Isolated polycystic liver disease not linked to polycystic kidney disease 1 and 2.

IGLESIAS, DM; PALMITANO, J. A; ARRIZURIETA, E.,; KORNBLIHTT AR; HERRERA M; BERNATH, V.A; MARTIN R

DIGESTIVE DISEASE AND SCIENCES

SPRINGER

Año: 1999 p. 385 - 388

0163-2116

Autosomal dominant polycystic liver disease occurs commonly in association with autosomal dominant polycystic kidney disease, types 1 and 2. It may also exist as a separate entity, genetically distinct from autosomal dominant polycystic kidney disease types 1 and 2, as has been recently established to exist in a Belgian family. We report here a large Argentinian family of Spanish-Belgian ancestry with autosomal dominant polycystic liver disease, where proximal and distal markers for both polycystic kidney disease 1 and 2 failed to demonstrate genetic linkage. The data support the notion that polycystic liver disease and autosomal dominant polycystic kidney disease may have separate chromosomal loci.