Panhypopituitarism Flashcards

1
Q

What are the hypothalamic causes of hypopituitarism?

A

Tumors

  • Craniopharyngioma
  • Metastasis from malignant tumors e.g. lung and breast CA

Infarct / Stroke (rare)

Radiation therapy
- Can lead to hypothalamic / pituitary dysfunction or both
Is dose and time-dependent, and may not become apparent until many years s/p treatment
- Hence impt for pt post-therapy to go for regular screening

Infiltrative lesions :

  • Marked thickening of infundibulum on MRI is a common finding
  • Conditions include Sarcoidosis and Langerhans cell histiocytosis

Infections: TB, Candida, Hanta virus

Traumatic brain injury (TBI)

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2
Q

What are the pituitary causes of hypopituitarism?

A

Mass lessions

  • Most commonly by a non-secretory pituitary macroadenoma > 1cm
  • Commonly: HG, Prolactinoma, Non-Secreting pituitary tumor

Pituitary surgery – Pituitary function may be better or worse afterwards

Pituitary radiation

Sheehan syndrome

Pituitary apoplexy

Snake bite: Russell viper bites have been shown to cause hemorrhagic necrosis of the pituitary

Hemochromatosis : Gonadotropin deficiency is the most common endocrine abnormality

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3
Q

How does non secretory pituitary adenoma present?

A

Presents with loss of multiple anterior pituitary hormone deficiencies

  • GH and gonadotrophins are usually first affected
  • TSH and ACTH are usually last to be affected

A/w HYPERPROLACTINAEMIA due to Stalk Compression Syndrome

  • Loss of dopaminergic inhibition from hypothalamus
  • Don’t confuse this w/ prolactinoma 🡪 will p/w macroadenoma w/ Prolactinaemia >4-5x ULN; whereas in SCS prolactin levels usually 2x ULN
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4
Q

What is Sheehan’s syndrome and how does it present?

A

Infarction after postpartum haemorrhage, often severe as to cause hypotension and require multiple transfusions

In severe hypopituitarism, patient may develop lethargy, anorexia, weight loss, and inability to lactate during the first days/weeks after delivery

In mild hypopituitarism, there may be delay in recognition for many year

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5
Q

What is pituitary apoplexy and how does it present?

A

Sudden haemorrhage into the pituitary gland, often into a pituitary adenoma

This may produce severe headache, double vision and sudden severe visual loss, sometimes followed by acute life-threatening hypopituitarism.

Often pituitary apoplexy can be managed conservatively with replacement of hormones and close monitoring of vision, although if there is a rapid deterioration in visual acuity and fields, surgical decompression of the optic chiasm may be necessary.

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6
Q

How does a patient with growth hormone deficiency present?

A
  • Growth Failure (during childhood) -> Dwarfism
  • ↓ bone density
  • Muscle atrophy & weakness
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7
Q

How does a patient with prolactin deficiency present?

A

Lactation Failure following delivery

Asymptomatic in Males

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8
Q

How does a patient with central TSH deficiency present?

A
  • Weight gain
  • LOA
  • Cold intolerance
  • Lethargy
  • Constipation
  • Dry skin
  • Menstrual Change (Oligo/Menorrhagia)
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9
Q

How does a patient with 2’ adrenal deficiency present?

A
  • HypoNa
  • HypoTN
  • Hypoglycaemia
  • Lethargy
  • Hypoandrogenism (in women)
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10
Q

How does a patient with ADH deficiency present?

A

Central DI 🡪 polyuria, polydipsia

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11
Q

How does a patient with oxytocin deficiency present?

A

No Effect

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12
Q

How does a male with 2’ hypogonadism present?

A
  • Testicular Atrophy
  • Gynecomastia
    Infertility, Loss of libido
    Menopausal / Andropausal S&S, Osteoporosis, CV S/E
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13
Q

How does a female with 2’ hypogonadism present?

A

Amenorrhea

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14
Q

What is Kallman’s syndrome?

A

Isolated gonadotrophin (GnRH) deficiency due to mutations in the KAL1 gene on the short arm of X Ch

Characterized by anosmia

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15
Q

What is septo- optic dysplasia?

A
  • Congenital syndrome associated with mutations in the HESX1 gene 🡪 resulting in developmental abnormality of the forebrain
  • Presents in childhood with a clinical triad of midline forebrain abnormalities, optic nerve hypoplasia and hypopituitarism.
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16
Q

What is ‘empty sella’ syndrome?

A

An ‘empty sella’ on pituitary imaging

This is sometimes due to a defect in the diaphragma and extension of the subarachnoid space (cisternal herniation) OR may follow spontaneous infarction or regression of a pituitary tumour

All or most of the sella turcica is devoid of apparent pituitary tissue, but, despite this, pituitary function is usually normal, the pituitary being eccentrically placed and flattened against the floor or roof of the fossa.

May be associated with a certain degree of decreased pituitary function, causing hypopituitarism

17
Q

What is the investigations performed for GH deficiency?

A

First measure IGF-1 levels 🡪 will be low

Then perform Insulin tolerance test / glucagon stimulation test

  • First, exclude CVD, epilepsy, and patients with severe untreated hypopituitarism
  • Involves overnight fast followed by IV insulin 0.15U/kg at time 0
  • Caution – must be done with care else may ppt severe neuroglycopenia 🡪 seizure, coma
  • Glucose, cortisol and GH levels are measured at 0, 30, 45, 60, 90, 120min
  • Is meant to ↑ GH and Cortisol!
18
Q

What is the investigations performed for Gonadotrophin deficiency?

A

Measure serum LH & FSH

Men: testosterone + SHBG

Women: Estradiol

Females: the presence of regular menstrual cycles effectively rules out gonadotropin deficiency; no further diagnostic test is required.

19
Q

What is the investigations performed for TSH deficiency?

A

do TFT – showing ↓ T3, ↓ T4, ↓ or normal TSH

20
Q

What is the investigations performed for ACTH deficiency?

A

Administer an 8am cortisol + ACTH + Synacthen Test showing

  • 8am cortisol & ACTH shows Hypocortisolism & low ACTH
  • If acute central A-I: optimal rise in cortisol
  • If chronic central A-I: suboptimal rise in Cortis
21
Q

What is the investigations performed for prolactin deficiency?

A

check serum prolactin levels

22
Q

What are the 2 hormones that needs to be replaced in a patient with pan hypopituitarism going for surgery for removal of adenoma?

A

The 2 main replaced hormones are Cortisol & Thyroxine

  • Cortisol, so that pt doesn’t develop Addisonian crisis intra-op
  • Thyroxine, so that pt doesn’t develop Myxedema Coma crisis intra-op

Always cortisol before thyroxine b/c:

  • Cortisol has a permissive effect on thyroxine
  • ↑ Thyroxine activity increases cortisol clearance 🡪 further lowers cortisol levels 🡪 ppt adrenal crisis
23
Q

What are the indications for growth hormone replacement?

A

Children for growth!

Adults exhibiting excessive tiredness, muscle weakness, weight gain 🡪 improves psychological well being

24
Q

How do you replace sex hormones in a young man?

A

we give testosterone (IM/Gel) for libido, sexual activity

  • ALONE CANNOT BE ENOUGH
  • If wants to father a child: give FSH & LH injections for spermatogenesis & testosterone production
25
Q

How do you replace sex hormones in an old man?

A

We will still give testosterone (IM/Gel) to prevent osteoporosis

26
Q

How do you replace sex hormones in a woman?

A

Young woman b4 menopause – Give HRT

Old woman – DO NOT GIVE HRT