MODY Flashcards
how strong is family history for MODY?
3 generation FH
what kind of inheritance is MODY?
autosomal dominant
age of onset of MODY?
<25
is MODY insulin dependent?
no
name the 3 methods that MODY can be acquired from?
glucokinase
HNF1 receptors
unknown
what happens in MODY from a glucokinase mutation?
glucokinase is less responsive to glucose so GLUT2 function altered = hyperglycaemia but because of normal insulin production loads is made so get hypo
what will OGTT of someone with a glucokinase mutation look like?
normal
what does the HbA1C of someone with a glucokinase mutation look like?
raised
when do glucokinase mutations arise in MODY?
onset at birth
how is a glucokinase mutation treated?
diet modifications, NO INSULIN
when do HNF1 mutations arise?
adolesence/young adult
is hyperglycaemia progressive in HNF1 or glucokinase mutations?
HNF1
how is HNF1 mutations treated?
1/3 diet, 1/3 OHA, 1/3 insulin
most common cause of MODY in UK?
HNF1 alpha
how can you tell the difference between MODY and diabetes via test
C peptide levels should be normal in MODY
how should neonatal diabetes be treated?
insulin within first 3 months of life
what are the 2 types of neonatal diabetes?
transient
permanent
which type of neonatal diabetes has the fastest diagnosistic time?
transient
which type of neonatal diabetes resolves?
transient
when does transient neonatal diabetes resolve?
12 weeks average
how is permanent neonatal diabetes treated?
lifelong insulin
how is transient neonatal diabetes treated?
insulin for 12 weeks then stop
what drug can also be given to HNF1 patients?
SUR
