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. Cause
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. Mechanism
Embryogenically, congenital hepatic fibrosis 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. Congenital hepatic fibrosis 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. Diagnosis
Liver biopsy is diagnostic. In biopsy there is diffuse periportal and perilobular fibrosis in broad bands, containing distorted duct like structure or microcyst formation. Management
Management includes control of esophageal bleeding/varices and treatment of associated renal disease if present. See also
Caroli disease
Polycystic kidney disease
Von Meyenburg complex
Biliary hamartomas

References
External links

GeneReviews/NCBI/NIH/UW entry on Congenital Hepatic Fibrosis Overview
Ectasia (), also called ectasis (), is dilation or distention of a tubular structure, either normal or pathophysiologic but usually the latter (except in atelectasis, where absence of ectasis is the problem). Specific conditions
Bronchiectasis, chronic dilatation of the bronchi
Duct ectasia of breast, a dilated milk duct. Duct ectasia syndrome is a synonym for nonpuerperal (unrelated to pregnancy and breastfeeding) mastitis. Dural ectasia, dilation of the dural sac surrounding the spinal cord, usually in the very low back. Pyelectasis, dilation of a part of the kidney, most frequently seen in prenatal ultrasounds. It usually resolves on its own. Rete tubular ectasia, dilation of tubular structures in the testicles. It is usually found in older men. Acral arteriolar ectasia
Corneal ectasia (secondary keratoconus), a bulging of the cornea.Vascular ectasiasMost broadly, any abnormal dilatation of a blood vessel, including aneurysms
Annuloaortic ectasia, dilation of the aorta. It can be associated with Marfan syndrome. Dolichoectasias, weakening of arteries, usually caused by high blood pressure. Intracranial dolichoectasias, dilation of arteries inside the head. Gastric antral vascular ectasia, dilation of small blood vessels in the last part of the stomach. Telangiectasias are small dilated blood vessels found anywhere on the body, but commonly seen on the face around the nose, cheeks, and chin. Venous ectasia, dilation of veins or venules, such as:
Chronic venous insufficiency, often in the leg
Jugular vein ectasia, in the jugular veins returning blood from the head

See also
All pages with titles beginning with Ectasia
All pages with titles containing Ectasia


== References ==