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.

How do enzymes work in CHI?

  • „„There are several brands of enzymes which comes in a capsule for example Creon®. Beads inside the capsule contain digestive enzymes. The outer capsule dissolves in the stomach.
  • „„The beads can be sprinkled onto food or in feeds or given with a small amount of puree in young children. They can be swallowed whole in older children.
  • „„The beads then move along to the small intestine where the coating dissolves releasing the enzymes. Food is then broken down and the nutrients are absorbed by the body.
  • „„Pancrex V® is a powder form of enzymes and can be mixed with water or milk and taken orally or through a nasogastric tube or gastrostomy feeding device.

When should enzymes be taken?

  • „„All food and drinks containing fat, including nutritional supplements, require enzymes. 
  • „„Enzymes need to be in the stomach at the same time as food.
  • „„Enzymes should be taken at the start of a meal or snack. If you are unsure how much will be eaten or the meal has several courses, the dose can be split.
  • If you forget to give enzymes at the start of a meal, it is not too late to take them at the end of a meal.
Compiled by: 
The Congenital Hyperinsulinism team in collaboration with the Child and Family Information Group
Last review date: 
August 2015


Please read this information sheet from GOSH alongside the patient information leaflet (PIL) provided by the manufacturer. If you do not have a copy of the manufacturer’s patient information leaflet please talk to your pharmacist. A few products do not have a marketing authorisation (licence) as a medicine and therefore there is no PIL.

For children in particular, there may be conflicts of information between the manufacturer’s patient information leaflet (PIL) and guidance provided by GOSH and other healthcare providers. For example, some manufacturers may recommend, in the patient information leaflet, that a medicine is not given to children aged under 12 years. In most cases, this is because the manufacturer will recruit adults to clinical trials in the first instance and therefore the initial marketing authorisation (licence) only covers adults and older children.  

For new medicines, the manufacturer then has to recruit children and newborns into trials (unless the medicine is not going to be used in children and newborns) and subsequently amend the PIL with the approved information. Older medicines may have been used effectively for many years in children without problems but the manufacturer has not been required to collect data and amend the licence. This does not mean that it is unsafe for children and young people to be prescribed such a medicine ‘off-licence/off-label’. However, if you are concerned about any conflicts of information, please discuss with your doctor, nurse or pharmacist.