ENDOCRINE - Pituitary conditions Flashcards
What is prolactin under tonic inhibition by?
Dopamine
What hormones are produced by the posterior pituitary?
ADH
Oxytocin
What is a pituitary adenoma?
Benign tumour of glandular tissue
Which syndrome are pituitary adenomas associated with
MEN I/IIa
Def microadenoma
<1cm
Def macroadenoma
> 1cm
What is the difference between functioning and non-functioning pituitary adenomas?
Functioning - secretory
PS: non-functioning pituitary adenoma (5)
Bitemporal hemianopia Ocular palsies Hypopituitarism Signs raised ICP e.g. headache Hypothalamic compression Sx
E.g.s of hypothalamic compression Sx (3)
Altered appetite
Thirst
Sleep/wake cycle
PS: functioning pituitary adenoma
Acromegaly
Hyperprolactinaemia
Cushings
Which hormones are rarely secreted from pituitary adenomas?
TSH
FSH
LH
PS hyperprolactinaemia (5)
Galactorrhoea Oligo/amenorrhoea Decreased libido Subfertility in M/ ereticle dysfunction Arrested puberty - younger pt
LT consequence hyperprolactinaemia
Osteoporosis
Causes hyperprolactinaemia
Prolactinoma
Breast stimulation/stress
Dx induced
Idiopathic
How is prolactinoma diagnosed?
Pituitary MRI
Following raised serum prolactin
Tx hyperprolactinaemia
DA agonists - ropinirole/bromocriptine
SE Ropinirole/bromocriptine (4)
N+V
Dizzy
Syncope
Fibrosis
What must be monitored on Ropinirole/bromocriptine
Echo due to fibrosis
XS GH in children –>
Gigantism
If prior to epiphyseal plate closure
XS GH in adults –>
Acromegaly
What is acromegaly almost always due to?
Pituitary tumour
Rarer causes acromegaly
Paraneoplastic - non- pituitary tumours
diagnosis acromegaly (2)
Raised IGF-1
GTT - normally suppresses GH secretion. Acromegaly - [ ] = 2mcg/ml at 2hrs
What does IGF-1 represent
GH levels over 24hrs
Sx acromegaly (9)
Change in appearance Increased hand/foot size Tired XS sweating Loss libido Sx DM Headaches Visual deterioration Sx hypopituitarism
Signs acromegaly (9)
Protruding mandible Prominent supraorbital ridge Interdental separation Large tongues Spade-llike hands/feet Tight rings VF defects HTN Hypopituitarism
Mx acromegaly
Somatostatin analogues to shrink tumour
Transphenoidal surgery
What is Cushing’s syndrome due to
Increased levels of glucocorticoid
Most common cause Cushings syndrome
Exogenous administration of steroids
Or XS endogenous secretions ACTH
ACTH dependent causes Cushings (2)
Cushings disease
Ectopic ACTH - non-pituitary ACTH secreting tumour
ACTH independent causes Cushings (2)
XS adrenal cortisol production - e.g. b.c adrenal tumour
Steroids
What is Cushings disease
Increased ACTH from the pituitary gland
Sx Cushings (= MOON FACE)
Menstrual changes Obesity (central) + striae Osteoporosis Neurosis/depression Facial plethora Altered mm (proximal myopathy) Calor skin Elevated BP (HTN)
What electrolyte disturbance may occur in Cushings (+ why)
Hypokalaemia
B/c of mineralcorticoid activity of cortisol
Diagnosis Cushings (3 tests)
1) Confirm raised cortisol w/ overnight dexamethasone suppression test
2) DDx ACTH indepedent + dependent causes - 9am + midnight plasma ACTH ((+ cortisol) levels
3) Which type of ACTH dependent is it? Low + high dose dexamethasone suppression test
Details of Overnight dexamethasone suppression test
Out pt
1mg PO dexamethasone given at night
Serum cortisol checked before + at 8am
Norm: -ve feedback –> cortisol levels decrease
Cushings: failure to suppress cortisol secretion
Details of 9am and midnight plasma ACTH (+cortisol) levels
If ACTH depressed/undetecable = adrenal cause likely
If ACTH is detectable = either pituitary or ectopic source
Details of low + high dose dexamethasone suppression test
Dexamethasone 2mg/6hr PO for 2d
Measure plasma + urinary cortisol 0 +48hrs
Complete/partial suppression = Cushings disease
No suppression = ectopic source
Mx Cushings disease
Transphenoidal surgery
Def hypopituritism
Defective production of all pituitary hormones
PS hypopituritism
Fatigue Myaglia HoTN Diabetes insipidis Hypothyroidism
Pituitary causes of hypopituirism (3)
Obliteration - 1’/mets tumour
Surgical removal/irradiation
Ischaemic necrosis b/c HoTN shock
hypothalamic causes of hypopituiritism (4)
1’ brain tumour = cranipharyngoma
Infarction
Sarcoid
Infection
Diagnosis hypopituritism
Pituitary hormones all low
Effector gland hormones = low
Low response to suppression tests
Imaging (to localise the pathology)
What is posterior pituitary disease usually a result of
Damage to hypothalamus (tumour invasion/infarction)
Failure of ADH prod –>
Diabetes insipidis
XS ADH production –>
SIADH
What is a pheochromocytoma
Catecholamine secreting tumour arising from sympathetic paraganglion cells (chromaffin cells)
What familial conditions are 10% of pheochromocytoma associated with?
