{"title":"[儿科急性肝衰竭的组织学特征:来自英国儿科肝病三级转诊中心的经验]。","authors":"Jens Stahlschmidt","doi":"10.1007/s00292-021-01025-5","DOIUrl":null,"url":null,"abstract":"<p><p>Paediatric acute liver failure (PALF; also fulminant hepatic failure, fulminant hepatitis) is a critical clinical syndrome that is characterised by a sudden, rapid deterioration and disease progression in a usually previously healthy child. The pathogenesis is an advanced degree of hepatocellular necrosis that exceeds the rate of hepatocyte regeneration. The diagnostic criteria of PALF (modelled on adult criteria) were developed by the \"Pediatric Acute Liver Failure (PALF) Study Group\" (NIH). The rule of the liver biopsy in PALF is controversial and in some cases contraindicated (coagulopathic state). In addition, extensive necrosis is a common finding in PALFs but may not be predictive of the overall outcome (transplantation versus continuous treatment) due to sampling issues. There are, however, some histological patterns that offer a degree of specificity that can be carefully considered in the overall clinical picture. The histopathologists will be part of a multidisciplinary team and can contribute to the diagnostic and prognostic pathway.The aetiologies of PALF are numerous and depend on age and geographical region. For all age groups the main causes can be divided into infectious, immunological, metabolic and toxin drug related. Rarer causes include circulatory disorders and malignancies. In the paediatric group, up to 30-50% of causes leading to PALF remain unknown.</p>","PeriodicalId":54641,"journal":{"name":"Pathologe","volume":" ","pages":"170-174"},"PeriodicalIF":0.0000,"publicationDate":"2021-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"[Histological features of paediatric acute liver failure : Experiences from a tertiary referral centre for paediatric liver disease in the UK].\",\"authors\":\"Jens Stahlschmidt\",\"doi\":\"10.1007/s00292-021-01025-5\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Paediatric acute liver failure (PALF; also fulminant hepatic failure, fulminant hepatitis) is a critical clinical syndrome that is characterised by a sudden, rapid deterioration and disease progression in a usually previously healthy child. The pathogenesis is an advanced degree of hepatocellular necrosis that exceeds the rate of hepatocyte regeneration. The diagnostic criteria of PALF (modelled on adult criteria) were developed by the \\\"Pediatric Acute Liver Failure (PALF) Study Group\\\" (NIH). The rule of the liver biopsy in PALF is controversial and in some cases contraindicated (coagulopathic state). In addition, extensive necrosis is a common finding in PALFs but may not be predictive of the overall outcome (transplantation versus continuous treatment) due to sampling issues. There are, however, some histological patterns that offer a degree of specificity that can be carefully considered in the overall clinical picture. The histopathologists will be part of a multidisciplinary team and can contribute to the diagnostic and prognostic pathway.The aetiologies of PALF are numerous and depend on age and geographical region. For all age groups the main causes can be divided into infectious, immunological, metabolic and toxin drug related. Rarer causes include circulatory disorders and malignancies. In the paediatric group, up to 30-50% of causes leading to PALF remain unknown.</p>\",\"PeriodicalId\":54641,\"journal\":{\"name\":\"Pathologe\",\"volume\":\" \",\"pages\":\"170-174\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2021-12-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Pathologe\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1007/s00292-021-01025-5\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2021/12/10 0:00:00\",\"PubModel\":\"Epub\",\"JCR\":\"Q3\",\"JCRName\":\"Medicine\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Pathologe","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1007/s00292-021-01025-5","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2021/12/10 0:00:00","PubModel":"Epub","JCR":"Q3","JCRName":"Medicine","Score":null,"Total":0}
[Histological features of paediatric acute liver failure : Experiences from a tertiary referral centre for paediatric liver disease in the UK].
Paediatric acute liver failure (PALF; also fulminant hepatic failure, fulminant hepatitis) is a critical clinical syndrome that is characterised by a sudden, rapid deterioration and disease progression in a usually previously healthy child. The pathogenesis is an advanced degree of hepatocellular necrosis that exceeds the rate of hepatocyte regeneration. The diagnostic criteria of PALF (modelled on adult criteria) were developed by the "Pediatric Acute Liver Failure (PALF) Study Group" (NIH). The rule of the liver biopsy in PALF is controversial and in some cases contraindicated (coagulopathic state). In addition, extensive necrosis is a common finding in PALFs but may not be predictive of the overall outcome (transplantation versus continuous treatment) due to sampling issues. There are, however, some histological patterns that offer a degree of specificity that can be carefully considered in the overall clinical picture. The histopathologists will be part of a multidisciplinary team and can contribute to the diagnostic and prognostic pathway.The aetiologies of PALF are numerous and depend on age and geographical region. For all age groups the main causes can be divided into infectious, immunological, metabolic and toxin drug related. Rarer causes include circulatory disorders and malignancies. In the paediatric group, up to 30-50% of causes leading to PALF remain unknown.
期刊介绍:
Der Pathologe is an internationally recognized journal and combines practical relevance with scientific competence. The journal informs all pathologists working on departments and institutes as well as morphologically interested scientists about developments in the field of pathology.
The journal serves both the scientific exchange and the continuing education of pathologists.
Comprehensive reviews on a specific topical issue focus on providing evidenced based information under consideration of practical experience.
Freely submitted original papers allow the presentation of important clinical studies and serve the scientific exchange.