小児門脈圧亢進症の免疫学的プロファイル

Portal hypertension is a clinical syndrome with potentially life-threatening complications. Diagnosis and management in children are complex due to the invasiveness of hepatic venous pressure gradient measurement and the limited number of definitive treatment options, so that liver transplantation often remains the only definitive treatment. The influence of the immune system on the development of portal hypertension has recently received attention; however, the connection between portal hypertension, impaired immune response, and the development of liver changes has not yet been fully elucidated. This review provides an overview of the main current knowledge on the role of cytokines, immune cells, and other molecules involved in the inflammation and vascular changes associated with portal hypertension. A better understanding of the pathogenesis of portal hypertension could address the need to identify non-invasive markers for the diagnosis of portal hypertension and predictors of its complications in children. Furthermore, understanding the strong interaction between the immune system and the development of portal hypertension could be useful for identifying new potential therapeutic options, orienting therapeutic management towards immunomodulatory approaches.