pancreatic agenesis, congenital

(redirected from pancreatic hypoplasia, congenital)

pancreatic agenesis, congenital

A syndrome (OMIM:260370) of endocrine and exocrine pancreatic insufficiency and neonatal onset of insulin-dependent diabetes.
 
Molecular pathology
Caused by mutations in PDX1, which encodes the insulin promoter factor-1 (IPF1).