Preferred Label : nesidioblastosis;

MeSH definition : 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.;

MeSH synonym : pancreatic nesidioblastosis; hyperinsulinism, familial, with pancreatic nesidioblastosis; nesidioblastosis, pancreatic; nesidioblastosis of pancreas; nesidioblastoses; nesidioblastoses, pancreatic; pancreatic nesidioblastoses; pancreas nesidioblastoses; pancreas nesidioblastosis;

Wikipedia link : https://en.wikipedia.org/wiki/Nesidioblastosis;

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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.

http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=FR&Expert=165988
2011
France
scientific and technical information
diazoxide
nesidioblastosis

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http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=FR&Expert=165985
2011
France
scientific and technical information
diazoxide
nesidioblastosis

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28/07/2025


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