hypoadrenalism feb 2015

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Hypoadrenalism – Case Presentation Dr. Adam Stokes ST4 in Anaesthesia & ICM 24 th February 2015

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Hypoadrenalism – Case Presentation Dr. Adam Stokes

ST4 in Anaesthesia & ICM

24th February 2015

Overview

1.  Case Presentation

2.  Adrenal Physiology

3.  Hypoadrenalism 1.  Causes

2.  Clinical Features

3.  Diagnosis

4.  Treatment

Case Presentation - Background !  40 year old man

!  PMH: Subclinical hypothyroidism (2008) – not on replacement

! Admission Sept 2014 with right flank pain, no cause found

! DH: Nil regular, NKDA.

!  SH: Non-smoker, occasional alcohol, worked up until last 2 weeks, living with parents.

Case Presentation !  Presentation

!  1/12 Hx of lethargy, weakness, muscular spasms

!  1/52 Hx of nausea and vomiting, hiccups

!  2-3/7 Hx of repeated vomiting, occasionally blood tinged, patient reports feeling “slow”

!  Saw GP 2/7 ago- plan for bloods and f/u, but parents concerned and rang 111, who advised 999.

Case Presentation ! Assessment on admission

!  Resp: RR 18, Sats 100% on air

!  CVS: HR 80, BP 130/73, paramedics reported no postural drop.

!  Neuro: GCS 14/15, mild confusion/slowness in speech, PERLA, BM 4.9, afebrile.

!  GI: Abdo soft non-tender, BS active

!  Tanned skin, but not obviously pigmented mucous membranes.

Case Presentation !  Initial results/management in ED

!  Lab- Na<100, K 5.7, Cl 67, Ur 6.1, Cr 74 CRP 36, Hb 17.5 WCC 4.1, Plt 166

!  CXR: NAD !  ECG: NAD !  ABG: pH 7.44, pCO2 3, pO2 10.6, Lac 0.7, BE -8.6, Na

<100, Cl 69 !  Random cortisol and TFTs sent !  Treatment:

!  Metoclopramide !  Lansoprazole !  Saline 1000ml (commenced prior to Na result) !  Hydrocortisone 100mg

Case Presentation ! Next 24 hours in HDU

!  Arterial line inserted for frequent ABGs

!  Urine and plasma osmolalities sent, urinary Na

!  Results: TFTs - T4 14.5 (N), TSH 29.7 (high), plasma osmolality 212, urine 836, urine Na 71, random cortisol 110

!  Slow Na correction with 1.8% NaCl

!  CT head: Thrombosed cerebral artery aneurysm in circle of Willis (?significance), nil else

Case Presentation ! Day 3

!  Short synacthin test performed: 30min cortisol 290, 60min 275: positive test for primary hypoadrenalism

!  Levothyroxine commenced, and after results of synacthin test, fludrocortisone/hydrocortisone replacement started

!  Patient became more agitated/confused (required DOLS)

!  Renin/Aldosterone/ACTH and autoantibody screen sent.

Case Presentation !  Days 4 -10

!  Gradual improvement in mental condition and weakness with Na up to 130

!  CT head discussed with Neuro SGH- probably incidental but will f/u in due course

!  Discharged with f/u in Endo clinic in 2/52

!  Results: !  ACTH high. !  Renin high. !  Aldosterone normal. !  Thyroid autoantibodies positive. !  Adrenal autoantibodies weakly positive.

Adrenal Physiology

Hypothalamus Pituitary

Adrenal Cortex

(Glomerulosa)

Adrenal Cortex

(Fasiculata)

Adrenal Cortex

(Reticularis)

ACTRH

ACTH

Trauma Temp Hypoglycaemia Exercise Stress

Adrenal Physiology

Zona glomerulosa Aldosterone

Na+ & water retention.

K+ secretion.

Zona fasiculata Cortisol and corticosterone

Gluconeogenesis

Proteolysis

Lipolysis

RBCs, plts, neuts.

Gastric acid & ulcers.

Reactivity to catecholeamines.

Reduced collagen & osteoporosis.

Anti-inflammatory.

