Pathophysiology of biochemical signs of primary biliary cholangitis
Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is a rare chronic autoimmune cholestatic liver disease, affecting mostly females. With PBС develops chronic cholangiopathy, this is accompanied by the development of gradually progressive liver fibrosis, which leads to...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
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Open Exploration Publishing Inc.
2023-08-01
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| Series: | Exploration of Digestive Diseases |
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| Online Access: | https://www.explorationpub.com/Journals/edd/Article/100524 |
| _version_ | 1797691638341959680 |
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| author | Vasiliy Ivanovich Reshetnyak Igor Veniaminovich Maev |
| author_facet | Vasiliy Ivanovich Reshetnyak Igor Veniaminovich Maev |
| author_sort | Vasiliy Ivanovich Reshetnyak |
| collection | DOAJ |
| description | Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is a rare chronic autoimmune cholestatic liver disease, affecting mostly females. With PBС develops chronic cholangiopathy, this is accompanied by the development of gradually progressive liver fibrosis, which leads to intrahepatic cholestasis. Defects in autoimmune tolerance are critical factors in the emergence of the disease. Biochemical signs in PBС appear already in the asymptomatic stage of the disease and they are associated with a disturbance of the secretion of bile acids. Understanding the pathophysiological mechanisms of these signs is essential to both the early diagnosis and treatment of PBC. Early diagnosis of the disease contributes to its more effective treatment. There are many scientifically based modern data on the pathophysiology of clinical and laboratory signs developing in PBС. The purpose of this review is to summarize the data available in the literature and those obtained by the authors on the mechanisms for the development of biochemical criteria for PBC and their diagnostic significance. The opportunity to present the pathophysiological mechanisms of the development of biochemical signs in patients with PBC is associated with the success in the development of modern research methods in biochemistry, molecular biology, and genetics. |
| first_indexed | 2024-03-12T02:17:04Z |
| format | Article |
| id | doaj.art-69f722bdfe834fec9972c5401179e49a |
| institution | Directory Open Access Journal |
| issn | 2833-6321 |
| language | English |
| last_indexed | 2024-03-12T02:17:04Z |
| publishDate | 2023-08-01 |
| publisher | Open Exploration Publishing Inc. |
| record_format | Article |
| series | Exploration of Digestive Diseases |
| spelling | doaj.art-69f722bdfe834fec9972c5401179e49a2023-09-06T07:41:31ZengOpen Exploration Publishing Inc.Exploration of Digestive Diseases2833-63212023-08-012414917110.37349/edd.2023.00024Pathophysiology of biochemical signs of primary biliary cholangitisVasiliy Ivanovich Reshetnyak0https://orcid.org/0000-0003-3614-5052Igor Veniaminovich Maev1https://orcid.org/0000-0001-6114-564XDepartment of Propaedeutics of Internal Diseases and Gastroenterology, A.I. Yevdokimov Moscow State University of Medicine and Dentistry, 127473 Moscow, Russian FederationDepartment of Propaedeutics of Internal Diseases and Gastroenterology, A.I. Yevdokimov Moscow State University of Medicine and Dentistry, 127473 Moscow, Russian FederationPrimary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is a rare chronic autoimmune cholestatic liver disease, affecting mostly females. With PBС develops chronic cholangiopathy, this is accompanied by the development of gradually progressive liver fibrosis, which leads to intrahepatic cholestasis. Defects in autoimmune tolerance are critical factors in the emergence of the disease. Biochemical signs in PBС appear already in the asymptomatic stage of the disease and they are associated with a disturbance of the secretion of bile acids. Understanding the pathophysiological mechanisms of these signs is essential to both the early diagnosis and treatment of PBC. Early diagnosis of the disease contributes to its more effective treatment. There are many scientifically based modern data on the pathophysiology of clinical and laboratory signs developing in PBС. The purpose of this review is to summarize the data available in the literature and those obtained by the authors on the mechanisms for the development of biochemical criteria for PBC and their diagnostic significance. The opportunity to present the pathophysiological mechanisms of the development of biochemical signs in patients with PBC is associated with the success in the development of modern research methods in biochemistry, molecular biology, and genetics.https://www.explorationpub.com/Journals/edd/Article/100524primary biliary cholangitis (pbc)cholestasisbiochemical signs of pbcalkaline phosphatase (ap)γ-glutamyl transferase (γ-gt)5’-nucleotidasedisorders of lipid metabolism |
| spellingShingle | Vasiliy Ivanovich Reshetnyak Igor Veniaminovich Maev Pathophysiology of biochemical signs of primary biliary cholangitis Exploration of Digestive Diseases primary biliary cholangitis (pbc) cholestasis biochemical signs of pbc alkaline phosphatase (ap) γ-glutamyl transferase (γ-gt) 5’-nucleotidase disorders of lipid metabolism |
| title | Pathophysiology of biochemical signs of primary biliary cholangitis |
| title_full | Pathophysiology of biochemical signs of primary biliary cholangitis |
| title_fullStr | Pathophysiology of biochemical signs of primary biliary cholangitis |
| title_full_unstemmed | Pathophysiology of biochemical signs of primary biliary cholangitis |
| title_short | Pathophysiology of biochemical signs of primary biliary cholangitis |
| title_sort | pathophysiology of biochemical signs of primary biliary cholangitis |
| topic | primary biliary cholangitis (pbc) cholestasis biochemical signs of pbc alkaline phosphatase (ap) γ-glutamyl transferase (γ-gt) 5’-nucleotidase disorders of lipid metabolism |
| url | https://www.explorationpub.com/Journals/edd/Article/100524 |
| work_keys_str_mv | AT vasiliyivanovichreshetnyak pathophysiologyofbiochemicalsignsofprimarybiliarycholangitis AT igorveniaminovichmaev pathophysiologyofbiochemicalsignsofprimarybiliarycholangitis |