Adrenals and Endocrine Pancreas Flashcards

1
Q

What conditions can cause adrenal hyperfunction?

A
  • Cushing’s syndrome
  • Conn’s syndrome
  • Adrenogenital syndrome and congenital adrenal hyperplasia
  • Adrenocortical neoplasms
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2
Q

Is cushing’s syndrome more common in males or females?

A

Females

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3
Q

What are features of Cushing’s syndrome?

A
  • Muscle catabolism
  • Fat
  • Abnormal collagen maturation (can cause stria)
  • Hypertension
  • Osteoperosis (absorbing more Ca2+)
  • Impaired glucose tolerance
  • Hirsutism or gyaenacomastia
  • Depression / psychosis
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4
Q

What is hirsutism?

A

Abnormal male-like hair growth on women

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5
Q

What fraction of Cushing’s syndromes are caused by a primary adrenal neoplasm?

A

~ 1/6

- 50:50 benign to malignant

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6
Q

What fraction of Cushing’s syndromes are related to ectopic ACTH production?

A

~ 1/6

  • Ectopic ACTH or related peptide
  • Often part of a paraneoplastic syndrome e.g SCLC
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7
Q

What are the levels of K+ in Conn’s syndrome?

A

Low (hypokalaemic)

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8
Q

WHat are the features of Conn’s syndrome?

A
  • Hypokalaemia
  • Muscle cramps and weakness
  • Metabolic alkalosis
  • High aldosterone
  • Low renin
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9
Q

What gender is affected more by Conn’s syndrome?

A

Females (4:1)

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10
Q

What percentage of Conn’s syndrome patients have an adrenal adenoma?

A

80%

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11
Q

What percentage of Conn’s syndrome patients have bilateral hyperplasia of zona glomerulosa but unkown cause?

A

20%

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12
Q

What enzyme deficiencies can cause congenital adrenal hyperplasia?

A
  • 21 hydroxylase deficiency (CYP21)

- 11-Beta Hydroxylase Deficiency

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13
Q

How common is congenital adrenal hyperplasia?

A

1 in 15000

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14
Q

Describe the mechanism of congenital adrenal hyperplasia?

A

Problems in metabolism where one does not get the negative feedback to stop ACTH so you get a drive and hyperplasia

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15
Q

A malignant tumour in the adrenal gland can come from what tissues?

A
  • Lung
  • Breast
  • Kidney
  • Colon
  • Melanoma
  • Lymphoma
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16
Q

How common are adrenal adenomas on autopsy?

A

Very common - though to be as high as 5%

  • So often not functional therefore not found in life
  • Low malignant potential
  • Only treated if functional or malignant
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17
Q

Adrenal carcinoma

A
  • Rare
  • Poor outlook
  • More often secrete sex steriods (Hirsutism)
  • Can be quite large (inches)
  • Invasive - other organs, fat
18
Q

What molecular changes can take place in an adrenal carcinoma?

A
  • Proliferation markers increased
  • Mutant p53 protein increased
  • IGF II increased
  • EGFR increased
19
Q

What gene mutations may cuase adrenal carcinoma?

A
  • p53

- MEN1 (multiple endocrine neoplasia)

20
Q

What are molecular changes useful for?

A
  • Identifying familial syndrome

- Possibly distinguishing adenoma from carcinoma

21
Q

What cells do phaeochromocytomas develop from?

A

Chromaffin cells

22
Q

How does phaeochromocytoma usually present?

A
  • Intermittent production of catecholamines
  • Hypertension
  • Sweating
  • Collapse
  • Glycosuria
  • When sympathetic NS activated - symptoms appear, overload of catecholamines
23
Q

What percentage of phaeochromocytomas are familail?

A

20%, may be opart of MEN

24
Q

What percentage of phaeochromocytomas are malignant?

A

10% (there is a spectrum of benign to frankly malignant)

25
Q

What is neuroblastoma?

A
  • Rare tumour
  • Children
  • Can appear late
  • Kidney
  • N-myc amplification or adrenal site worse prognosis
26
Q

What can the causes of acute adrenal hypofunction be?

A
  • Meningococcal septicaemia
  • Disseminated intravascular coagulation - causes coagulation in organs using upclotting factors leading to haemorrhages and purpuric rashes
27
Q

What What can the causes of chronic adrenal hypofunction be?

A
  • Addison’s disease (autoimmune adrenalitis)
  • Metastasis
  • Amyloid
  • TB
28
Q

WHat are some symptoms of Addison’s disease?

A
  • Lethargy
  • Weakness
  • Anorexia
  • Pigmentation of skin and mucus membranes (ACTH build up)
29
Q

What can the causes of Addison’s disease be?

A
  • Autoimmune adrenalitis > 75%
  • TB
  • Amyloid
  • Metastasis
30
Q

What are some associations of Addison’s disease?

A
  • Vitiligo

- Diabetes

31
Q

How can type 2 Diabetes cause B cells not to produce insulin ?

A

Amyloid deposited late in islets causing loss in islets

32
Q

How much concordance is there of type 1 diabetes in mono-zygotic twins?

A

40%

33
Q

How common is type 1 diabetes?

A

1 in 500

34
Q

How can a virus ultimately lead to type 1 diabetes?`

A
  • Viral infection of B cell
  • Interferon-alpha expression by B cell
  • Islet inflammation
  • Class II MHC expression by B cell
  • Induction of autoimmunity to B cell
  • B cells destroyed
  • Diabetes
35
Q

What are other less common causes of diabetes?

A
  • Pancreatitis
  • Cystic fibrosis
  • Tumour
36
Q

What can a G cell Gastrinoma cause?

A

Zollinger-Ellison syndrome

37
Q

What can a vipoma (vasoactive intestinal peptide) cause?

A

Verner-Morrison SYndrome (pancreatic cholera)

38
Q

What 2 genes can cause multiple different endocrine tumours?

A
  • Multiple endocrine neoplasia 1 (MEN1)
  • MEN2
    Can be spontaneous or familial
39
Q

What happens to glucose levels in acute pancreatitis?

A

Falls - hypoglycaemia

40
Q

What can happen to the pancreas [macroscopicaly] in pancreatitis?

A
  • Calcification
  • Fibrous tissue
    Primarrily affects exocrine
41
Q

How can chronic pancreatitis lead to insulin insufficiency (type 1)?

A

Fibrotic destruction of islets

42
Q

What are different neuroendocrine neoplasms?

A
  • Glucagonoma
  • Somatostatinoma
  • Insulinoma
  • Vipoma
  • Gastrinoma
    Varibale malignant potential
    Stomach and SI most malignant