Xanthomatous biliary cirrhosis

Abstract

Summary Marked improvement in the serum lipid pattern in a case of xanthomatous biliary cirrhosis treated with the rice diet (Kempner) has been demonstrated. There is a cardiac element in this case that is deteriorating and the two etiologic possibilities are outlined. The flat oral glucose tolerance curve has been shown to be due to increased utilization by getting a similar picture on the intravenous test. There is a failure of conversion of galactose as seen by the high blood levels but rapid absorption evidently occurs. Epinephrine does not appear to be effective in raising blood sugar as there is probably no storage in the liver due to high utilization rate. It was found possible to elevate the esterification of cholesterol 25 per cent on a short course of cortisone therapy even in the face of previous alcoholic history and prior fixed values. Bioassay for adrenal activity showed normal corticoid excretion but low 17-ketosteroid excretion. The cardinal features of this syndrome are re-emphasized and further information cited. The authors wish to express their appreciation to Drs. W. Wallop and W. Long, internes, and Dr. Henry Schwartz, chief biochemist, Philadelphia General Hospital and Dr. Harry Shay, Temple Hospital, for their kind cooperation.