Monogenic diabetes

There are two types of monogenic diabetes – Neonatal and MODY. 

In some cases, monogenic diabetes can be managed with specific tablets and in other cases insulin treatment is needed. It’s important to see a specialist paediatric endocrinologist who understands this rare form of diabetes so that the right treatment can be put in place. 

Neonatal Diabetes

Neonatal Diabetes is a form of diabetes that is diagnosed under the age of six months, but it is not an autoimmune condition like type 1 diabetes (where the insulin producing cells in the pancreas are destroyed by the body).

It is caused by a change in the gene that affects insulin production which means blood glucose levels rise. Some people with neonatal diabetes may also have some developmental delay (eg muscle weakness, learning difficulties) and epilepsy.

There are two types of neonatal diabetes – transient (doesn’t last forever and usually resolves before the age of 12 months but can come back later in life, generally in the teenage years) or permanent which lasts forever.

Children with Neonatal diabetes are best managed by a specialist paediatric endocrinologist.

MODY (Maturity Onset Diabetes of the Young)

About 2% of people with diabetes have MODY which is caused by a change (mutation) in a single gene. If a parent has this mutated gene, any child they have, has a 50 per cent chance of inheriting it from them. If a child does inherit this mutation, they will usually develop MODY before the age of 25, regardless of their weight, lifestyle, ethnic group etc.

Additional genetic tests need to be considered in the following situation:

• atypical type 1 diabetes
• no history of DKA
• atypical type 2 diabetes
• under 35yo
• no obesity, hypertension, dyslipidaemia or PCOS
• strong family history of type 2 diabetes

People with suspected MODY need to be referred to an endocrinologist for specialist assessment and management advice.