Men 2a/b
NFM
Von Hippel-Lindau syndrome
PS pheochromocytoma
Severe HTN unresponsive to medical Sx Sweating / heat intolerance Visual disturbances/headaches Seizures Palpitation Chest tightness Dyspnoea Postural hotn Abdo pain N+ C
What makes pt with pheochromocytoma Sx worse?
Stress
Exercise
Drugs
Which drugs must NOT be given alone to pt w/ pheochromocytoma
B-blockers
due to LF HTN episodes
Diagnosispheochromocytoma
3x24h urine - raised free metadrenaline + normadrenaline
CT/MRI - locate tumour
Mx pheochromocytoma (3)
Alpha block - phenoxybenzamine
B blockers once alpha established
Surgical excision
What is Addisons
Primary adrenal insufficiency due to destruction of adrenal cortex
How does Addisons differ from HPA disease?
HPA spares mineralcorticoid production (which = stim’d by ATII)
Most common cause Addisons UK
Autoimmune (80%)
Most common cause of Addisons worldwide
TB
Other causes Addisons
Overwhelming sepsis
Mets - lung/breast
Lymphoma
Waterhouse-Friderichsen syndrome
What is Waterhouse Friderichsen syndrome?
Adrenal gland failure because of bleeding into the adrenal glands
What is Waterhouse-Friderichsen syndrome most commonly caused ny
N.meningitides
Sx Addisons (3)
W loss/malaise/weakness/myalgia
Syncope
Depression
Signs Addisons (4)
Pigmentation esp on palmar creases + new scars
POstural HoTN
Signs dehydration
Loss body hair
Ix Addisons (6)
FBC U+E Ca Glucose Short ACTH stimulation/Synacthen test 9am cortisol/ACTH test
FBC results Addisons
Anaemia
U+E results Addisons
Low Na
High K
Uraemia
Ca2+ in Addisons
Raised
Glucose in Addisons
Low
B/c lack of cortisol
Short ACTH stim test/Synacthen test (for Addisons)
Give tetracosactide IM (ACTH analogue)
measure plasma cortisol before + at 30mins after
2nd value >550nmol/L excludes Addisons
9am ACTH/Cortisol test Addisons
Raised ACTH
Low/normal cortisol confirms Addisons
Ix for cause of Addisons (3)
21-hydroxylase adrenal antibodies - autoimmune disease
CXR for TB
Adrenal CT - look for TB/mets if antibodies negative
Mx Addisons (3)
LT glucocorticoid cover: 15-25mg HC daily in 3 divided doses
LT mineralocorticoid cover: fludrocortisone 50-200micrograms daily
Steroid card/Addisons bracelet _ IM HC in case of Addisonian crises
How should steroids be taken in Addisons + why
Avoid given late in day
B/c can cause insomnia
When does LT mineralcorticoid cover need to be prescribed in Addisons (3)
If postural HoTN
If Low Na
If high K
When should extra doses of steroids be prescribed in Addisons?
If strenuous exercise
Double for: surgery, febrile illness, trauma
Def Addisonian crisis
Severely inadequate levels of cortisol, occurring either as 1st PS of Adrenal disease or triggered by physiological events
PS Addisonian crisis (6)
Fever N+V Shock Hypoglycaemia Hyponatraemia Hyperkalaemia
Mx Addisonian crises
IV Fl
IV Hydrocortisone
What is CAH (Congenital adrenal hyperplasia)
Congenital deficiency in 21-alpha-hydroxylase
Role of 21-a-hydroxylase
Prod of mineralcorticoid s + glucocorticoids, but not sex hormones
CAH - levels of difference hormones
Aldosterone decreases Cortisol decreases ACTH hence rises Precursors e.g. progesterone build up —> alt Sex hormone pathways Hence testosterone increased
CF CAH in Females
Virilisation of external genitalia —> clitoral hypertrophy + variable fusion of labia
CF CAH in males
Enlarged penis Pigmented scrotum Salt losing crises 80% M at 1-3w age Or Non-salt losing M —> hypervirilisation - early pubarche, adult body odour, mom build
Ix CAH
17-a-hydroxyprogesterone levels MARKLY RAISED = diagnostic
Extra Ix for diagnosis CAH salt losers
Low Na
High K
Metabolic acidosis
Mx CAH
Steroid cover as per Addisons
What are CAH at risk of
Addisonian Crises
Who gets Conn’s syndrome?
Young females
What is COnns
Adrenal adenoma —> 1’ hyperaldosteronism
What are the 2 main causes of 1’ hyperaldosteronism
Conns (60%)
Bilateral Adrenal hyperplasia (30%)
PS hyperaldosteronism (3)
Mostly asymp
To resistant HTN —> headache
Features of hypokalaemia —> cramps/weakness/tetany
Biochemical features Conns (2)
Hypokalaemia
Elevated aldosterone: renin ratio
Ix Conns
Plasma Aldosterone levels NOT suppressed by fludrocortisone administration
Adrenal CT to differentiate COnns from adrenal hyperplasia
Adrenal scintigraphy - unilateral uptake in Conns
Mx COnns (2)
Laparoscopic adrenalectomy
Spironolactone pre-op (control HTN/hypoK)