Beyond MODY: Other Forms

MODY dominates the conversation about monogenic diabetes, but more than 20 genes have been identified as causes, and the group is broader than MODY alone.

Neonatal diabetes appears in the first six to twelve months of life. It is rarer than MODY, affecting around 1 in every 90,000 babies, and it can be permanent or transient — temporary, resolving in infancy, sometimes returning years later. It has its own category on this site because the reader there is a parent rather than the patient.

Syndromic forms are those where diabetes comes alongside features in other organs. The clearest example within MODY is HNF1B, which affects the kidneys as well as the pancreas, often producing cystic kidney disease.

Recessive forms exist too, needing an altered gene copy from both parents rather than one. These are rarer and tend to appear earlier and more severely.

There are also forms involving genes affecting insulin action rather than insulin production, and mitochondrial forms where diabetes travels with hearing loss.

The practical point is not to memorise this list. It is that "monogenic diabetes" is a group rather than a single condition, and the specific gene is what determines your treatment, your outlook, and what your relatives should be told.

If you have a confirmed genetic result, the gene name on it is the useful piece of information — not the umbrella term.