Liver involvement in systemic amyloidosis is frequent but is rarely of clinical importance. Five patients with severe cholestatic jaundice are described and an additional 20 from published reports are reviewed. The most frequent presenting symptoms were lethargy and abdominal pain, which were present for a median of 11 months before the onset of jaundice. Hepatomegaly, usually marked, was present in 92%, with ascites in 56% of the cases. The serum bilirubin concentration was noticeably high and the serum globulin low. Histology of the liver showed considerable perisinusoidal deposition with a slight predilection for the periportal area. Two patients presented with predominant centrilobular deposition. Congo red staining was not uniformly positive. A variety of treatment regimens was tried but median survival was only three months from the onset of jaundice.

At one time it was thought that biliary epithelial cells simply formed the lining to the tubular conduits which constitute the biliary tract. Development of in vitro systems for culturing biliary epithelial cells has enabled functional studies which increasingly show that this is far from true, and that biliary epithelial cells do have important functional roles. Disruption of these functions may be involved in the generation of pathology. Most functional studies to date have utilised cells isolated from rat liver. Increasingly, variations are being found between human and animal cells both in terms of function and phenotype. The relevance of animal cells in the study of human disease therefore remains obscure. Human biliary tract disease has to date been studied almost exclusively by examination of histological sections. The development of improved methods for isolating highly pure biliary epithelial cells from human liver provides a new technology with which to investigate directly the dynamics of human biliary epithelial cell biology and pathobiology. It is predicted that further progress will now be made in dissecting the biology and physiology of human biliary epithelium.

A 90 year old woman with alveolar rhabdomyosarcoma of the gall bladder is described. The patient died with tumour metastasis four months after diagnosis despite complete excision of the tumour. The histological, immunohistochemical, and ultrastructural features of the tumour are summarised and published works are reviewed.