scholarly journals Primary biliary cholangitis

Author(s):  
I. M. Iljinsky ◽  
O. M. Tsirulnikova

Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is an organ-specific autoimmune disease predominantly affecting middle-aged women. It does not occur in children. PBC prevalence varies depending on the geographic location of the country. Over the past 30 years, there has been an increased incidence of PBC, while significant progress has been made in understanding the pathogenesis of PBC due to the development of innovative technologies in molecular biology, immunology and genetics. The presence of antimitochondrial antibodies and cholestasis on biochemical analysis is sufficient to make a diagnosis, without the need for liver biopsy. Small- and medium-sized bile ducts are the targets of PBC. In the first stage of the disease, granulomatous destruction of the bile ducts occurs; in the second stage, loss of bile ducts, their proliferation, increased size of the portal tracts with chronic inflammation; in the third stage - fibrosis with septal formation, loss of bile ducts and cholestasis; in the fourth stage - liver cirrhosis. Previously, the survival rate of PBC patients ranged from 7.5 to 16 years. However, it has improved significantly with ursodeoxycholic acid and obeticholic acid treatment. If there is no effect from treatment and end-stage liver failure sets in, liver transplantation is performed.

1998 ◽  
Vol 107 (9) ◽  
pp. 745-752 ◽  
Author(s):  
Paul J. Donald

Many surgical procedures have been devised to manage laryngotracheal stenosis secondary to trauma. Laryngotracheal atresia is the most severe form and the most difficult to repair. The Meyer procedure is a three-stage operation that provides structural support that is covered with mucosa. A laryngotracheal trough is created and a carved trough-shaped cartilage graft is placed above and lateral to it in the first stage. The skin over the graft is replaced by buccal mucosa in the second stage. In the last stage, the cartilage graft with overlying mucosa is swung onto the trough as a composite flap replacing the anterior and lateral laryngeal and tracheal walls. Attempt at reconstruction was made in 8 patients. All but one lesion was secondary to endotracheal intubation. Two patients were unable to be taken to completion of the third stage. Of the remaining 6 patients, all have a functional voice and only 1 remains cannulated at night.


2020 ◽  
Vol 51 (Special) ◽  
Author(s):  
Naji & Taha

The current research was aimed to evaluation of suitability  the transfer of smart agriculture in Iraqi agricultural systems so. identifying weaknesses and reasons Identifying proposals for improvement. The input evaluation process consisted of four stages: First Stage: Determination of the set of criteria which the dissemination of the smart agriculture method should be made in the light.. Second Stage: Presentation of evaluation suitable criteria the transfer of the smart agriculture method in the agricultural systems of Iraq in its preliminary form to a group of experts . to express their opinion in the field and items of the proposed criteria and the required amendments for the purpose of finalizing those criteria. Third stage: Surveying of within the work area . Fourth stage: Compare between criteria and surveying , and Judgment .The research concluded that most evaluation suitable criteria are compatible with the transfer of smart agriculture in Iraqi agricultural systems as being partially applied.


Gut ◽  
2018 ◽  
Vol 67 (9) ◽  
pp. 1568-1594 ◽  
Author(s):  
Gideon M Hirschfield ◽  
Jessica K Dyson ◽  
Graeme J M Alexander ◽  
Michael H Chapman ◽  
Jane Collier ◽  
...  

Primary biliary cholangitis (formerly known as primary biliary cirrhosis, PBC) is an autoimmune liver disease in which a cycle of immune mediated biliary epithelial cell injury, cholestasis and progressive fibrosis can culminate over time in an end-stage biliary cirrhosis. Both genetic and environmental influences are presumed relevant to disease initiation. PBC is most prevalent in women and those over the age of 50, but a spectrum of disease is recognised in adult patients globally; male sex, younger age at onset (<45) and advanced disease at presentation are baseline predictors of poorer outcome. As the disease is increasingly diagnosed through the combination of cholestatic serum liver tests and the presence of antimitochondrial antibodies, most presenting patients are not cirrhotic and the term cholangitis is more accurate. Disease course is frequently accompanied by symptoms that can be burdensome for patients, and management of patients with PBC must address, in a life-long manner, both disease progression and symptom burden. Licensed therapies include ursodeoxycholic acid (UDCA) and obeticholic acid (OCA), alongside experimental new and re-purposed agents. Disease management focuses on initiation of UDCA for all patients and risk stratification based on baseline and on-treatment factors, including in particular the response to treatment. Those intolerant of treatment with UDCA or those with high-risk disease as evidenced by UDCA treatment failure (frequently reflected in trial and clinical practice as an alkaline phosphatase >1.67 × upper limit of normal and/or elevated bilirubin) should be considered for second-line therapy, of which OCA is the only currently licensed National Institute for Health and Care Excellence recommended agent. Follow-up of patients is life-long and must address treatment of the disease and management of associated symptoms.


2014 ◽  
Vol 25 (25) ◽  
pp. 155-165 ◽  
Author(s):  
Ioannis A. Pissourios

Abstract The article aims at exploring the literature on town centre delimitation methods over the last sixty years. Specifically, the first section explores the content of the term town centre, while the second one proposes an organisation of town centre delimitation research in three main periods. The third section comprises the main corpus of the article, as several town centre delimitation methodologies of the last sixty years are presented and discussed. For their presentation, a three tiered analysis is introduced. In the first stage the decisions of researchers regarding centrality estimators are discussed, in the second stage the study focuses on the choices of the spatial units, in which the chosen variables will be studied, and in the third stage the study discusses the methods that are used to characterise each of the studied spatial units as central versus non-central. Based on the analysis, the article concludes that town centre delimitation is an issue which transcends various scientific disciplines and that each of these disciplines comprehends the centre of a town in a unique way. Thus, future methods of town centre delimitation should take into account the choices made in each of the three stages presented above, and should also link the above choices to the objectives and the theoretical context of the study.


