Tip of the iceberg: A comprehensive review of liver disease in Inborn errors of immunity. [Review]
Tip of the iceberg: A comprehensive review of liver disease in Inborn errors of immunity. [Review]
- 2022
Inborn errors of immunity (IEIs) comprise numerous rare, inherited defects of the immune system that affect about 500,000 people in the United States. As advancements in diagnosis through genetic testing and treatment with targeted immunotherapy and bone marrow transplant emerge, increasing numbers of patients survive into adulthood posing fresh clinical challenges. A large spectrum of hepatobiliary diseases now present in those with immunodeficiency diseases, leading to morbidity and mortality in this population. Awareness of these hepatobiliary diseases has lagged the improved management of the underlying disorders, leading to missed opportunities to improve clinical outcomes. This review article provides a detailed description of specific liver diseases occurring in various inborn errors of immunity. A generalized approach to diagnosis and management of hepatic complications is provided and collaboration with hepatologists, immunologists, and pathologists is emphasized as a requirement for optimizing management and outcomes. Copyright This article is protected by copyright. All rights reserved.
English
0270-9139
10.1002/hep.32539 [doi]
IN PROCESS -- NOT YET INDEXED
MedStar Washington Hospital Center
Internal Medicine Residency
Journal Article
Review
Inborn errors of immunity (IEIs) comprise numerous rare, inherited defects of the immune system that affect about 500,000 people in the United States. As advancements in diagnosis through genetic testing and treatment with targeted immunotherapy and bone marrow transplant emerge, increasing numbers of patients survive into adulthood posing fresh clinical challenges. A large spectrum of hepatobiliary diseases now present in those with immunodeficiency diseases, leading to morbidity and mortality in this population. Awareness of these hepatobiliary diseases has lagged the improved management of the underlying disorders, leading to missed opportunities to improve clinical outcomes. This review article provides a detailed description of specific liver diseases occurring in various inborn errors of immunity. A generalized approach to diagnosis and management of hepatic complications is provided and collaboration with hepatologists, immunologists, and pathologists is emphasized as a requirement for optimizing management and outcomes. Copyright This article is protected by copyright. All rights reserved.
English
0270-9139
10.1002/hep.32539 [doi]
IN PROCESS -- NOT YET INDEXED
MedStar Washington Hospital Center
Internal Medicine Residency
Journal Article
Review