Congenital liver fibrosis

Hodgkin s disease (31) parasites hepatolithiasis Caroli s syndrome congenital liver fibrosis... 

Cholangiodysplasia or congenital liver fibrosis (microcystic liver), congenital polycystic disease... 

Fig. 32.17 Congenital liver fibrosis onset of drrhotic transformation, pathologically augmented bile-duct aggregates in portal and septal areas (Sirius red)...

This congenital clinical picture was first described in the form of intrahepatic stones by H.R. Vachell et at in 1906. Later on, in 1958 J. Caroli et at were able to establish the association between characteristic congenital bile-duct alterations, cholangitis, cholangiolithiasis and renal cyst formation. In 1964, J. Caroli et at differentiated a particular form with simultaneous congenital liver fibrosis (which had already been described by D.V.S. Kerr et at in 1961). Both forms are autosomal recessive. 

Congenital cystic fibrosis (20) Fulminant liver failure (55)... 

This rare form, which is inherited via a recessive autosomal route, was described in 1968 as a variant of BRIC. (1, 2) The genetic defect is located on chromosome 15 q. A specific feature of this disease in neonates is giant-cell hepatitis with cholestasis, (s. p. 417) From about the 6 year of life until puberty, pathogenetic hypoplasia or ectasia of the lymphatic vessels with oedema are found in the lower extremities. It is not clear whether (suspected) congenital hyperplasia of lymphatic vessels in the liver is the prime cause of cholestasis. Pronounced fibrosis is often in evidence, whereas cirrhosis only occurs rarely. 

Delayed portal blood flow Congenital fibrosis Liver cirrhosis Lymphoma Nodular regenerative hyperplasia Retroperitoneal fibrosis Stenoses/strictures ... 

Other etiologies Congenital, idiopathic, hereditary (trypsinogen gene mutations), cystic fibrosis, inflammatory bowel disease, peptic ulcer disease, solid organ transplantation (liver, kidney, heart), refeeding... 

Dietary deficiency of vitamin E in human beings is unknown, although patients with severe fat malabsorption, cystic fibrosis, some forms of chronic liver disease or (very rare) congenital lack of plasma 3-lipoprotein suffer deficiency because they are unable to absorb the vitamin or transport it around the body. They suffer from severe damage to nerve and muscle membranes. 

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