Clinical Features and Risk Factors of Autoimmune Liver Involvement in Pediatric XXXX .

@entity1 @entity75 @entity409 @entity31 @entity14361 @entity29 @entity255
BACKGROUND: @entity409 is reported in up to 7.8% of @entity1 with @entity29 . A distinct @entity29 phenotype has been suggested in adults and in small pediatric cohorts. AIMS: To evaluate the features of @entity29 associated with @entity409 and to analyze the characteristics of the @entity409 . METHODS: Information on @entity1 was obtained from the Italian Pediatric @entity29 Registry. Data of @entity1 with and without @entity409 were compared. RESULTS: @entity409 was detected in 6.8% of the 677 @entity1 enrolled and was significantly associated with the diagnosis of @entity31 (83%), with @entity14361 (84%), and with perinuclear anti-neutrophil cytoplasmic antibody positivity (41%) (all ps < 0.05). @entity409 was defined as sclerosing @entity255 in 61% of the @entity1 and as an overlap syndrome in 33%. Concomitant intra and extrahepatic biliary involvement was reported in 61% of cases, while exclusive @entity75 were reported in 21%. Hepatobiliary complications were observed in 9% of the @entity1 during follow-up. CONCLUSIONS: @entity409 , especially sclerosing @entity255 , was significantly more common in @entity1 with extensive @entity31 . Although complications are relatively rare in the pediatric age, monitoring is recommended.
