Tags: Disease.
Johanson–Blizzard syndrome (JBS) is a rare sometimes fatal autosomal recessive multisystem congenital disorder featuring abnormal development of the pancreas nose and scalp with mental retardation hearing loss and growth failure. It is sometimes described as a form of ectodermal dysplasia.The disorder is especially noted for causing profound developmental errors and exocrine dysfunction of the pancreas and it is considered to be an inherited pancreatic disease.