Congenital hepatic fibrosis
Encyclopedia
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 most severe form of PKD. Some suggest that these two conditions are one disorder with different presentation.
Embryogenically
, CHF is due to malformation of the duct plate, a round structure appearing in the eighth week of gestation
that is formed by primitive hepatocytes, which differentiate into cholangiocytes
. CHF usually presents in adolescent or young adulthood, but onset of signs and symptoms can range from early childhood through mid-life. Clinical features may vary but commonly include Cholangitis, hepatomegaly
and signs of portal hypertension.
Heredity
Heredity is the passing of traits to offspring . This is the process by which an offspring cell or organism acquires or becomes predisposed to the characteristics of its parent cell or organism. Through heredity, variations exhibited by individuals can accumulate and cause some species to evolve...
fibrocystic liver disease associated with proliferation of interlobular bile ducts within the portal areas and fibrosis
Fibrosis
Fibrosis is the formation of excess fibrous connective tissue in an organ or tissue in a reparative or reactive process. This is as opposed to formation of fibrous tissue as a normal constituent of an organ or tissue...
that do not alter hepatic lobular
Hepatic lobule
A hepatic lobule is a small division of the liver defined at the histological scale. It should not be confused with the anatomic lobes of the liver , or any of the functional lobe classification systems....
architecture. The fibrosis would affect resistance in portal veins leading to portal hypertension
Portal hypertension
In medicine, portal hypertension is hypertension in the portal vein and its tributaries.It is often defined as a portal pressure gradient of 10 mmHg or greater.-Causes:Causes can be divided into prehepatic, intrahepatic, and posthepatic...
.
Overview
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 diseasePolycystic kidney disease
Autosomal dominant polycystic kidney disease is an inherited systemic disorder that predominantly affects the kidneys, but may affect other organs including the liver, pancreas, brain, and arterial blood vessels...
, the most severe form of PKD. Some suggest that these two conditions are one disorder with different presentation.
Embryogenesis
Embryogenesis is the process by which the embryo is formed and develops, until it develops into a fetus.Embryogenesis starts with the fertilization of the ovum by sperm. The fertilized ovum is referred to as a zygote...
, CHF is due to malformation of the duct plate, a round structure appearing in the eighth week of gestation
Gestation
Gestation is the carrying of an embryo or fetus inside a female viviparous animal. Mammals during pregnancy can have one or more gestations at the same time ....
that is formed by primitive hepatocytes, which differentiate into cholangiocytes
Cholangiocytes
Cholangiocytes are the epithelial cells of the bile duct. They are cuboidal epithelium in the small interlobular bile ducts, but become columnar and mucus secreting in larger bile ducts approaching the porta hepatis and the extrahepatic ducts....
. CHF usually presents in adolescent or young adulthood, but onset of signs and symptoms can range from early childhood through mid-life. Clinical features may vary but commonly include Cholangitis, hepatomegaly
Hepatomegaly
Hepatomegaly is the condition of having an enlarged liver. It is a nonspecific medical sign having many causes, which can broadly be broken down into infection, direct toxicity, hepatic tumours, or metabolic disorder. Often, hepatomegaly will present as an abdominal mass...
and signs of portal hypertension.
See also
- Other fibrocystic liver diseases:
- Caroli diseaseCaroli diseaseCaroli disease is a rare inherited disorder characterized by dilatation of the intrahepatic bile ducts. There are two types of Caroli disease, the most common being the simple, or isolated case where the bile ducts are widened by ectasia. The second, more complex, cause is commonly known as Caroli...
- Polycystic kidney diseasePolycystic kidney diseaseAutosomal dominant polycystic kidney disease is an inherited systemic disorder that predominantly affects the kidneys, but may affect other organs including the liver, pancreas, brain, and arterial blood vessels...
- Von Meyenburg complexVon Meyenburg complexIn histopathology, a bile duct hamartoma, also von Meyenburg complex, Meyenburg complex and biliary hamartoma, is a benign tumour-like malformationof the liver....
- Biliary hamartomas
- Caroli disease