Congenital hepatic fibrosis Congenital hepatic fibrosis Congenital hepatic fibrosis is an inherited fibrocystic liver disease associated with proliferation of interlobular bile ducts within the portal areas and fibrosis that do not alter hepatic lobular architecture. The fibrosis would affect resistance in portal veins leading to portal hypertension. The condition is usually congenital, but sporadic cases have also been reported. It may be associated with other congenital defects, commonly with autosomal recessive polycystic kidney disease, the most severe form of PKD. Some suggest that these two conditions are one disorder with different presentation. Embryogenically, congenital hepatic fibrosis is due to malformation of the duct
Organ of Zuckerkandl Organ of Zuckerkandl The organ of Zuckerkandl is a chromaffin body derived from neural crest located at the bifurcation of the aorta or at the origin of the inferior mesenteric artery. It can be the source of a paraganglioma. The term para-aortic body is also sometimes used to describe it, as it usually arises near the abdominal aorta, but this term can be the source of confusion, because the term "corpora paraaortica" is also used to describe the aortic body, which arises near the thoracic aorta. This diffuse group of neuroendocrine sympathetic fibres was first described by Emil Zuckerkandl, a