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1

MODY inheritence

Autosomal dominant

2

Becker inheritence

X-linked recessive

3

Most common form of MODY?

HNF1 alpha

4

HNF1 MODY?

Most common form, lowers the amount of insulin made by the pacnreas

5

Onset, treatment and complications of glucokinase MODY?

Onset at birth
Stable hyperglycaemia
Diet treatment
Complications rare

6

Onset, treatment and complications of transcription factor mutation MODY? (e.g. HNF1 alpha)

Adolescence/young adult onset
Progressive hyperglycaemia 1/3 diet, 1/3 OHA, 1/3 Insulin
Complications frequent

7

Treatment proven to work really well in HNF1 alpha MODY?

Gliclazide

8

Treatment for neontatal diabetes?

Requires insulin treatment within first 3 months of life
(remember you have transient and permanent neonatal diabetes though)

9

Transient neonatal diabetes

Diabetes usually diagnosed < 1 week
Resolves median 12 weeks
Stop insulin

10

Permanent neonatal diabetes

Diabetes usually diagnosed 0 - 6 weeks
Lifelong treatment with insulin

11

This type of drug binds to the KATP channel in the pancreatic beta cell?

Sulphonylurea
(stops hyperpolarisation = cell becomes depolarised = calcium enters = insulin released)

12

Mutations in Kir6.2 associated with what?

Associated with PNDM (permanent neonatal diabetes)

13

Neonatal diabetes can be caused by mutations in which channel?

Potassium channel mutations