Pituitary disease Flashcards

1
Q

1) What is acromegaly?
2) What is the most common cause of acromegaly?
3) In 25-30% of cases of acromegaly, what is co-secreted?

A

1) The clinical manifestation of the presence/ production of excess growth hormone.
2) A pituitary adenoma which is hormone secreting.
3) Prolactin is co-secreted in 25-30% cases.

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2
Q

1) What is a rare cause of acromegaly?

2) Describe how a pituitary adenoma can affect a patient’s eyesight.

A

1) Rarely, acromegaly can be caused secondary to a cancer such as lung/ pancreatic cancer that secretes excessive GHRH or GH ectopic ally.
2) The optic chasm sits just above the pituitary gland. Pressure on the optic chasm from a pituitary tumour can cause bitemporal hemianopia.

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3
Q

1) How does growth hormone stimulate bone and soft tissue growth?
2) What causes gigantism?

A

1) GH stimulates bone and soft tissue growth through IGF-1.

2) Excess GH before epiphyseal growth plate fusion causes gigantism.

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4
Q

How do pituitary adenomas arise?

A

From the proliferation of single, mutated pituitary cells where genetic alterations promote cellular growth rate,

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5
Q

Name 3 things that patients may complain of when presenting with potential acromegaly.

A
Rings not fitting
Old shoes not fitting
Developing a wonky bite
Developing curly hair
Putting on weight
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6
Q

1) Describe the onset of acromegaly.

2) What 3 categories can presenting features of acromegaly fit into?

A

1) There is an insidious onset often resulting in diagnostic delay and signs can predate diagnosis by 4 years or more.
2) Signs/ symptoms of a space occupying lesion, tissue overgrowth or organ dysfunction.

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7
Q

State 4 signs or symptoms of a space occupying lesion which could be presenting features in patients with acromegaly.

A

Headaches
Bitemporal hemianopia
Cranial nerve palsies
Hypopituitarism (if pituitary stalk compression occurs)

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8
Q

State 5 possible signs or symptoms of tissue overgrowth which could be presenting features in patients with acromegaly.

A
Prominent forehead (frontal bossing)
Large nose
Large hands and feet
Large tongue (macroglossia)
Prominent, protruding jaw (prognathism)
Decreased libido and alterations in sexual functioning
Arthralgia/ arthritis
Acanthosis ingrains
Coarse skin and skin thickening
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9
Q

State the blood tests you might order for a patient with suspected acromegaly.

A
IGF-1 (elevated)
Cortisol (may be low)
Calcium/ phosphate (may be high)
FBC
U&Es
Creatinine
LFTs
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10
Q

Why can cortisol levels be low in acromegaly?

A

Because GH is an important regulator of conversion of cortisone to cortisol and its action is to inhibit this conversion, so excess GH = low cortisol levels.

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11
Q

What is the standard method used to diagnose acromegaly and how is the diagnosis made?

A

The oral glucose tolerance test is used if baseline IGF-1 values are abnormal. It is the standard method to confirm an acromegaly diagnosis.

If the lowest GH value during the OGTT is >1mcg/L then acromegaly is confirmed.

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12
Q

Why is the oral glucose tolerance test used to confirm acromegaly diagnosis?

A

Physiological GH secretion is inhibited by hyperglycaemia, but in acromegaly GH secretion is autonomous and does not suppress with hyperglycaemia and may paradoxically rise with hyperglycaemia.

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13
Q

Suggest 5 other investigations aside from blood tests or the oral glucose tolerance test which may be required in a patient with acromegaly.

A
CXR
CT
MRI of pituitary fossa
ECG and ECHO
Visual field testing
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14
Q

Describe the management aims for patients with acromegaly.

A

To control the symptoms caused by the local effects of the tumour and those due to the excess hormone production, and to normalise hormone levels.

No single treatment is effective on its own, so a combination of treatments (often a 3 part strategy) is usually required.

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15
Q

1) What is usually first line management for the treatment of acromegaly?
2) What are patients with residual disease after first line management offered?

