Congenital Extrahepatic Portosystemic Shunt Complicated by the Development of Hepatoblastoma: A Case Report and Review of Literature.

Congenital portosystemic shunts (CPSS) or congenital extrahepatic portosystemic shunts (CEPS) is a rare malformation. This congenital anomaly presents with a diverse array of clinical manifestations, ranging from asymptomatic to severe complications such as cardiac failure, pronounced pulmonary hypertension, and widespread pulmonary arteriovenous malformations. CPSS increases the risk of developing benign or malignant liver tumors, including nodular regenerative hyperplasia, focal nodular hyperplasia, hepatic adenoma, hepatocellular carcinoma, and hepatoblastoma.

Six-year follow-up of a child with familial chylomicronemia syndrome: disease course and effectiveness of gemfibrozil treatment --case report and literature review.

Familial chylomicronemia syndrome (FCS) is a rare autosomal recessive disease affecting lipoprotein metabolism. FCS is estimated to occur in 1 in 1-2 million individuals and can be diagnosed at any age, equally affecting all genders, races, and ethnicities. The condition is characterized by hypertriglyceridemia, which may predispose patients to acute pancreatitis.