Hyposecretion of APG hormones Flashcards

1
Q

5 hormones released from adenohypophysis?

A
FSH/LH
Prolactin
ACTH
TH
GH
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2
Q

What determines the disorder category?

A

The level at which the defect lies

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3
Q

Primary endocrine gland disease?

A

Lies in the ENDOCRINE GLAND

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4
Q

Secondary endocrine gland disease?

A

Defect is in the ANTERIOR PITUITARY HORMONE

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5
Q

Hypopituitarism?

A

Decreased production of SPECIFIC hormones

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6
Q

Panhypopituitarism?

A

Decreased production of ALL anterior pituitary hormones

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7
Q

2 types of causes?

A

Congenital (rare)

Acquired

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8
Q

Cause of congenital panhypopituitarism?

A

Rare

Cause: mutations of TFs genes needed for normal anterior pituitary development (PROP1 mutation)

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9
Q

Presentations of congenital panhypopituitarism?

A

Deficient in GH and at least ONE MORE anterior pituitary hormone

Short stature
Hypoplastic APG on MRI

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10
Q

Cause of acquired panhypopituitarism?

A
x Tumours
x Radiation
x Infection
x Traumatic brain injury
x Infiltrative disease
x Inflammatory
x Pituitary apoplexy
x Sheehan's Syndrome (peri-partum infarction)
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11
Q

Acquired panhypopituitarism:

Tumours, Radiation, Infection, Infiltrative disease, Pituitary apoplexy?

A

Tumours - hypothalamic (craniopharyngiomas) & pituitary (adenomas, metastases, cysts)

Radiation - hypothalamic/pituitary damage (GH most vulnerable, TSH least!)

Infection - meningitis

I.D - often invovles pituitary stalk (neurosarcoidosis)

P.A - haemorrhage (less commonly infarction)

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12
Q

Causes of panhypopituitarism?

A

Simmond’s Disease

Symptoms due to deficient hormones

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13
Q

FSH/LH on panhypopituitarism?

A

2o hypogonadism

Reduced libido
2o amenorrhoea (F)
Erectile dysfunction (M)
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14
Q

ACTH on panhypopituitarism?

A
2o hypoadrenalism (cortisol deficiency)
Fatigue
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15
Q

TSH on panhypopituitarism?

A

2o hypothyroisism

Fatigue

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16
Q

What is Sheehan’s Syndrome?

A

Post-partum Hypopituitarism

2o to hypotension (PPH - post partum haemorrhage)

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17
Q

What causes Sheehan’s Syndrome?

A

Anterior pituitary englarges in pregnancy (due to lactotroph hyperplasia)

SO the hypotension from loss of blood from giving birth = pituitary becomes under perfused = infarction

(Posterior PG usually NOT affected)

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18
Q

Easy presentations of Sheehan’s Syndrome to notice?

A

Failure of lactation - PRL deficiency

Failure to resume menses post-delivery

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19
Q

Hard presentations of Sheehan’s Syndrome to notice?

A

Lethargy
Anorexia
Weight loss (TSH, ACTH, GH) deficiency

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20
Q

What is pituitary apoplexy?

A

Intra-pituitary haemorrhage (or less commonly infarction)

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21
Q

What type of patients can present with pituitary apoplexy?

A

Dramatic presentation in patients w. pre-existing pituitary tumours (adenomas)

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22
Q

What can be used for pituitary apoplexy?

A

Precipitated by anti-coagulants

23
Q

Symptoms of pituitary apoplexy?

A

SEVERE sudden onset headache

Visual field defect - compressed optic chiasm (above PG)
x Bitemporal hemianopia

Cavernous sinus
x Diplopia - double vision (CNIV & VI)
x Ptosis - drooping upper eyelid (CNIII)

24
Q

Type of biochemical diagnosis for hypopituitarism which is limited?

A

Basal plasma [ ] of pituitary OR target endocrine gland hormones

25
Q

Why is this type of biochemical diagnosis limited?

A

Cortisol - undetectable as [ ] varies throughout the day

T4 - has a LONG half-life (6 days) so many take a while to fall

FSH/LH - cyclical

GH/ACTH - pulsatile

26
Q

What is the alternative biochemical diagnosis method for hypopituitarism?

A

Stimulated pituitary function tests

27
Q

Methods used for the alternative biochemical diagnosis method for ACTH & GH hypopituitarism?

A

ACTH & GH - ‘stress’ hormones with ‘stress’ defined as HYPOGLYCAEMIA

So an insulin-induced hypogylcaemis stimulates GH and ACTH (cortisol measured) release

28
Q

Methods used for the alternative biochemical diagnosis method for TSH hypopituitarism?

