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What is Congenital Hyperinsulinism (CHI)?

Hyperinsulinism is the inappropriate and unregulated insulin secretion in relation to the blood glucose concentration. It is the main cause of recurrent and persistent hypoglycaemia in infancy and childhood and is a major cause of hypoglycaemic brain injury.

Normally, blood glucose levels are regulated by insulin secretion, however, in Hyperinsulinism, the beta-cells of the pancreas release insulin inappropriately all the time causing low blood glucose levels (hypoglycaemia). The high levels of insulin prevent ketones being made and so the brain is not only starved of its vital fuel glucose, but also prevents ketone bodies being used as alternative fuels.

Becky C 3

  • Hyperinsulinism affects between 1:30,000 and 1:40,000 babies in the UK and Ireland and a estimated global prevalence of 1:50,000).
  • It typically presents in the neonatal period, infancy and childhood.
  • It can be transient, persistent, mild or severe.

 So far, up to 12 genetic mutations have been identified along with links to other syndromes such as Beckwith Wiedemann, Turner and Kabuki syndrome.

Other causes of hypoglycaemia can include fasting, postprandial (after meals including dumping syndrome and being protein sensitive), as well as being exercise induced.

Many children with Hyperinsulinism can suffer other issues in relation to the management of the condition such as fluid overload, cardiac/respiratory issues, infections, feeding issues (loss of orality), gastro-oesophageal reflux and feed associated problems such as intolerances and allergies.

Early recognition and prompt treatment are vital in preventing hypoglycaemic brain injury.

The Childrens Hyperinsulinism Charity

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