@entity788 is the fifth leading cause of @entity5 in developed countries. Several family and population studies have suggested that there is a genetic predisposition in about 10% of cases. Despite this, pedigrees showing a definite Mendelian inheritance pattern are quite rare. Recently, a family came to our attention with several cases of @entity788 . A detailed family medical history was obtained from the index @entity1 . Medical records, including death certificates, histopathology, radiology, and laboratory reports from several family members were reviewed. Computerized tomography scans and CA19-9 serum assays were performed on selected family members. The family seems to have a syndrome of @entity420 , accompanied by @entity8 . Affected family members without @entity788 have elevated serum CA19-9 levels. This seems to be a previously undescribed syndrome. The family may be carrying a @entity5 suppressor gene mutation specific for @entity788 .

A syndrome of hereditary pancreatic adenocarcinoma and XXXX .

@entity1 :: ('9606', 'Species') :: ['patient'] @entity788 :: ('MESH:D010190', 'Disease') :: ['Pancreatic adenocarcinoma', 'pancreatic cancer', 'pancreatic adenocarcinoma'] @entity8 :: ('MESH:D007674', 'Disease') :: ['multiple cysts of the liver and kidneys'] @entity5 :: ('MESH:D009369', 'Disease') :: ['tumor', 'cancer death'] @entity420 :: ('MESH:D000230', 'Disease') :: ['autosomal dominant adenocarcinoma of the pancreas']
@entity8 :: (MESH:D007674,Disease) :: ['cysts of the liver and kidneys']
