Biliary Atresia: Aetiology, Diagnosis and Treatment
Despite extensive clinical and basic research, we know very little about the aetiology and pathomechanism of biliary atresia (BA). Today, it is a matter of common sense that early diagnosis (screening programs), sequential surgical treatment (the Kasai procedure and optional liver transplantation),...
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MDPI - Multidisciplinary Digital Publishing Institute
2024
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| Առցանց հասանելիություն: | ONIX_20240704_9783725812172_96 |
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Չկան պիտակներ, Եղեք առաջինը, ով նշում է այս գրառումը!
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| description | Despite extensive clinical and basic research, we know very little about the aetiology and pathomechanism of biliary atresia (BA). Today, it is a matter of common sense that early diagnosis (screening programs), sequential surgical treatment (the Kasai procedure and optional liver transplantation), and thorough follow-up in paediatric liver units improve survival rates among BA patients with native livers. However, BA still remains the most frequent indication of paediatric liver transplantation in patients with an unfavourable outcome after the Kasai procedure because the survival with native liver drops below 30% over the long term. Therefore, the following aspects should be considered alongside individual health issues: first, early organ replacement is a multifactorial burden for the growing organism, and second, every patient who survives with their native liver relieves the limited availability of donor organs and benefits national health expenditures with economic savings. From that point of view, international and interdisciplinary strategies are mandatory, in as much as BA is a rare disease. Over the past several years, national and international initiatives have been established in order to improve screening, centralization, diagnosis, as well as surgical and adjuvant treatment protocols. One such initiative is the recently launched European rare diseases network “rare-liver”. |
| format | Online |
| id | doab-20.500.12854ir-139300 |
| institution | Directory of Open Access Books |
| language | eng |
| publishDate | 2024 |
| publishDateRange | 2024 |
| publishDateSort | 2024 |
| publisher | MDPI - Multidisciplinary Digital Publishing Institute |
| publisherStr | MDPI - Multidisciplinary Digital Publishing Institute |
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| spelling | doab-20.500.12854ir-1393002024-07-04T09:39:22Z Biliary Atresia: Aetiology, Diagnosis and Treatment Petersen, Claus biomarker cholangiocyte ductular reaction liver fibrosis pediatric liver disease pregnancy biliary atresia chorine esterase MELD score liver transplantation Kasai operation liver transplant etiology adjuvant therapy budesonide cholangitis Kasai hepatoportoenterostomy cytomegalovirus survival native liver antiviral treatment newborn screening public health epidemiology liver nodules hepatocellular carcinoma regenerative nodules focal nodular hyperplasia Kasai portoenterostomy transplant waiting list pediatric liver transplantation referral practice outcome kasai portoenterostomy primary liver transplantation outcomes perinatal liver inflammation innate immune system monocytes cholangiopathy liver histology transplant-free survival corticosteroids ursodeoxycholic acid cytotoxic T cells perinatal liver injury hematopoietic stem and progenitor cells myeloid progenitor cells liver transition adolescence adherence readiness professionals parents pediatric liver surgery hepatoblastoma pediatric surgery workforce subspecialization thema EDItEUR::M Medicine and Nursing thema EDItEUR::M Medicine and Nursing::MJ Clinical and internal medicine::MJH Gastroenterology Despite extensive clinical and basic research, we know very little about the aetiology and pathomechanism of biliary atresia (BA). Today, it is a matter of common sense that early diagnosis (screening programs), sequential surgical treatment (the Kasai procedure and optional liver transplantation), and thorough follow-up in paediatric liver units improve survival rates among BA patients with native livers. However, BA still remains the most frequent indication of paediatric liver transplantation in patients with an unfavourable outcome after the Kasai procedure because the survival with native liver drops below 30% over the long term. Therefore, the following aspects should be considered alongside individual health issues: first, early organ replacement is a multifactorial burden for the growing organism, and second, every patient who survives with their native liver relieves the limited availability of donor organs and benefits national health expenditures with economic savings. From that point of view, international and interdisciplinary strategies are mandatory, in as much as BA is a rare disease. Over the past several years, national and international initiatives have been established in order to improve screening, centralization, diagnosis, as well as surgical and adjuvant treatment protocols. One such initiative is the recently launched European rare diseases network “rare-liver”. 