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Endocrine- Diabetes > Other Types of Diabetes > Flashcards

Other Types of Diabetes Flashcards

1
Q

MODY is most likely to be diagnosed as what other type of diabetes?

A

Type 1

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2
Q

Where is the defect in MODY?

A

The pancreatic beta cell (primarily insulin secretion)

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3
Q

Is there a family history in MODY?

A

Very strong (usually 3 generations)

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4
Q

How is MODY inherited?

A

Autosomal dominant

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5
Q

Is MODY insulin dependent?

A

No

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6
Q

What is the age of onset in MODY?

A

Usually < 25

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7
Q

What is the most common type of MODY?

A

Transcription factor defects

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8
Q

What is MODYX?

A

When you are convinced a patient has MODY but there is no genetic cause

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9
Q

What is MODY2?

A

Problem with the glucokinase enzyme

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10
Q

What transcription factors are most commonly defective in MODY?

A

HNF1 alpha (MODY3), HNF4 alpha (MODY1)

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11
Q

Is MODY polygenic or monogenic?

A

Monogenic

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12
Q

What is the difference in the times of onset of glucokinase MODY and transcription factor MODY?

A

Glucokinase = from birth // Transcription factor = adolescence/early adulthood

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13
Q

Which type of MODY has progressive hyperglycaemia?

A

Transcription factor (glucokinase is stable)

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14
Q

What is the treatment for glucokinase MODY?

A

Diet control

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15
Q

What treatment options are there for transcription factor MODY?

A

Diet control, oral hypoglycaemic (sulphnylurea), insulin

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16
Q

In which type of MODY are complications more likely to occur?

A

Transcription factor

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17
Q

Glucokinase MODY can give a slightly raised HbA1c- when is this relevant?

A

Pregnancy

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18
Q

What is a good test to tell MODY from type 1 diabetes?

A

C-peptide

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19
Q

Will patients with MODY have C-peptide?

A

Yes (insulin is produced, it is not secreted so well)

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20
Q

MODY is a glucose sensing defect, what does this mean?

A

The blood glucose threshold for insulin is increased // normal BG will always be raised in MODY

21
Q

What is the role of glucokinase?

A

Glucose –> Glucose-1-phosphate

22
Q

Patients with mutations in glucokinase (glucokinase MODY) will have abnormal function of what?

A

GLUT2 transporter

23
Q

What is the normal blood glucose for someone with MODY?

A

7mmol/l (around 5 is normal)

24
Q

HNF, which is implicated in transcription factor MODY, is important for what?

A

Pancreatic foetal development and neogenesis // Regulate beta cell function- including GLU2 and insulin secretion

25
Q

Patients with transcription factor MODY are very receptive to treatment with what?

A

Sulphonylureas

26
Q

Children who are diagnosed with diabetes aged < 6 months are most likely to have what?

A

MODY

27
Q

What is transient neonatal diabetes?

A

Usually diagnosed < 1 week and is not permanent

28
Q

What is permanent neonatal diabetes?

A

Appears within the first 6 months of life and stays

29
Q

Patients with permanent neonatal diabetes are insensitive to what? Why?

A

ATP- there are mutations in the K+ channel

30
Q

What is the effect of the K+ channel being unable to shut in permanent neonatal diabetes?

A

No insulin is produced

31
Q

What is used as treatment for neonatal diabetes?

A

Sulphonylureas

32
Q

How is a diagnose of LADA established?

A

Presence of elevated pancreatic auto-antibodies // Do not require insulin at diagnosis

33
Q

When does LADA usually present?

A

25-40

34
Q

Which sex is LADA more common in? Are individuals usually overweight?

A

Male // Not necessarily overweight

35
Q

What is LADA often associated with?

A

Other autoimmune conditions

36
Q

What is gestational diabetes?

A

When diabetes develops in pregnancy and resolves post-natally

37
Q

There is an increased risk of what with gestational diabetes?

A

Type 2 diabetes in the future

38
Q

When during pregnancy is gestational diabetes most common?

A

Third trimester

39
Q

What is the reason for gestational diabetes?

A

Pregnancy hormones decrease the action of insulin at its receptors (insulin resistance)

40
Q

How does drug related diabetes occur?

A

Some medications will increase BG and this leads to insulin resistance

41
Q

In what condition do more than 25% of patients have diabetes aged 20 years?

A

CF

42
Q

When does screening for diabetes begin in CF? Which types of CF are most likely to cause diabetes?

A

Aged 10 // Those with more severe mutations

43
Q

What is the preferred treatment for diabetes in CF?

A

Insulin

44
Q

How is Wolfram Syndrome inherited?

A

Autosomal recessive

45
Q

What conditions does Wolfram Syndrome consist of?

A

DM, DI, optic atrophy, deafness, neurological abnormalities

46
Q

How is Barder-Biedl Syndrome inherited?

A

Autosomal recessive

47
Q

How do patients with Barder-Biedl Syndrome look?

A

Very obese

48
Q

What conditions are associated with Barder-Biedl Syndrome?

A

Diabetes, mental retardation, hearing and visual impairment, hypogonadism, polydactyly

49
Q

Who does Barder-Biedl Syndrome occur in?

A

Children whose parents are related

Endocrine- Diabetes (11 decks)

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