Bilateral renal dysplasia, pancreatic fibrosis, intrahepatic biliary dysgenesis, and situs inversus totalis in a boy

This is a case report of a Japanese newborn infant showing bilateral renal dysplasia, severe pancreatic fibrosis simulating cystic fibrosis, intrahepatic biliary dysgenesis, and situs inversus totalis. The chromosomal analysis was normal. A review of the literature showed that there was another case exactly the same. These two rare cases had no difinite evidence of blood relationship, but occurred in the same town in a remote area.