Familial hepatic cirrhosis.

Interest has been revived in the aetiology of hepatic fibrosis in infants by the recent communications of Stokes, Berk, Malamut, Drake, Barondess, Bashe, Wolman, Farquhar, Bevan, Drummond, Maycock, Capps and Bennett (1954) and Dible, Hunt, Pugh, Steingold and Wood (1954). Dible et al. showed that hepatitis can occur during intra-uterine life with fatal results in the neonatal period, or perhaps later in infancy and childhood. Their eight cases formed a homogeneous group, with similar histological appearances in the liver, and included siblings. Stokes et al. described a female carrier of an icterogenic virus following homologous serum jaundice, who gave birth to a child who subsequently developed hepatic fibrosis and died at 18 months. They also showed that the plasma of known icterogenic carriers was capable of producing hepatitis in healthy volunteers. This work has thrown some light on hepatic fibrosis in siblings, and it seems likely that the infants described by Dible et al. are only extreme examples, and that many affected infants must survive with some degree of liver damage. However, there is no indication so far as to the cause of this hepatitis; the common virus of infective hepatitis, virus A, does not seem to be involved. The case of Stokes et al. indicates that placental transfer of virus can occur, but the frequency of this is not known. Some cases of hepatic fibrosis in siblings may be due to Rh iso-immunization (Drummond and Watkins, 1946; Craig, 1950), but Gerrard (1952) in a follow-up survey of Rh-affected children could find no evidence of permanent liver damage. A few examples are seen in rare familial states such as Lignac-Fanconi's disease and hepato-lenticular degeneration (Wilson's disease), but the liver damage occurring here is only part of the pathological process, although in Wilson's disease it may be of primary importance. Cases of hepatic disease in siblings where the aetiology is completely unknown have been called 'familial hepatic cirrhosis' (Weber, 1946). The family described in this communication is one of seven children; two certainly and most probably a third suffered from severe hepatic disease which caused death in all three before the age of 1 year. An amino-aciduria in certain members of the surviving family is also described.