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Endocrine > hypopituitarism > Flashcards

hypopituitarism Flashcards

1
Q

approach to dx and management

A

-The goal is always to maintain homeostasis
-Using physiology and pathophysiology to explain the symptoms and signs.
-What if any, are the risk factors, that might result in the pathology?
-How would you approach treating the symptoms and signs?
-What does follow up entail?
-How would you explain it to the patient?

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2
Q

hypopituitarism dx + tx

A

Diagnosis:
-Imaging tests: ideally MRI
-Measurement of pituitary hormone levels -> Basally and after various provocative stimuli

Treatment:
-Removal of any tumor/suppression medications
-Administration of replacement hormones

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3
Q

Hypopituitarism is a ______ syndrome, with _______ of anterior pituitary function +/______

A

Endocrine deficiency syndrome

Partial or complete loss of ANTERIOR pituitary function

With or without associated mass lesions

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4
Q

Causes of Primary Hypopituitarism causes

A

Pituitary tumors
-adenoma
-craniopharyngioma (MCC in kids)

infarction or ischemic necrosis:
-hemorrhagic infarction (pituitary apoplexy)
-shock- sheehan’s syndrome
-vascular thrombosis or aneurysm

infiltrative disorders
-Hemochromatosis
-Langerhans’ cell histiocytosis- (Hand-Schüller-Christian disease)

inflammatory processes
-Meningitis
-Pituitary abscess
-Sarcoidosis

Iatrogenic
Autoimmune

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5
Q

primary hypopituitarism causes just list the major categories

A

-Pituitary tumors
-Infarction or ischemic necrosis
-Inflammatory processes
-Infiltrative disorders
- Iatrogenic
-Autoimmune dysfunction

“PIIIIA”

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6
Q

secondary hypopituitarism

A

-Hypothalamic tumors
-Neurohormone deficiencies of the hypothalamus
-Trauma
-Inflammatory diseases
-Iatrogenic

“HNT II”

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7
Q

Mortality Morbidity of Hypopituitarism. Which hormone is most urgent?

A

ACTH»> MOST URGENT WITHOUT YOU WILL DIE**

next TSH» GH» FSH/LH

ACTH → Adrenal Crisis = Life-Threatening
- especially if its sudden loss of production rather than slowly progressive
TSH → Myxedema Coma = Life-Threatening
GH deficiency is more morbid in children than adults
FSH/LH Deficiency with Hypogonadism is more insidiously morbid
Congenital hypopituitarism has greater morbidity effects

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8
Q

With hypopituitarism - what order do we lose hormones?

A

MC GH is lost first **
- then gonadotropins -> then TSH and ACTH

GH → FSH/LH → TSH → ACTH **
The body tries to preserve the hormones needed for survival
–panhypopituitarism:

ADH deficiency = rare in primary pituitary ds
- common with stalk and hypothalamic lesions

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9
Q

Onset of hypopituitarism is usually

A

insidious - may not be recognized by pt
-occasionally onset is sudden or dramatic

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10
Q

other presenting features of hypopituitarism if its space occupying

A

Pt with SPACE occupying lesion may present with headaches or visual field deficits: optic chiasm lesion
-BITEMPORAL HEMANOPSIA

Pt with large lesions involving the hypothalamus:
- may present with polydipsia and syndrome of SIADH

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11
Q

investigation/dx of hypopituitarism

A

ideally MRI***
- HRCT w/wo contrast to r/o structural abnormalities

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12
Q

T4, TSH in generalized hypopituitarism vs primary thyroid deficiency

A

Generalized hypopituitarism/secondary:
- LOW in BOTH T4, TSH

Primary hypothyroidism:
- INCREASED TSH
- LOW T4

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13
Q

acute adrenal insufficiency: urgent treatment

A

NEED IMMEDIATE TX W GLUCOCORTICOIDS

-100 mg hydrocortisone IV, then 50-100mg every 6hr and fluid replacement
-immediate referral for visual field loss is required -> bitemporal hemianopsia
-refer immediately to an experienced neurosurgeon

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14
Q

Pituitary Apoplexy complications

A
  • Catastrophic adrenal failure
  • visual field loss
  • raised ICP
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15
Q

Pituitary Apoplexy tx

A

Needs Immediate treatment with Glucocorticosteroids!!!!!
If visual field loss = Referral to neurosurgery
- bitemporal hemianopsia

Immediate surgery warranted if:
-Visual field disturbances
-Oculomotor palsies
-Somnolence progresses to coma because of hypothalamic compression
-Although management with high-dose corticosteroids and general support may suffice in a few cases, transsphenoidal decompression of the tumor should generally be undertaken promptly

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16
Q

tx of hypopituitarism

A

Goal: Hormone replacement of hypo-functioning target glands
If tumor:
Transsphenoidal removal or irradiation
Prolactinoma: Dopamine agonist is given as initial tx