2024-07-04T09:39:19Z 2024-07-04T09:39:19Z 2024 book ONIX_20240704_9783725812172_96 9783725812172 9783725812189 https://directory.doabooks.org/handle/20.500.12854/139300 eng application/octet-stream Attribution-NonCommercial-NoDerivatives 4.0 International https://mdpi.com/books/pdfview/book/9296 https://mdpi.com/books/pdfview/book/9296 MDPI - Multidisciplinary Digital Publishing Institute 10.3390/books978-3-7258-1218-9 10.3390/books978-3-7258-1218-9 46cabcaa-dd94-4bfe-87b4-55023c1b36d0 9783725812172 9783725812189 218 open access |
| spellingShingle | biomarker cholangiocyte ductular reaction liver fibrosis pediatric liver disease pregnancy biliary atresia chorine esterase MELD score liver transplantation Kasai operation liver transplant etiology adjuvant therapy budesonide cholangitis Kasai hepatoportoenterostomy cytomegalovirus survival native liver antiviral treatment newborn screening public health epidemiology liver nodules hepatocellular carcinoma regenerative nodules focal nodular hyperplasia Kasai portoenterostomy transplant waiting list pediatric liver transplantation referral practice outcome kasai portoenterostomy primary liver transplantation outcomes perinatal liver inflammation innate immune system monocytes cholangiopathy liver histology transplant-free survival corticosteroids ursodeoxycholic acid cytotoxic T cells perinatal liver injury hematopoietic stem and progenitor cells myeloid progenitor cells liver transition adolescence adherence readiness professionals parents pediatric liver surgery hepatoblastoma pediatric surgery workforce subspecialization thema EDItEUR::M Medicine and Nursing thema EDItEUR::M Medicine and Nursing::MJ Clinical and internal medicine::MJH Gastroenterology Biliary Atresia: Aetiology, Diagnosis and Treatment |
| title | Biliary Atresia: Aetiology, Diagnosis and Treatment |
| title_full | Biliary Atresia: Aetiology, Diagnosis and Treatment |
| title_fullStr | Biliary Atresia: Aetiology, Diagnosis and Treatment |
| title_full_unstemmed | Biliary Atresia: Aetiology, Diagnosis and Treatment |
| title_short | Biliary Atresia: Aetiology, Diagnosis and Treatment |
| title_sort | biliary atresia aetiology diagnosis and treatment |
| topic | biomarker cholangiocyte ductular reaction liver fibrosis pediatric liver disease pregnancy biliary atresia chorine esterase MELD score liver transplantation Kasai operation liver transplant etiology adjuvant therapy budesonide cholangitis Kasai hepatoportoenterostomy cytomegalovirus survival native liver antiviral treatment newborn screening public health epidemiology liver nodules hepatocellular carcinoma regenerative nodules focal nodular hyperplasia Kasai portoenterostomy transplant waiting list pediatric liver transplantation referral practice outcome kasai portoenterostomy primary liver transplantation outcomes perinatal liver inflammation innate immune system monocytes cholangiopathy liver histology transplant-free survival corticosteroids ursodeoxycholic acid cytotoxic T cells perinatal liver injury hematopoietic stem and progenitor cells myeloid progenitor cells liver transition adolescence adherence readiness professionals parents pediatric liver surgery hepatoblastoma pediatric surgery workforce subspecialization thema EDItEUR::M Medicine and Nursing thema EDItEUR::M Medicine and Nursing::MJ Clinical and internal medicine::MJH Gastroenterology |
| topic_facet | biomarker cholangiocyte ductular reaction liver fibrosis pediatric liver disease pregnancy biliary atresia chorine esterase MELD score liver transplantation Kasai operation liver transplant etiology adjuvant therapy budesonide cholangitis Kasai hepatoportoenterostomy cytomegalovirus survival native liver antiviral treatment newborn screening public health epidemiology liver nodules hepatocellular carcinoma regenerative nodules focal nodular hyperplasia Kasai portoenterostomy transplant waiting list pediatric liver transplantation referral practice outcome kasai portoenterostomy primary liver transplantation outcomes perinatal liver inflammation innate immune system monocytes cholangiopathy liver histology transplant-free survival corticosteroids ursodeoxycholic acid cytotoxic T cells perinatal liver injury hematopoietic stem and progenitor cells myeloid progenitor cells liver transition adolescence adherence readiness professionals parents pediatric liver surgery hepatoblastoma pediatric surgery workforce subspecialization thema EDItEUR::M Medicine and Nursing thema EDItEUR::M Medicine and Nursing::MJ Clinical and internal medicine::MJH Gastroenterology |
| url | ONIX_20240704_9783725812172_96 |