Congenital hepatic fibrosis

Congenital hepatic fibrosis

    * Introduction
    * Signs and symptoms
    * Diagnosis
    * Treatment
It is autosomal recessive hereditary disease that mainly affects the liver and kidneys. It is characterized by liver fibrosis, portal hypertension and cystic kidney disease. Polycystic disease belongs to the category, which includes Caroli disease, autosomal recessive polycystic kidney disease and autosomal dominant polycystic kidney disease.
Most infants with this diagnosis predominantly renal involvement and renal failure will die in early childhood. Over 25% of patients with renal failure sucumba. Cholangitis contributes significantly to morbidity and mortality in congenital hepatic fibrosis. When lesions predominate in clinical liver disease, affected children may remain asymptomatic until adolescence or adulthood. Also, and renal lesions will remain asymptomatic until adolescence.
Symptoms include abdominal pain, enlargement of the spleen and kidney complications of portal hypertension such as esophageal and gastric varices and their bleeding. Pathological condition is usually diagnosed in childhood because of hepatomegaly and variceal bleeding. Diagnosis is made by combining ultrasound for liver and kidney, computed tomography scan, angiography vascular, gastrointestinal endoscopy and biopsy to determine hepatic veins.
Congenital hepatic fibrosis differs from liver cirrhosis because the cells remain functional. There is no cure for this disease. You can choose to redefine a path are intestinal blood that perfuzeaza intestine and thus prevent bleeding vericiala. Singereaza esophageal varices which can be treated by digestive endoscopy.
Pathogenesis and causes
Result from congenital hepatic fibrosis precursurilor embryological malformation of the bile ducts, periportal fibrosis and secondary biliary strictures. This sequence leads to portal hypertension. This precursor is represented by a cylindrical layer of cells surrounding a branch of the portal vein. It is a precursor of the intrahepatic bile ducts. Progressive Remodeling begins in week 12 of pregnancy. Both interlobular and intralobular ducts develop from the same precursor. Lack of remodeling results in the persistence of excess embryonic structures. This abnormality was called ductal malformation.
Family fibrocystic disease is characterized by varying degrees of ductal structures and fibrosis with persistent ductal dilation. Liver fibrosis is a congenital malformation of the small interlobular bile ducts, while Caroli's disease involving large intrahepatic bile ducts. Classical renal lesion associated with polycystic kidney disease is disease that causes a severe impairment of renal function. Liver disease progresses through the development of portal hypertension associated with splenomegaly and esophageal varices. Congenital hepatic fibrosis is characterized by intrahepatic form of portal hypertension caused by intrahepatic obstruction that affects the blood supply of the liver and lead to the development of cavernous transformation secondary to portal vein and increased venous pressure.
Congenital hepatic fibrosis is associated with cholangitis. This was repeated colangitei or may influence the incidence of liver status and prognosis. Frequently associated with liver damage renal involvement characterized by cystic tubular dialatarea that affects the bone marrow and renal cortex. The more the patient survives more with both renal pathology is less prevalent.