Wellness Tips

Hypoketotic Hypoglycemia: Causes, Symptoms, and Treatment

Hypoglycemia is a condition where your blood sugar drops too low. Hypoketotic hypoglycemia occurs when you have low blood sugar due to the body’s inability to break down fats for energy.

Different conditions can cause hypoglycemia. With hypoketotic hypoglycemia, you have low blood sugar due to the body’s inability to break down fats for energy.

Hypoketotic hypoglycemia is linked to fatty acid oxidation disorders (FAODs). It’s most commonly seen in babies and young children.

Here’s what you need to know about this form of hypoglycemia, its symptoms, and treatment options.

Hypoketotic hypoglycemia affects your blood sugar (glucose) level and energy. The term hypoketotic refers to a low level of ketones in the blood. When your body breaks down fats for energy, it produces ketones.

Usually, when you’re not eating regular meals (fasting) or when you’re ill, your body releases fatty acids from fat tissue to provide energy. When this process is interrupted in some way, your body can’t get the energy it needs.

As a result, both blood glucose and energy levels drop, causing hypoketotic hypoglycemia.

Symptoms of hypoketotic hypoglycemia are due to low glucose levels in the blood.

They include:

If hypoglycemia is left untreated, symptoms may become more severe and may include:

  • vision changes
  • weakness
  • confusion
  • clumsiness
  • seizures
  • fainting

Hypoglycemia is primarily seen in people with diabetes.

But it may also affect people with other health issues, including kidney disease or severe infection. This may also be a side effect of certain medications.

With any form of hypoglycemia, low blood sugar is what causes symptoms.

Hypoketotic hypoglycemia involves both low blood glucose and low ketones.

It usually presents in the first 2 years of life and is typically linked to genetically inherited metabolic disorders. The symptoms may be nearly identical, but both the blood sugar and ketone levels are low.

Hypoketotic hypoglycemia is caused by issues with metabolism called FAOD.

When blood glucose is low due to fasting or illness, your body uses fats for energy. If you have FAOD, your body is unable to break down fats for energy. In this situation, both blood sugar and energy levels get low.

The most common type of FAOD is medium-chain acyl-CoA dehydrogenase deficiency (MCADD). An estimated 1 in 50,000 people are born with this condition. It’s inherited during conception in an autosomal recessive manner.

Other types of FAOD include:

  • very long-chain acyl-CoA dehydrogenase deficiency
  • long-chain 3-hydroxy acyl-CoA dehydrogenase deficiency
  • trifunctional protein deficiency

Carnitine transport disorders may also cause hypoketotic hypoglycemia. The substance carnitine is necessary for producing energy from fatty acids. When this process is interrupted, you may experience symptoms of hypoketotic hypoglycemia.

These disorders include:

  • carnitine palmitoyltransferase type 1 deficiency
  • carnitine-acylcarnitine translocase deficiency
  • carnitine palmitoyltransferase type 2 deficiency
  • carnitine transporter deficiency

Treating hypoketotic hypoglycemia involves treating the underlying condition that causes it. This means treating the FAOD, which causes the body’s inability to convert fats to energy.

The most common cause of FAOD is MCADD. The primary treatment goal for MCADD is avoiding fasting. Regular food intake may help prevent hypoglycemia by keeping blood sugar levels steady.

According to experts, treatment involves:

  • avoiding foods and formulas that contain medium-chain fatty acids
  • working with specialists on nutrition balance to help prevent too much weight gain or type 2 diabetes
  • monitoring carbohydrate intake closely during illness, especially fever
  • wearing a medical alert bracelet in case of emergency so doctors can treat hypoglycemia in a timely manner

While controversial, experts say that supplementation with L-carnitine is another possible treatment option. Your doctor can help determine whether supplementation may be helpful.

To treat acute hypoglycemic episodes, it’s advisable to consume simple carbohydrates.

If you can’t eat food by mouth, glucagon can be administered. In the hospital, you may receive intravenous (IV) glucose (pure, fast-acting sugar) to boost your blood sugar.

Hypoketotic hypoglycemia is a potential complication of FAODs. In many countries and regions, newborns are screened for FAODs. This helps identify babies born with this condition before they experience a metabolic crisis.

Symptoms usually occur in the first couple years of life and can become life threatening if not treated.

Treatment depends on the specific underlying cause. Many people see improvement by avoiding fasting and working closely with an endocrinologist and dietitian to help prevent associated complications.

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