Can alagilles syndrome be mistook portal pulmonary hypertension?
Alagille syndrome is an autosomal dominant disorder also referred to as Alagille- Watson syndrome, syndromic bile duct paucity and arteriohepatic dysplasia The course is characterized by recurrent episodes of cholestasis. The long- term prognosis is related to the severity and duration of early cholestasis, severity of complex cardiovascular abnormalities and the severity of liver disease and its associated portal hypertension. Portal Hypertension Synonyms and related keywords: cirrhosis, variceal hemorrhage, ascites, portal vein. Portal hypertension may be defined as a portal pressure gradient of 12 mm Hg or greater and is often associated with varices and ascites. Many conditions are associated with portal hypertension, of which cirrhosis is the most common cause. Alagille syndrome is a dominantly inherited systemic disorder consisting of abnormalities of the liver, heart, eye, spine, facies, kidney, vasculature and other organs. It is caused by mutations in Jagged1, a ligand in the
