What’s nesidioblastosis?

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Nesidioblastosis is a rare condition causing dangerous hypoglycemia due to excessive insulin, usually in children under 2 years old. The term fell out of favor due to its association with beta cell abnormalities, which can occur without hypoglycemia. Adult-onset nesidioblastosis can occur after gastric bypass surgery and its cause is unclear. Clinicians should educate postoperative patients about hypoglycemia symptoms and screen for nesidioblastosis.

Nesidioblastosis is dangerous persistent hypoglycemia, or low blood glucose levels, caused by too much insulin, a condition called hyperinsulinism when it’s not caused by an insulin-producing tumor. Hypoglycemia is a potentially dangerous condition that can cause symptoms such as headache, confusion, tremors, blurred vision and heart palpitations. It can also cause seizures, brain damage, coma or even death. Newborns with this condition need surgery to remove part of the pancreas — called a partial pancreatectomy — or they can suffer severe brain damage or death.

Scientist George F. Laidlaw coined the term “nesidioblastosis” in 1938 to refer to children with pancreatic abnormalities, including too many pancreatic beta cells, beta cells where they shouldn’t be, and beta cells that are larger than normal. The term “nesidioblastosis” fell out of favor when scientists discovered that some people have the physical characteristics of nesidioblastosis without developing hypoglycemia, so doctors instead began identifying the disorders based on their symptoms, such as persistent hyperinsulinemic hypoglycemia from (PHHI) or congenital hyperinsulinism. Scientists in the 1980s discovered that many patients with hyperinsulinism have lower levels of somatostatin, a hormone that inhibits insulin release, so hyperinsulinism involves more than just abnormal beta cells. Although persistent hypoglycemia caused by hyperinsulinism usually occurs in children 2 years of age and younger when the pancreas is still developing, doctors began using the term nesidioblastosis in early 21 century to refer to persistent hyperinsulinemic hypoglycemia in adults who develop beta-cell abnormalities following bypass surgery.

In the 1930s, doctors noticed that some children who developed severe persistent hypoglycemia had very high levels of insulin in their blood. The pathologists found that many of these children had specific pancreatic abnormalities involving beta cells, which are insulin-producing cells located in the pancreas’ islets of Langerhans. Children with severe hypoglycemia due to excessive insulin production had enlarged beta cells and beta cells where there shouldn’t be any, such as in and around the pancreatic ducts.

Scientists in the 1970s and 1980s discovered that some people have the pancreatic abnormalities seen in nesidioblastosis without suffering from persistent hypoglycemia or hyperinsulinemia. Pathologists have found abnormal formation and positioning of beta cells in people with cystic fibrosis, Zollinger-Ellison syndrome, or sudden infant death syndrome, and even in healthy newborns. Hyperinsulinism is usually not caused by beta cell problems alone, but by a combination of problems, including too few pancreatic delta cells, which release somatostatin, a hormone that inhibits insulin release. These discoveries caused the name “nesidioblastosis” to fall out of favor with many physicians, so that “PHHI”, “congenital hyperinsulinism”, “nesidiodysplasia”, and “islet cell dematuration syndrome” became the preferred terms.

Although nesidioblastosis almost always occurs in children 2 years of age and younger, rare cases can occur in adults, sometimes as a side effect of gastric bypass surgery. As of early 2011, the cause of adult-onset nesidioblastosis remained unclear. Some adults with nesidioblastosis have antibodies to beta cells in their blood serum, which could cause excessive beta cell growth as a reaction to attack by the body’s immune system; other adult patients with nesidioblastosis have elevated levels of some beta-cell growth factors and increased numbers of beta-cell growth factor receptors in the pancreas, which could cause abnormal beta-cell growth. Both of these conditions have been found in gastric bypass patients who develop nesidioblastosis. Nesidioblastosis is a rare complication of gastric bypass surgery, the serious nature of the disorder and the increasing number of gastric bypass procedures mean that clinicians need to educate postoperative patients about the symptoms of hypoglycemia and screen hypoglycemic patients for nesidioblastosis .

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