17
Q

DDx of generalized hypopituitarism

A

-anorexia nervosa- maintenance of secondary sexual characteristics despite amenorrhea -> increased levels of basal growth hormone and cortisol
-alcohol liver disease of hemochromatosis- evidence of liver disease -> lab testing
-myotonia dystrophica- progressive weakness, premature balding, cataracts, facial features of accelerated aging -> lab testing
-polyglandular autoimmune disease

18
Q

hypopituitarism in children -Presentation

A

-Height is below the 3rd percentile, and growth velocity
-Skeletal maturation > 2 yr behind chronologic age
-Fails to begin pubertal development

19
Q

Hypopituitarism in children: causes

A

MCC is craniopharyngioma- pituitary tumor**

Other causes

Langerhans’ cell histiocytosis

Midline defects- Cleft Palate

Radation of CNS, idiopathic (5%), hereditary

20
Q

Hypopituitarism in children: work up and tx

A

Work-up:
-Assess bone age: X-ray of the left hand
-Skeletal maturation usually delayed to the same extent as height
-CT or MRI of brain
-R/o calcifications & tumors
-Mid to late childhood: IGF-1 levels measured

Treatment:
-Surgery
-GH replacement + other hormones (Cortisol, Thyroid, gonadal sex steroids)

21
Q

GH deficiency: adults vs kids

A

Adults
- undetectable
-usually asymptomatic
-decreased energy
-?? accelerates atherosclerosis

Children:
-abnormally slow growth
-short stature with NORMAL proportions

22
Q

ACTH deficiency sx

A

aka Hypoadrenalism

Sx:
-fatigue
-weakness
-wt loss
-hypotension
-hypoglycemia
-intolerance to stress and infection
-decreased axillary and pubic hair
-NO hyperpigmentation
- low plasma and urinary STEROID levels ( will become normal after ACTH replacement)

23
Q

ACTH deficiency can lead to _______. how do you treat it?

A

Hypoadrenalism
-tx is with cortisol replacement

24
Q

ACTH investigations

A

Standard ACTH simulation test **
-Safe & less labor intensive
-Synthetic ACTH (cosyntropin) is administered IV/IM
-Plasma cortisol response measured 30 and 60 min later

Normal:
- Cortisol levels ↑↑↑ (> 21 g/dL) and Aldosterone ↑ (>4 ng/dL above baseline)

Insulin Tolerance Test: Hazardous do in pts with severe
-Panhypopituitarism
-Diabetes mellitus
-Elderly
-Contraindicated in those:
-Coronary artery disease
-Epilepsy

25
Q

lack of LH and FSH: children, premenopause women and men

A

Children:
- delayed puberty
-short stature

Premenopausal women:
-amenorrhea
-reduced libido
-regression of secondary sexual characteristics
-infertility

men:
-ED
-testicular atrophy
-reduced libido
-regression of secondary characteristics
-decreased spermatogenesis with infertility

26
Q

kallmann’s syndrome associated factor and s+s

A

GnRH deficiency:
- Associated with midline facial defects

S/S:
Anosmia: cannot smell
Cleft Palate/Lip
Color Blindness
hypogonadism: reduced sex hormones -> infertile, no puberty
-1/2 of these pts have unilateral renal agenesis (kidney fails to develop)

-some pts also exhibit cryptorchidism (undescended testes), micropenis, sensorineural deafness, cerebellar ataxia (affecting coordination and balance), cognitive problems, bimanual synkinesis (mirror movements of hands), and a high arched palate.
-some affected women have menarache followed by secondary amenorrhea

27
Q

Kallmann’s syndrome is lack of what hormone?

A

Lack of gonadotropin-releasing hormone (GnRH)

28
Q

simmond’s syndrome

A

aka Panhypopituitarism:
- involvement of ALL pituitary hormones

Often: only 1+ pituitary hormones involved resulting in partial hypopituitarism

“Simms game = PAN; you can control the whole world”

29
Q

Sheehan’s syndrome

A

Pituitary necrosis from hypovolemia and shock
- Due to increased blood demand from pituitary and decreased blood supply lost during pregnancy

S/S:
- Lactation does not start after childbirth
- Fatigue
- Loss of secondary sex characteristics (pubic and axillary hair loss)

30
Q

Who does Sheehan’s syndrome affect and what is it

A

Affects postpartum women *
- aka: postpartum pituitary gland necrosis
-rare condition that can occur in women who experience severe blood loss (hemorrhage) or hypovolemic shock during or after childbirth

31
Q

pituitary apoplexy sx

A

Symptoms:
-severe headache
-stiff neck
-fever
-visual field defects: bilateral hemi
-oculomotor palsies
- CSF ± Blood

May present with edema which if compresses hypothalamus:
- Risk of Somnolence or Coma

Varying degrees of hypopituitarism may develop suddenly:
- many present with vascular collapse bc of deficiency ACTH and cortisol

32
Q

Pituitary apoplexy definition + diagnostic studies

A

Sudden enlargement of pituitary caused by:
- pituitary tumor*
- hemorrhagic infarction of pituitary gland
- medical emergency!!! - ACUTE hormonal deficiencies

need MRI** to assess hemorrhage

Endocrine (11 decks)

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