MODY Flashcards
What is MODY?
Maturity onset diabetes of the young - a group of hereditary diabetes caused by a defect of insulin production
Which pattern of inheritance is MODY?
Autosomal dominant
Is MODY insulin dependant?
No
What are the two main kinds of MODY?
Glucokinase mutations
Transcription factor mutations (HNF-1a, HNF-1b, HNF-4a)
When is the usual onset of glucokinase mutation MODY?
Birth
What is the progression of glucokinase mutation MODY?
Stable hyperglycaemia, complications rare
What is the progression of transcription factor mutation MODY?
Progressive hyperglycaemia, complications frequent
What is the most common kind of MODY?
Transcription factor MODY, particularly HNF1a
When does transcription factor mutation MODY present?
Late teens/early adulthood
How is glucokinase mutation MODY treated?
Diet
How is transcription factor mutation MODY treated?
1/3 diet, 1/3 oral hyperglycaemic agents, 1/3 Insulin
How is neonatal diabetes treated?
Immediate insulin therapy
What are the two types of neonatal diabetes?
Transient and permanent
When does transient neonatal diabetes tend to resolve?
~12 weeks
