NCPH is a heterogeneous group of liver disorders of vascular origin, leading to PHT with
near normal HVPG. NCPF/IPH is a disorder of young adults or middle aged women …

Objectives: The published natural history of congenital hepatic fibrosis (CHF) was examined
to inform clinical decision making in autosomal recessive polycystic kidney disease …

PORTAL hypertension may result from extrahepatic venous obstruction, distortion of the
intrahepatic vasculature, or obstruction of the hepatic veins. In Great Britain the syndrome is …

Cholestatic jaundice in early infancy is a complex diagnostic problem. Misdiagnosis of
cholestasis as physiologic jaundice delays the identification of severe liver diseases. In the …

Non-cirrhotic portal hypertension (NCPH) encompasses a wide range of vascular conditions
leading to portal hypertension (PHT) associated with normal or mildly elevated hepatic …

Chronic cholestasis due to heritable causes is usually diagnosed in childhood. However,
many cases can present and survive into adulthood. The time course varies considerably …

The depth of our knowledge regarding mast cells has widened exponentially in the last 20
years. Once thought to be only important for allergy-mediated events, mast cells are now …

Background. Congenital hepatic fibrosis is a hereditary fibropolycystic disease caused by
ductal plate malformation. It is characterized by portal hypertension, but the manifestations …

Abnormalities in primary cilia lead to diseases called ciliopathies. Multiple organ
involvement is the norm since primary cilia are present in most cells. When cholangiocyte …

Ductal plate malformations (DPMs) represent developmental biliary disorders with a wide
phenotypic spectrum. This study characterizes DPM in 30 Boxer dogs. Median age was 1.5 …