MODY

Question 1

How is MODY inherited?

Answer 1

autosomal dominant

Question 2

Age of onset of MODY

Answer 2

usually before 25 years of age,

Question 3

What are the 5 most common types of MODY?

Answer 3

MODY1 (HNF4A mutation)
MODY2 (GCK mutation)
MODY3 (HNF1A mutation)
MODY4 (PDX1)
MODY5 (HNF1B)

Question 4

Which of the 5 most common types of MODY is most prevalent?

Answer 4

MODY3 (HNF1A mutation) ~60%

MODY2 (GCK mutation) ~20%

Question 5

How do patients normally present with MODY?

Answer 5

• mild non-ketotic hyperglycemia - incidental finding/ found on screening
• may be found during pregnancy
• don’t usually have DKA unless under severe stress
• normal weight
• no signs of insulin resistance

Question 6

Describe the difference in diabetic complication between different MODY subtypes

Answer 6

MODY2 = mild fasting hyperglycemia
> rarely develop complications,

MODY3 or MODY1 may have progressive hyperglycemia
=> higher risk for complications

Question 7

How is MODY treated?

Answer 7

MODY2 - no tx req’d as only mild hyperglycaemia

MODY3 - low-dose sulphonylureas

*sometimes patients may need insulin during pregnancy