The lpac syndrome an unknown entity but exists: study of three cases

Low Phospholipid Associated Cholelithiasis syndrome (LPAC) is a disease of the young woman. The diagnosis is suspected by the presence of certain criteria and confirmed by genetic testing. We report the case of three brothers who have presented with recurrent biliary pain and an abnormal liver function. Their parents were first degree cousins who had been surgically treated for a biliary disease. The diagnosis of LPAC was backed by clinical and radiological arguments. The three brothers were treated by ursedesoxycholic acid with regression of clinical signs and normalization of liver function.