MODY Flashcards

1
Q

What kind of genetic inheritance does MODY have?

A

Autosomal dominant

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2
Q

What kind of genetic inheritance does MODY have?

A

Autosomal dominant

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3
Q

Is MODY IDDM or NIDDM?

A

Non-insulin dependent

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4
Q

At what age is onset?

A

Less than 25

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5
Q

Which transcription factors are prominently modified in MODY?

A

HNFalpha (in majority)

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6
Q

In the minority of MODY cases, where is the mutation?

A

Glucokinase

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7
Q

What is the onset of MODY in glucokinase mutated MODY?

A

At birth.

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8
Q

How is glucokinase mutated MODY managed?

A

Diet treatment

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9
Q

How is transcription factor mutated MODY managed?

A

1/3 diet, 1/3 OHA, 1/3 insulin

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10
Q

What class of drug, other than insulin, is used to treat HNFa MODY?

A

Sulphonylureas

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11
Q

Give an example of an SU

A

Gliclazide

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12
Q

Is MODY IDDM or NIDDM?

A

Non-insulin dependent

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13
Q

At what age is onset?

A
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14
Q

Which transcription factors are prominently modified in MODY?

A

HNFalpha (in majority)

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15
Q

In the minority of MODY cases, where is the mutation?

A

Glucokinase

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16
Q

What is the onset of MODY in glucokinase mutated MODY?

A

At birth.

17
Q

How is glucokinase mutated MODY managed?

A

Diet treatment

18
Q

How is transcription factor mutated MODY managed?

A

1/3 diet, 1/3 OHA, 1/3 insulin

19
Q

What class of drug, other than insulin, is used to treat HNFa MODY?

A

Sulphonylureas (small dosages)

20
Q

Give an example of an SU

A

Gliclazide

21
Q

What is the follow up for a GCK MODY?

A

No follow up. Not on any medication

22
Q

What is transient neonatal diabetes?

A

Diagnosed in less than 1 week

resolves in 3 months

23
Q

What is the defect in permanent neonatal diabetes?

A

K channels

24
Q

Where do sulphonylureas work on?

A

SUR1 channels on K channels