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What causes it?
Ketotic hypoglycaemia means high levels of ketones in the body and low blood sugar (glucose) levels. Our body uses glucose for energy which we get from our diet and any excess is stored in the liver and stored fat. When we eat regular meals, our body uses the glucose in the meals as an energy source and therefore does not need to use much fat.
When our body needs more energy, such as during illness or after long periods without food it needs to break down fat quickly. However, the brain cannot use fats for energy straight away and it needs to be broken down into ketones. If a child has ketotic hypoglycaemia they are more likely to have low blood sugar during any illness. Their body will break down fat into ketones as usual, but these build up and become harmful
How common is it?
We do not know the exact prevalence of ketotic hypoglycaemia but it is believed to be the most common cause of hypoglycaemia (low blood sugar) in children who have not been diagnosed with diabetes between 0.5 and 6 years of age.
What are the signs and symptoms?
Ketotic hypoglycaemia mainly affects children between 0.5 to 6 years of age. The disorder usually becomes apparent when a young child is unwell with an infection or bug and goes to bed without much food in the evening. In the morning the child may have neurological signs such as confusion, personality or behavioural changes (e.g., irritability), drowsiness, and or seizures which leads to the parents or carers seeking medical advice.
Other symptoms may include:
- Feeling shaky or dizzy
- Sweating or appearing feverish
- Pale skin colour
- Muscle weakness
- Clumsy or jerky movements
- Poor attention span
- Tingling sensations around the mouth
These symptoms appear in episodes. Children with ketotic hypoglycaemia are generally well between these episodes. Episodes are triggered by long periods without food or any illness or vomiting which is often linked to an infection or virus.
Children may have several episodes over time however they will often grow out of these by age 6 with the condition resolving itself.
How is it diagnosed?
Diagnosis is usually made during the first episode. Ketones accumulate in the urine and so they can easily be tested for. In rare cases, Ketotic hypoglycaemia can be a sign of other disorders so additional tests are usually done during the first or second episode to rule these out.
Can it be treated?
Children often recover quickly from the first episode after being given glucose. Once a diagnosis is made, the child will be seen regularly to review how they are doing and advice will be provided about feeding times and treatment. These regular reviews will continue until the child has grown out of the condition.
Treatment is aimed at avoiding long periods without food and ensuring the child has frequent, balanced, small meals and snacks including a carbohydrate-rich snack before bedtime.
You will be given an emergency regimen which is to be given to the child during any signs of illness or if the child has not had food for some time and is experiencing symptoms. This is a special high energy drink that can be given at home. Symptoms should resolve quickly once given.
A metabolic dietitian will be able to advise on how to make and administer the drink and will regularly review this to ensure the treatment is always tailored to the age of the child
Seek urgent medical treatment by taking your child to the closest A&E department in the following circumstances:
- If the child has not responded to the emergency regimen or is showing signs of deterioration.
- If they are unable to take the emergency regimen for any reason (i.e. refusal, vomiting, drowsiness)
If the child is unconscious ring 999 for an ambulance.
The child should be admitted without delay if the parents/carers have deemed it valid for the child to be brought to hospital. Always take details of your emergency regimen with you and contact details of your metabolic team.
Your local hospital will provide intravenous dextrose (a form of sugar given through the vein) until the child recovers.
If you have any concerns about the child’s health or whether to start the emergency regimen then contact your metabolic team and attend your local hospital for tests.