2025 Volume 41 Issue 2 Pages 81-91
This article reviews three pediatric-onset liver diseases: Fontan-associated liver disease (FALD), liver cirrhosis caused by biliary atresia, and MASLD/MASH associated with hypopituitarism. All of these conditions carry a high risk of progression to liver fibrosis and cirrhosis, and require multidisciplinary collaborative treatment and long-term follow-up from childhood into adulthood. FALD is mainly caused by chronically elevated central venous pressure in the Fontan circulation, with imaging required for assessment of hepatic fibrosis and nodules. In biliary atresia, hepatic fibrosis often progresses even after a Kasai operation, with complications such as cholangitis, hepatolithiasis, and portal hypertension. Hypopituitarism predisposes a patient to MASLD/MASH, which may advance to cirrhosis, particularly due to reduced GH and IGF-1. MRI techniques, especially PDFF, are promising for noninvasive quantification of hepatic steatosis.