A

TRH (in injection) stimulated TSH release

29
Q

Methods used for the alternative biochemical diagnosis method for FSH & LH hypopituitarism?

A

GnRH stimulated FSH & LH release

30
Q

What is the radiological diagnosis for hypopituitarism?

A

Pituitary MRI - may reveal specific pituitary pathology e.g. haemorrhage (apoplexy), adenoma, empty sella (thin rim of pituitary tissue)

31
Q

Why can an CT scan not be used for hypopituitarism diagonsis?

A

Can only see blood in fossa

32
Q

HRT for ACTH?

A

Deficient Hormone: ACTH

Replacement: Hydrocortisone (cannot replace w. ACTH as pulsatile)

Check: Serum cortisol

33
Q

HRT for TSH?

A

Deficient Hormone: TSH

Replacement: Thyroxine

Check: Serum free T4

34
Q

HRT for Women LH/FSH?

A

Deficient Hormone: LH/FSH

Replacement: HRT (E2 progestagen)

Check: Symptom improvement, withdrawal bleeds

35
Q

HRT for Men LH/FSH?

A

Deficient Hormone: LH/FSH

Replacement: Testosterone

Check: Symptom improvement, serum testosterone

36
Q

HRT for GH?

A

Deficient Hormone: GH

Replacement: GH

Check: IGF1, growth chart (children)

37
Q

Effects of GH (somatotrophin) deficiency in children and adults?

A

Children - short stature

Adults - effects less clear

38
Q

7 causes of short stature?

A

x Genetic - Down’s/Turner’s/Prader Willi Syndrome
x Emotional deprivation
x Systemic disease - CF, rheumatoid arthritis
x Malnutrition
x Malabsorption - coeliac disease (go gluten-free diet!)
x Endocrine disorders - Cushing’s syndrome, Hypothyroidism, GH deficiency, poorly controlled T1DM
x Skeletal dysplasias

39
Q

3 points of short stature causation in children on the GROWTH AXIS?

A

Hypothalamus - Prader-Willi Syndrome

Anterior pituitary hormone (lack of GH) - Pituitary Dwarfism

GH receptor defect - Laron Dwarfism

40
Q

Prader Willi Syndrome?

A

GH deficiency 2o to hypothalamic dysfunction

41
Q

Dwarfism?

A

ACHONDROPLASIA
(skeletal abnormality - endocrine system is FINE)

Mutation in FGF3
Abnormality in growth plate chondrocytes - impaired linear growth

42
Q

Presentation of dwarfism?

A

Average size trunk

Short arms & legs

43
Q

Pituitary dwarfism?

A

Childhood GH deficiency

44
Q

Laron dwarfism?

A

Mutation in GH receptors

45
Q

What can be used to treat laron dwarfism?

A

Treated with IGF-1 in childhood - can increase height

46
Q

How can you diagnose short stature?

A

Mid-parental height - predicted height mapped out and then followed

47
Q

3 causes of ACQUIRED GH deficiency in adults?

A

In adults, hypothalamus OR APG is normally affected

x Trauma
x Pituitary Tumour/Surgery
x Cranial Radiotherapy

48
Q

How can you diagnose GH deficiency in adults?

A

Cannot measure GH as pulsatile in nature

Provocative challenge i.e. STIMULATION TEST

49
Q

4 GH provocation tests?

A

GHRH + arginine [i.v.] (arginine stimulated GH production)

Insulin [i.v.] - via. hypoglycaemia (if deficient, very low GH response - should normally increase)

Glucagon [i.m.] - drives up GH and ACTH (as induces vomiting which is a stress-factor)

Exercise

50
Q

What is used as GH therpary?

A

Human recombinant GH (somatotropin)

51
Q

How is GH therapy administrated and monitored?

A

Daily, subcutaneous injection

Monitor respone to IGF-1 (what liver makes in response to GH)

52
Q

Symptoms of GH deficiency in adults?

A

x Reduced lean mass
x Increase adiposity
x Increase waist:
hip
x Reduced muscle strength & bulk (so reduced exercise performance [ONE of the PROVOCATION TESTS!!!!]
x Decreased HDL and Increased LDL
x Affects mental health - affecting quality of life

53
Q

Benefits of GH therapy in adults?

A

(literally opp. of the symptoms!)

54
Q

Potential risks of GH therpay in adults?

A

Increases susceptibility to cancer

Expensive! (£42k)