Nesidioblastosis
Synonyms
Hyperinsulinism, Familial, with Pancreatic Nesidioblastosis
Nesidioblastoses
Nesidioblastoses, Pancreatic
Nesidioblastosis of Pancreas
Nesidioblastosis, Pancreatic
Pancreas Nesidioblastoses
Pancreas Nesidioblastosis
Pancreatic Nesidioblastoses
Pancreatic Nesidioblastosis
An inherited autosomal recessive syndrome characterized by the disorganized formation of new islets in the PANCREAS and CONGENITAL HYPERINSULINISM. It is due to focal hyperplasia of pancreatic ISLET CELLS budding off from the ductal structures and forming new islets of Langerhans. Mutations in the islet cells involve the potassium channel gene KCNJ11 or the ATP-binding cassette transporter gene ABCC8, both on CHROMOSOME 11.