Congenital Diabetes: Know What Causes Diabetes In Infants And How It Can Be Treated

The term "infant" is typically applied to young children under one year of age; however, definitions may vary and may include children up to two years of age. When a human child learns to walk, the term "toddler" may be used instead. Neonatal diabetes is a rare form of diabetes that is usually diagnosed in children under 6 months of age. Neonatal diabetes mellitus (also termed congenital diabetes, or diabetes of infancy) is highly likely to be due to an underlying monogenic defect when it occurs under 6 months of age.

Early recognition and urgent genetic testing are important for predicting the clinical course and raising awareness of possible additional features, and in many cases these are essential for guiding appropriate and cost-effective treatment.

Very rarely, babies are born with diabetes. This is called neonatal diabetes and is caused by a problem with the genes. Neonatal diabetes can disappear by the time the child is 12 months old, but the diabetes usually returns later in life.

There are two main types of neonatal diabetes:

• Transient Neonatal Diabetes Mellitus
• Permanent Neonatal Diabetes Mellitus

Transient neonatal diabetes mellitus (TNDB) is a type of diabetes that appears within the first few weeks of life but is transient; affected infants go into remission within a few months, with possible relapse to permanent diabetes in adolescence or adulthood.

Approximately 70% of cases are caused by the over activity of certain genes. Genetic causes include mutations in the KCNJ11 and ABCC8 genes, which usually cause permanent neonatal diabetes and these mutations can be assessed in the laboratory quiet early. Treatment may include rehydration and intravenous insulin at the time of diagnosis, followed by subcutaneous insulin.

Affected infants with TNDB have hyperglycaemia and an excessive loss of fluids (dehydration), usually beginning in the first week of life. Signs and symptoms of this form of diabetes are transient, which means that they gradually lessen over time and generally disappear between the ages of 3 months and 18 months. Diabetes may recur, however, especially during childhood illnesses or pregnancy.

Permanent neonatal diabetes mellitus (PNDB) is a type of diabetes that appears within the first 6 months of life and persists throughout life. Affected individuals have slow growth before birth followed by hyperglycaemia, dehydration and failure to thrive in infancy.

DEND syndrome is a very rare, generally severe form of neonatal diabetes mellitus (NDM, see this term) characterized by a triad of developmental delay, epilepsy, and neonatal diabetes.

Neonatal diabetes will usually either be treated with a drug called glibenclamide or with insulin. If neonatal diabetes is transient, it won't require treatment during the years in which it is resolved. However, the condition should be monitored for reappearance of diabetes in adolescence and later years.

Children under three to five years of age with type I diabetes comprise a small proportion of all those with this disorder: less than 1% of all children are diagnosed in the first year of life, and less than 2% of children attending large paediatric diabetes centres fall into the under three-year age group. Nevertheless, recent experience, backed up by epidemiological studies, suggests a significant trend towards diagnosis of type I diabetes at a younger age

Infant and toddlers with diabetes pose a series of important challenges to health care professionals. First, at the time of diagnosis children often do not have classical symptoms and secondly it is difficult to set up a therapeutic regimen for them. When a child has diabetes, it affects the entire family. Siblings might resent the extra attention that a child with diabetes gets, as well as sacrifices (like eating healthier foods at family meals or going along to doctor appointments) made for the sibling. And sometimes they're the target of anger and resentment because they don't have to deal with the issues that the child with diabetes faces.

• Acknowledge your child's feelings.
• Encourage active health care management.
• Build independence.
• Help kids find their strengths.

(Dr S K Wangnoo, Head, Apollo Centre for Obesity, Diabetes and Endocrinology, Indraprastha Apollo Hospital)

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