POLYCYSTIC KIDNEY-DISEASE, BILIARY DYSGENESIS IN A PATIENT WITH LARSENS-SYNDROME

Larsen's syndrome is characterised by multiple joint dislocations, fla t face and talipes equinovarus. There is an autosomal dominant form an d also a more severe autosomal recessive form. Several types of polycy stic kidney disease have been reported in children. In this report we present an infant with a severe form of Larsen's syndrome (thought to be lethal Larsen-like), infantile-type polycystic kidney disease, bili ary dysgenesis and osteosclerosis.