Babies born with congenital hyperinsulinism (CHI) are at risk of suffering from permanent brain damage

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Babies born with congenital hyperinsulinism (CHI) are at risk of suffering from permanent brain damage and life-long disability.

Yet some will go on to suffer more severely than others as a result of their disease profile, report the researchers in an article published in Frontiers in Endocrinology.

The research team have found that it is possible to predict when and how the disease may affect the child in the long-term.

“One of the problems facing clinicians is that it is really difficult to predict which babies will have problems after surgical treatment.


What is hypoglycemia in a newborn baby?

Hypoglycemia is when the level of sugar (glucose) in the blood is too low. Glucose is the main source of fuel for the brain and the body. In a newborn baby, low blood sugar can happen for many reasons. It can cause problems such as shakiness, blue tint to the skin, and breathing and feeding problems.

What causes hypoglycemia in a newborn baby?

Hypoglycemia can be caused by conditions such as:

  • Poor nutrition for the mother during pregnancy
  • Making too much insulin because the mother has poorly controlled diabetes
  • Incompatible blood types of mother and baby (severe hemolytic disease of the newborn)
  • More insulin in the baby’s stool for other reasons, such as a tumor of the pancreas
  • Birth defects
  • Congenital metabolic diseases or hormone deficiencies. Some of these run in families.
  • Not enough oxygen at birth (birth asphyxia)
  • Liver disease
  • Infection

Which newborns are at risk for hypoglycemia?

Babies are more likely to have hypoglycemia include:

  • Babies born to mothers with diabetes
  • Babies who are small for gestational age or growth-restricted
  • Preterm babies, especially those with low birth weights
  • Babies born under significant stress
  • Babies with mothers treated with certain medicines such as terbutaline
  • Babies who are large for their gestational age

What are the symptoms of hypoglycemia in a newborn baby?

Signs of low blood sugar may not be obvious in newborn babies. The most common signs include:

  • Shakiness
  • Blue tint to skin and lips (cyanosis)
  • Stopping breathing (apnea)
  • Low body temperature (hypothermia)
  • Floppy muscles (poor muscle tone)
  • Not interested in feeding
  • Lack of movement and energy (lethargy)
  • Seizures

The signs of hypoglycemia can be like other health conditions. Make sure your child sees his or her healthcare provider for a diagnosis.

How is hypoglycemia in a newborn baby diagnosed?

A simple blood test for blood glucose levels can diagnose the problem.

How is hypoglycemia in a newborn baby treated?

Treatment will depend on your baby’s gestational age and overall health. Treatment includes giving the baby a fast-acting source of glucose. This may be as simple as a glucose and water mixture or formula as an early feeding. Or your baby may need glucose given through an IV. The baby’s blood glucose levels are checked after treatment to see if the hypoglycemia occurs again.

What are possible complications of hypoglycemia in a newborn baby?

The brain needs blood glucose to function. Not enough glucose can harm the brain’s ability to function. Severe or long-lasting hypoglycemia may cause seizures and serious brain injury.

What can I do to prevent hypoglycemia in my newborn baby?

In many cases, there may not be a way to prevent hypoglycemia in a newborn baby. For a baby with risk factors, healthcare providers will need to watch carefully for the signs and treat as soon as possible. Mothers with diabetes should keep their blood glucose levels in a normal range during pregnancy. This may help lower the risk for their baby.

When should I call my child’s healthcare provider?

Call your baby’s healthcare provider right away you see signs of low blood sugar in your baby. Give your baby formula or a glucose and water mixture, if advised.

Key points about hypoglycemia in a newborn baby

  • Hypoglycemia is a condition in which the level of glucose in the blood is lower than normal.
  • A baby is at risk if he or she has a mother with diabetes, is preterm, or is large.
  • If your baby has signs of hypoglycemia, give him or her formula or glucose and water mixture.

Next steps

Tips to help you get the most from a visit to your child’s healthcare provider:

  • Know the reason for the visit and what you want to happen.
  • Before your visit, write down questions you want answered.
  • At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you for your child.
  • Know why a new medicine or treatment is prescribed and how it will help your child. Also know what the side effects are.
  • Ask if your child’s condition can be treated in other ways.
  • Know why a test or procedure is recommended and what the results could mean.
  • Know what to expect if your child does not take the medicine or have the test or procedure.
  • If your child has a follow-up appointment, write down the date, time, and purpose for that visit.
  • Know how you can contact your child’s provider after office hours. This is important if your child becomes ill and you have questions or need advice.

Image from pancreas of a healthy child


Image from a pancreas of a child with diffuse hyperinulinism

New data give some important clues that will help clinicians to know how much extra care each baby is likely to need”, explains Dr. Karen Cosgrove, a member of the research team from The University of Manchester.

Although generally rare, affecting just 1 in 50,000 children in the UK, CHI can be as common as cystic fibrosis (1 in 2,500) in children of European Jewish descent or when born into communities where cousins marry.

Up until now, scientists have understood that there are two main subtypes of the disease- known as diffuse and focal.

Diffuse CHI affects the entire pancreas, whilst focal CHI affects just one area of the organ, forming a focal lesion.

The team from Manchester now understand that focal CHI can be further categorised into two types- spreading focal lesions and isolated focal lesions.

Spreading focal lesions are generally larger and spread outwards into areas of healthy cells.

Isolated focal lesions, on the other hand, have a capsule around them which keeps the diseased cells separate from healthy cells.

The research team investigated the cases of 25 infants with focal CHI to see how the two types of lesions influenced their long-term outcomes.

The researchers found that babies with spreading focal lesions suffered more severely from the disease and were diagnosed earlier.

These infants were more likely to suffer brain damage which permanently affected their development, learning and behaviour.

In contrast, in infants with isolated lesions, the disease was diagnosed later and surgery to remove the lesion was less complicated.

These new data help to explain why new-born babies diagnosed with the same disease may go on to have very different outcomes and could influence the way clinicians choose to manage each new case of CHI.

“Although the impact of these findings is limited to centres with surgical resources, for those centres the data is immediately applicable and translatable clinically” explains Professor Mark Dunne from The University of Manchester and lead author on the study.

More information: Ross J. Craigie et al. Clinical Diversity in Focal Congenital Hyperinsulinism in Infancy Correlates With Histological Heterogeneity of Islet Cell Lesions, Frontiers in Endocrinology (2018). DOI: 10.3389/fendo.2018.00619
Provided by University of Manchester

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