INVOLVEMENT OF EXOCRINE PANCREAS IN WILSONS DISEASE

A normal exocrine pancreatic function was demonstrated by the secretin-pancreozymin-test in five patients with Wilson's disease either without (n=2) or with cirrhosis of the liver but without portal hypertension (n=3). In another patient with cirrhosis of the liver without portal hypertension the pancreas was normal at post mortem examination. In two patients with cirrhosis of the liver and portal hypertension bicarbonate (n=1) and amylase secretion (n=2) were diminished. The regression of portal hypertension under therapy with penicillamine in one of the latter cases was paralleled by the return to normal of exocrine pancreatic function. It is concluded that exocrine pancreatic insufficiency in Wilson's disease is dependent on the development and the progression of chirrhosis of the liver and not due to a primary manifestation of the disease itself. © 1978 Springer-Verlag.