MODY - monogenic diabetes

Question 1

What does MODY stand for?

Answer 1

Maturity Onset Diabetes of the Young

Question 2

What are the 2 most common mutations in MODY?

Answer 2

HNF1-alpha

Glucokinase

Question 3

What effect does the Glucokinase mutation have on blood glucose levels?

Answer 3

Increase the baseline point to about 7 mmol/L

• insulin is still produced
• glucose levels are well controlled at this slightly higher level

Question 4

What are the effects on blood glucose of HNF transcription factor mutations?

Answer 4

Progressive hyperglycaemia with age

Question 5

How is MODY with a glucokinase mutation treated?

Answer 5

Diet only

Question 6

What drugs work particularly well in MODY patients with HNF1-alpha mutations?

Answer 6

Sulfonylureas (Gliclazide)

Question 7

C-peptide is present in Type 1 diabetes (T/F)

Answer 7

False

-no endogenous diabetes produced

Question 8

What is the mode of inheritance of MODY?

Answer 8

autosomal dominant

Question 9

What is the cause of neonatal diabetes? What is the appropriate treatment?

Answer 9

potassium channel gene mutations

Sunlfonylureas (Gliclazide)