Advances in the diagnosis and treatment of pediatric idiopathic non-cirrhoticportal hypertension
Idiopathic non-cirrhotic portal hypertension(INCPH)is extremely rare in children and exhibits clinical presentations and etiological characteristics distinct from those in adults.While adult patients typically have normal transaminase and bilirubin levels,pediatric patients are more likely to present with liver function ab-normalities,including jaundice.The potential etiologies of INCPH include autoimmune diseases,infections,thrombophilia,drugs,and toxins.A notable feature of pediatric INCPH is the possibility of familial cases,with reported associations to mutations in genes such as HLR-DR3,FOPV,KCNN3,and DGUOK.Therefore,genetic testing should be considered when diagnosing INCPH in children.Early-stage pediatric INCPH may lack overt manifesta-tions of portal hypertension,which can prevent a diagnosis based on classic INCPH criteria.It is recommended to expand the definition of INCPH to better capture relevant data and improve understanding of its characteristics in children.
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