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The Pancreas/Insulin

The Pancreas

The pancreas is a small organ found near the stomach that has two main roles in the body:

  • Endocrine role – this means that it releases hormones into the bloodstream, in this case beta-cells release insulin (which lowers blood glucose) and alpha-cells release glucagon (which raises blood glucose by releasing it from stores).
  • Exocrine role – this means that it releases enzymes via a duct into the small intestine for digestion of fats and proteins.  This shouldn’t affect hyperinsulinism patients unless they have a near-total pancreatectomy, in which case they will need to take enzyme-replacements with their meals.

In hyperinsulinism the endocrine function, specifically the beta-cells are affected.

In the focal form of the disease the affected cells are located together in a cluster.  This focus is generally spherical between 2-10mm but can be much bigger or even have an “octopus” shape.  If the focus is removed this should cure the hyperinsulinism.

In the diffuse form, all the beta cells in the pancreas are affected.  Therefore treatment aims to stop all of these cells from producing too much insulin.  This is done through medication.  If this fails then doctors may decide to remove most of the pancreas. However, this does not remove all the beta cells and the hyperinsulinism may persist.

What is Insulin and now does it work?

Alder Hey Patient Information leaflet:

Insulin is a hormone, which controls the level of glucose (sugar) in the blood. Insulin is released by specialised beta-cells in the pancreas.

As food is eaten, blood glucose rises and the pancreas releases insulin to keep the blood glucose in the normal range. Insulin acts by driving glucose into the cells of the body. This action of insulin has two effects:

  1. Maintaining blood glucose levels
  2. Storing glucose as glycogen in the liver During a fast, insulin secretion is turned off, allowing the stores of glucose in glycogen to be released into the bloodstream to keep blood glucose normal.

In addition, with the switching off of insulin secretion, protein and fat stores become accessible and can be used instead of glucose as sources of fuel.

In this way, whether one eats or is fasting blood glucose levels remain in the normal range and the body has access to energy at all times.

What goes wrong in CHI?

 In CHI, the close regulation of blood glucose and insulin secretion is lost. The pancreas, which is responsible for insulin secretion, is blind to the blood glucose level and continues to release insulin despite low blood glucose level.

As a result, the baby or child with CHI develops severe hypoglycemia and ends up needing large amounts of glucose to maintain normal blood glucose levels.


Pancreas Infographic

Surgical Intervention – Pancreatectomy

Surgery is considered secondary to medical treatments and is often used only where drug treatment has been ineffective. 

Surgery is usually an option for those with focal disease who have identified areas of the pancreas with defective beta cells (usually following a PET scan*) and may offer a cure for CHI.

More extensive surgery to remove all or most of the pancreas is only considered an option for those with diffuse disease where medical intervention has failed. It should be noted this carries that this carries an increased risk of longer-term effects such as diabetes or pancreatic insufficiency and is not usually seen as curative. 

The Children’s Hospital of Philadelphia has more information on pancreatectomies and what to expect: 

Family story on pancreatectomy coming soon 

PET Scan

A PET scan gives very detailed, three-dimensional images of the body. It works by injecting an isotope. The isotope used is called 18-F-DOPA so you may hear the scan referred to by this name. PET scanning is a relatively new technology. The focal lesion is very tiny and cannot be seen by other X-ray techniques. With this scan, the doctors are trying to identify the area of the pancreas from which excessive insulin is being produced. The treatment recommended by the doctors depends on the results of the scan.

Pancreatic Surgery Infographic

Pancreatic enzyme replacement in congenital hyperinsulinism

Digestive enzymes are made in the pancreas. The fat, protein and carbohydrate in food is broken down by the enzymes to release nutrients. In congenital hyperinsulinism (CHI), if surgery has removed all or part of the pancreas, the food cannot be digested and absorbed by the body. This is called malabsorption and causes loose or oily stools, wind, stomach ache and poor weight gain.

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