A

1) Trans-sphenoidal surgery

2) Adjuvant drug treatment to reduce GH levels.

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16
Q

What is the 3 part strategy for the treatment of acromegaly?

A

1) Remove the source of excessive GH.
2) Medication/ radiotherapy as an adjunct.
3) GH receptor antagonist.

17
Q

State which drugs can be used as adjuncts after surgery for patients with acromegaly.

A
Somatostatin analogues (block GH) such as Octreotide. 
Dopamine agonists such as Bromocriptine.
18
Q

1) How is refractory disease treated for patients with acromegaly?
2) What is the 3rd line drug management or acromegaly and when can it be used?

A

1) With radiotherapy (radiotherapy can also be used when surgery is contraindicated however, mortality rates are higher).
2) Pegvisomat is used in patients with inadequate response to surgery or radiotherapy and in those who are resistant or intolerant to somatostatin analogues.

19
Q

How is Pegvisomat administered?

A

Daily by subcutaneous injection.

20
Q

1) What is hypopituitarism?

2) What is panhypopituitarism?

A

1) A condition involving decreased secretion of anterior pituitary hormones.
2) A condition where there is a deficiency in all anterior pituitary hormones.

21
Q

1) What is hypopituitarism?
2) What is panhypopituitarism?
3) What can cause panhypopituitarism?

A

1) A condition involving decreased secretion of anterior pituitary hormones.
2) A condition where there is a deficiency in all anterior pituitary hormones.
3) Irradiation, surgery or pituitary tumours.

22
Q

In hypopituitarism, in what order are the anterior pituitary hormones affected in?

A
GH
FSH and LH
TSH
ACTH
Prolactin

**There may also be decreased secretion of other hormones due to either tumour mass or lack of preceding hormones.

23
Q

1) The features of hypopituitarism present due to what?

A

1) Lack of hormones.

24
Q

1) The features of hypopituitarism present due to what?
2) What does the absence of prolactin cause?
3) What does absence of ACTH cause?
4) What does absence of TSH cause?

A

1) Lack of hormones.
2) Absent lactation.
3) Symptoms as for adrenal insufficiency (no skin hyperpigmentation though).
4) Symptoms as for hypothyroidism.

25
Q

Describe some of the signs or symptoms of the absence of growth hormone.

A
Central obesity
Atherosclerosis
Dry wrinkly skin
Decreased strength
Decreased balance
Decreased exercise ability
Decreased cardiac output
Osteoporosis
Decreased glucose levels
26
Q

Describe some of the signs or symptoms of the absence of FSH/LH.

A
Oligomenorrhoea/ amenorrhoea
Decreased fertility
Decreased libido
Decreased muscle bulk
Osteoporosis
Breast atrophy
Dyspareunia
Erectile dysfunction
Hypogonadism.
27
Q

List 5 potential basal investigations that you would do for patients with potential hypopituitarism.

A
LH/FSH
Testosterone/ oestradiol
TSH
T4
Prolactin
IGF-1
Cortisol
Blood glucose 
U&Es: could be dilution hyponatraemia
FBC: could be normocytic, normochromic anaemia
28
Q

Aside from bloods, name 3 other investigations that could be done for a patient with potential hypopituitarism.

A

Cranial MRI: Assess for cause
Short synacthen test
Insulin tolerance test to assess adrenal and GH axes (glucagon stimulation test can be used if insulin tolerance test is contraindicated
GNRH-arginine test used to assess body’s ability to make GH.

29
Q

What are the mainstays of treatment for patients with hypopituitarism?

A

Surgical or medical removal or treatment of the causative lesion and medical replacement of hormones if necessary.

30
Q

Describe medical treatments which may be required as forms of hormone replacement.

A

Glucocorticoids: if there is impairment HPA axis.
Levothyroxine: if secondary hypothyroidism present.
Testosterone/oestrogen replacement: if gonadotrophin deficiency.
Somatotrophin: mimics human GH.
DHEA: in hypoandrogenic women may improve wellbeing and sexual function.