract that are caused by abnormal embryologic development of the ductal plates. These lesions (congenital hepatic fibrosis, biliary hamartomas, autosomal dominant polycystic disease, Caroli disease, choledochal cysts) can be clinically silent or can cause signs and symptoms such as cholangitis, portal hypertension, gastrointestinal bleeding, infections, and space-occupying masses. The different types of fibropolycystic liver disease demonstrate characteristic findings at computed tomography (CT) and magnetic resonance (MR) imaging. Patients with congenital hepatic fibrosis typically have imaging…

Cerebrovascular involvement in fabry disease: current status of knowledge.

2014

Fabry disease (FD) is a rare and highly debilitating lysosomal storage disorder that results from a total lack of, or deficiency in, the enzyme α-galactosidase A (α-Gal A) because of mutations in the GLA gene.1 FD is inherited as an X-linked trait; many of the male patients develop a classic severe phenotype with early onset of symptoms, whereas heterozygous females exhibit phenotypes ranging from asymptomatic to major involvement of vital organs.2 Most families inherit private mutations; to date, >600 mutations have been identified and are listed in the online FD database (Fabry-database.org).3 The deficiency in α-Gal A causes the accumulation of globotriaosylceramide (GL-3; also abbreviat…

Distribution of factor XIIIa containing cells and collage nous components in radicular cysts: histochemical and immunohistochemical study

2011

Objective: 1) To differentiate various connective tissue layers in radicular cysts 2) To find the correlation between FXIIIa containing cells and fibrosis. Factor XIII, fibrin stabilizing factor, stabilizes the clot for blood coagulation and is also responsible for connective tissue organization in healing and tissue repair. FXIIIa has an important role during the process of fibrosis in various lesions, so we conducted a study to find its role in radicular cyst. Study Design: 10 cases of radicular cyst, reported in our hospital were selected. Clinical and follow up data of those cases were collected from the archives. Material and Methods: Histochemical analysis with Masson’s trichrome was …

Altered elemental profile as indicator of homeostatic imbalance in pathogenesis of oral submucous fibrosis

2002

Oral submucous fibrosis (OSF) is a potential precancerous condition of the oral cavity and oropharynx. The etiopathogenesis of this complex precancerous condition is still obscure. In addition to deleterious oral habits, malnutrition, and possible genetic predisposition, altered bioelemental status is also likely to play an important role in its pathogenesis. The present study analyzed 68 elements by inductively coupled plasma-mass spectroscopy in oral mucosa of normal and OSF individuals and some interesting alterations in elemental profile in the diseased tissue have been noted, indicating a homeostatic imbalance. These bioelemental alterations leading to homeostatic imbalance might be co…

Dupuytren's contracture: an update of biomolecular aspects and therapeutic perspectives.

2005

The so-called fibrogenic cytokines, able to induce the growth of fibroblasts and their differentiation into myofibroblasts and to stimulate their production of extracellular matrix, are involved in the genesis of Dupuytren’s contracture. Although many studies have been made of biomolecular aspects of palmar fibromatosis, practical applications from them are still far from imminent because of the real difficulty of blocking their action in vivo, even in a chronic, progressive lesion such as Dupuytren’s disease. Consequently, surgical excision of the palmar fascia still remains the treatment of choice.

Fibrospleen: Measuring spleen stiffness by transient elastography increases accuracy of staging of liver fibrosis and of portal hypertension in chron…

2009

Reliability of liver stiffness measurement in non-alcoholic fatty liver disease: the effects of body mass index

2011

Summary Background  Liver stiffness measurement (LSM) using transient elastography (TE) is used to stage fibrosis in patients with liver disease, diagnostic reliability and the factors affecting its performance in patients with non-alcoholic fatty liver disease (NAFLD) are incompletely understood. Aim  To assess LSM. Methods  Consecutive NAFLD patients (n = 169), assessed by liver biopsy (Kleiner score), anthropometrical, biochemical and metabolic features, underwent LSM using TE with standard M probe. Results  Liver stiffness measurement was not reliable in 23 patients (14%) due to obesity. Among patients with a reliable TE, a LSM value >7.25 kPa was the best cut-off for predicting signifi…

Th2-M2 immunity in lesions of muscular sarcoidosis and macrophagic myofasciitis

2015

Objective To analyse the paradox of a lack of giant cell formation and fibrosis in chronic lesions of macrophagic myofasciitis (MMF) in comparison with muscular sarcoidosis (MuS). Methods Inflammatory lesions and contiguous muscle regions from biopsy samples of 10 patients with MuS and 10 patients with MMF were cut out by laser microdissection. Mediators of the T helper cell (Th)1 inducing classical macrophage activation (e.g. STAT1, IFNγ and CXCR3), and Th2 inducing alternative activation of macrophages (e.g. CD206/MRC1, STAT6, SOCS1), molecules involved in development of fibrosis (e.g. TGFβ) and giant cells (e.g. TYROBP), were assessed by immunohistochemistry and real-time polymerase chai…

Fibroblast Growth Facor 19, a Downregulated Factor in Idiopathic Pulmonary Fibrosis, Inhibits Mice Lung Fibrosis

2020

Role of JAK2/STAT3 pathway in vascular function of pulmonary fibrosis patients

2015

Background: Idiopathic Pulmonary Fibrosis (IPF) is a chronic progressive lung disease with a life expectancy of 2-5 years. A proportion of IPF patients develop pulmonary hypertension (PH), characterized by vasoconstriction and remodeling of pulmonary arteries. Currently, no therapy can improve survival of patients diagnosed with this disease. JAK2/STAT3 molecular route is overexpressed in proliferative disorders, however, its role in PH- associated IPF is unknown. Objective: To analyze the role of JAK2/STAT3 in vascular function of IPF patients with PH. We hypothesized that inhibition of JAK2, STAT3 or JAK2/STAT3 may improve vascular function. Methods: Human precision cut lung slices and ar…