2018 ◽  
Vol 11 ◽  
pp. 175628481878740 ◽  
Author(s):  
Priscila Santiago ◽  
Andrew R. Scheinberg ◽  
Cynthia Levy

Cholestatic liver diseases result from gradual destruction of bile ducts, accumulation of bile acids and self-perpetuation of the inflammatory process leading to damage to cholangiocytes and hepatocytes. If left untreated, cholestasis will lead to fibrosis, biliary cirrhosis, and ultimately end-stage liver disease. Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are the two most common chronic cholestatic liver diseases affecting adults, and their etiologies remain puzzling. While treatment with ursodeoxycholic acid (UDCA) has significantly improved outcomes and prolonged transplant-free survival for patients with PBC, treatment options for UDCA nonresponders remain limited. Furthermore, there is no available medical therapy for PSC. With recent advances in molecular biochemistry specifically related to bile acid regulation and understanding of immunologic pathways, novel pharmacologic treatments have emerged. In this review, we discuss the standard of care and emphasize the various emerging treatments for PBC and PSC.


2015 ◽  
Vol 5 (3) ◽  
pp. 185-200
Author(s):  
Robert Z. Birdwell

Critics have argued that Elizabeth Gaskell's first novel, Mary Barton (1848), is split by a conflict between the modes of realism and romance. But the conflict does not render the novel incoherent, because Gaskell surpasses both modes through a utopian narrative that breaks with the conflict of form and gives coherence to the whole novel. Gaskell not only depicts what Thomas Carlyle called the ‘Condition of England’ in her work but also develops, through three stages, the utopia that will redeem this condition. The first stage is romantic nostalgia, a backward glance at Eden from the countryside surrounding Manchester. The second stage occurs in Manchester, as Gaskell mixes romance with a realistic mode, tracing a utopian drive toward death. The third stage is the utopian break with romantic and realistic accounts of the Condition of England and with the inadequate preceding conceptions of utopia. This third stage transforms narrative modes and figures a new mode of production.


2019 ◽  
Author(s):  
Lucy Armstrong ◽  
Lorna Hogg ◽  
Pamela Charlotte Jacobsen

The first stage of this project aims to identify assessment measures which include items on voice-hearing by way of a systematic review. The second stage is the development of a brief framework of categories of positive experiences of voice hearing, using a triangulated approach, drawing on views from both professionals and people with lived experience. The third stage will involve using the framework to identify any positve aspects of voice-hearing included in the voice hearing assessments identified in stage 1.


2021 ◽  
pp. 1-24
Author(s):  
Philipp Klar ◽  
Georg Northoff

The existential crisis of nihilism in schizophrenia has been reported since the early days of psychiatry. Taking first-person accounts concerning nihilistic experiences of both the self and the world as vantage point, we aim to develop a dynamic existential model of the pathological development of existential nihilism. Since the phenomenology of such a crisis is intrinsically subjective, we especially take the immediate and pre-reflective first-person perspective’s (FPP) experience (instead of objectified symptoms and diagnoses) of schizophrenia into consideration. The hereby developed existential model consists of 3 conceptualized stages that are nested into each other, which defines what we mean by existential. At the same time, the model intrinsically converges with the phenomenological concept of the self-world structure notable inside our existential framework. Regarding the 3 individual stages, we suggest that the onset or first stage of nihilistic pathogenesis is reflected by phenomenological solipsism, that is, a general disruption of the FPP experience. Paradigmatically, this initial disruption contains the well-known crisis of common sense in schizophrenia. The following second stage of epistemological solipsism negatively affects all possible perspectives of experience, that is, the first-, second-, and third-person perspectives of subjectivity. Therefore, within the second stage, solipsism expands from a disruption of immediate and pre-reflective experience (first stage) to a disruption of reflective experience and principal knowledge (second stage), as mirrored in abnormal epistemological limitations of principal knowledge. Finally, the experience of the annihilation of healthy self-consciousness into the ultimate collapse of the individual’s existence defines the third stage. The schizophrenic individual consequently loses her/his vital experience since the intentional structure of consciousness including any sense of reality breaks down. Such a descriptive-interpretative existential model of nihilism in schizophrenia may ultimately serve as input for future psychopathological investigations of nihilism in general, including, for instance, its manifestation in depression.


2021 ◽  
Vol 22 (15) ◽  
pp. 8139
Author(s):  
Tomoko Tadokoro ◽  
Asahiro Morishita ◽  
Tsutomu Masaki

Remarkable progress has been made in the treatment and control of hepatitis B and C viral infections. However, fundamental treatments for diseases in which liver fibrosis is a key factor, such as cirrhosis, alcoholic/nonalcoholic steatohepatitis, autoimmune hepatitis, primary biliary cholangitis, and primary sclerosing cholangitis, are still under development and remain an unmet medical need. To solve this problem, it is essential to elucidate the pathogenesis of liver fibrosis in detail from a molecular and cellular perspective and to develop targeted therapeutic agents based on this information. Recently, microRNAs (miRNAs), functional RNAs of 22 nucleotides, have been shown to be involved in the pathogenesis of liver fibrosis. In addition, extracellular vesicles called “exosomes” have been attracting attention, and research is being conducted to establish noninvasive and extremely sensitive biomarkers using miRNAs in exosomes. In this review, we summarize miRNAs directly involved in liver fibrosis, miRNAs associated with diseases leading to liver fibrosis, and miRNAs related to complications of cirrhosis. We will also discuss the efficacy of each miRNA as a biomarker of liver fibrosis and pathology, and its potential application as a therapeutic agent.


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