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.

Also Known As:

Nesidioblastosis of Pancreas; Pancreatic Nesidioblastosis; Nesidioblastoses; Nesidioblastoses, Pancreatic; Nesidioblastosis, Pancreatic; Pancreas Nesidioblastoses; Pancreas Nesidioblastosis; Pancreatic Nesidioblastoses; Hyperinsulinism, Familial, with Pancreatic Nesidioblastosis

Digestive System Diseases: 1042
- Pancreatic Diseases: 1635
  - Congenital Hyperinsulinism: 516
    - Nesidioblastosis: 279
Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 933
- Diseases Newborn Infant: 266
  - Congenital Hyperinsulinism: 516
    - Nesidioblastosis: 279
Nutritional and Metabolic Diseases: 15
- Metabolic Diseases: 10142
  - Glucose Metabolism Disorders: 375
    - Hypoglycemia: 22833
      - Congenital Hyperinsulinism: 516
        Nesidioblastosis: 279
    - Hyperinsulinism: 9517
      - Congenital Hyperinsulinism: 516
        Nesidioblastosis: 279

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