Giant-cell hepatitis

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. 

PFIC 2 This gene mutation, which is located on chromosome 2q24, impairs the canalicular bile salt export pump (BSEP), resulting in the accumulation of bile acids in hepatocytes. Laboratory tests show an increase in bile acid, AP and LAP levels with lowered gamma GT and cholesterol levels. Pruritus is common. There is histological evidence of neonatal giant cell hepatitis. Prognosis is poor. 

Fig. 22.5 Numerous multinuclear giant cells (centre of picture) as well as bile duct proliferation (arrow) in so-called giant-cell hepatitis (HE)...

In 1991 in Canada, J. Phillips et al. determined the presence of paramyxoviruses in patients with an infaust course of giant-cell hepatitis out of 10 patients, only 5 survived with the help of liver transplantation. Paramyxovirus nucleocapsid protein, with a diameter of 12-17 nm, was detected in the cytoplasm of the hepato-cytes. (74) Until then, this paramyxovirus had been unknown. It can cause severe acute hepatitis, which might even be fatal. (71, 73, 76) (s. p. 417 )... 

Cairns, A., McMahon, RJt.T. Giant-cell hepatitis associated with systemic lupus erythematosus. J. Chn. Pathol. 1996 49 183-184... 

Dohmen, K., Ohtsuka, S., Nakamura, H., Arase, K., Yokogawa, Y., Asayama, R., Kuroiwa, S., Ishibashi, H. Post-infantile giant-cell hepatitis in an elderly female patient with systemic lupus erythematosus. X Gastroenterol. 1994 29 362-368... 

Syncytial giant cell hepatitis occurred in a 26-year-old woman who used Isabgol (170). Autoimmune disease and viral infections were excluded. 

About 75% of cases of hepatitis in the neonate are idiopathic giant cell hepatitis, a disorder of unknown etiology characterized by cholestatic jaundice. There is a familial trend that may reflect an autosomal recessive inheritance. Jaundice appears withm the first 2 weeks. The child initially appears well and gains weight. The liver and spleen then become enlarged and stools become pale. Serum aminotransferases are usually >400 U/L the prothrombin time is prolonged. Liver biopsy reveals characteristic giant cells with hepatocyte... 

Setchell, K.D., Schwarz, M., O Connell, N.C., Lund, E.G., Davis, D.L., Lathe, R., Thompson, H.R., Weslie Tyson, R., Sokol, RJ., Russell, D.W. 1998. Identification of a new inborn error in bile acid synthesis mutation of the oxysterol 7ot-hydroxylase gene causes severe neonatal liver disease. J. Clin. Invest. 102 1690-1703. Clayton, P.T., Leonard, J.V., Lawson, A.M., Setchell, K.D., Andersson, S., Egestad, B., Sjovall, J. 1987. Familial giant cell hepatitis associated with synthesis of 3p,7a-dihydroxy- and 3p,7a,12a-trihydroxy-5-cholenoic acids. J. Clin. Invest. 79 1031-1038. 

Acute liver failure was reported in a 41-year-old woman who had been taking black cohosh for 2 weeks the dose and product used were not specified. In this case, the woman developed giant cell hepatitis that increased in severity after cessation of black cohosh (Dunbar and Solga 2007). 

Clayton, P.T., Leonard, J.V., Lawson, A.M., Setchell, K.D., Andersson, S., Egestad, B., Sjovall, J. (1987) Eamilial giant cell hepatitis associated with synthesis of 3 beta, 7 alpha-dihydroxy-and 3 beta,7 alpha, 12 alpha-trihydroxy-5-cholenoic acids. /. Clin. Invest., 79(4), 1031-1038. 

Clayton PT, Casteels M, Mieli Vergani G, Lawson AM. Familial giant cell hepatitis with low bile acid concentrations and increased urinary excretion of specific bile alcohols a new inborn error of bile acid synthesis Pediatr Res 1995, 37 424-431... 

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