Intermittent Intrahepatic Cholestasis of Unknown Etiology in Five Young Males from The Faroe Islands

Abstract

Clinical and biochemical findings in 5 patients from the Faroe Islands with intermittent intrahepatic cholestasis of unknown etiology are described. This brings the number of recorded cases which fulfill the suggested criteria for the syndrome to 24. The Faroe patients are all males and born between 1938 and 1943. In three patients the episodes of jaundice started during the first years of life, in two they started after puberty. Four of the patients had symptoms of pancreatic affection,, in one the
diagnosis of chronic pancreatitis was confirmed by calcifications demonstrated on X-ray. Two patients are distantly related, and one patient has a sister who presumably suffers from the same disease. It ís conjectured that the pathogenesis is a defect in bile acid metabolism.