Immunosuppression. Zona reticularis

Androgens (& small amount of

cortisol)

ACTH K+ RAAS

Hypoadrenalism ! Causes

! Clinical features

! Diagnosis

!  Treatment

Causes

!  Secondary

!  Mass lesions – pituitary adenomas

!  Pituitary surgery / radiation

!  Isolated ACTH deficiency (autoimmune or genetic)

!  Infiltrative lesions

!  Infarction (Sheehans)

!  TBI

!  Tertiary !  Chronic high dose

glucocorticoid therapy !  Mass lesions !  Radiation !  Infiltrative lesions – e.g.

sarcoidosis !  TBI !  Infections

!  Primary Adrenal Insufficiency

!  Idiopathic / Autoimmune

!  Infectious adrenalitis

!  Bilateral adrenal infarct

!  Metastatic disease

!  Drugs

Clinical Features ! Depends on rate & extent of loss of adrenal

function, whether mineralocorticoid production is preserved and degree of stress. !  Adrenal crisis

!  Chronic primary adrenal insufficiency

!  Secondary or tertiary adrenal insufficiency

Clinical Features ! Adrenal Crisis

!  Occurs in:

!  Undiagnosed primary adrenal insufficiency subject to serious infection/stress.

!  Known primary adrenal insufficiency who does not take sufficient glucocorticoid during infection/stress.

!  Bilateral adrenal infarct/haemorrhage.

!  Less frequently with secondary/tertiary adrenal insufficiency.

!  Abrupt withdrawal of glucocorticoid therapy.

Clinical Features !  Adrenal Crisis:

!  Features: !  Shock (predominant feature) !  Anorexia, N&V, weight loss. !  Weakness, fatigue, lethargy, confusion, coma. !  Abdo pain. !  Hypoglycaemia. !  Fever. !  Hyponatraemia, hyperkalaemia, hypercalcaemia,

eosinophilia. !  Hyperpigmentation or vitiligo. !  Autoimmune endocrine deficiencies e.g. hypothyroid

Clinical Features ! Chronic Primary Adrenal Insufficiency

!  May have features of glucocorticoid, mineralocorticoid and androgen deficiency.

!  Insidious onset

!  Non-specific features.

!  Difficult to diagnose.

Clinical Features - CPAI

Symptom   Frequency  (%)  Weakness,  )redness,  fa)gue   100  Anorexia   100  GI  symptoms   92  

Nausea   86  

Vomi)ng   75  

Cons)pa)on   33  

Abdo  pain   31  

Diarrhoea   16  Salt  craving   16  Postural  hypotension   12  Muscle/joint  pains   6-­‐13  

Sign  Weight  loss   100  Hyperpigmenta)on   94  Hypotension   88-­‐94  Vi)ligo   10-­‐20  Auricular  calcifica)on   5  

Laboratory  Abnormality  

Electrolyte  disturbance   92  

Hyponatraemia   88  

Hyperkalaemia   64  

Hypercalcaemia   6  

Azotaemia   55  

Anaemia   40  

Eosinophilia   17  

Clinical Features !  Secondary or Tertiary Adrenal Insufficiency

!  Similar to chronic primary adrenal insufficiency with the following exceptions:

!  Hyperpigmentation not present as ACTH not increased.

!  Dehydration not present, and less hypotension.

!  Hyperkalaemia not present, reflecting presence of aldosterone.

!  Less GI symptoms.

!  Hypoglycaemia more common.

!  Manifestations of pituitary/hypothalamic tumour.

Diagnosis !  Three stage process:

1.  Demonstrating inappropriately low cortisol

2.  Determine if cortisol deficiency is independent/dependent of ACTH deficiency + evaluation of mineralocorticoid secretion in pts without ACTH deficiency.

3.  Seeking treatable cause of the primary disorder.

Diagnosis – Low Cortisol !  Serum cortisol conc

!  Morning serum cortisol conc !  Low is strongly suggestive of adrenal insufficiency

!  Morning salivary cortisol conc !  For screening

!  Afternoon/night serum cortisol !  No value

!  Urinary cortisol !  Low in adrenal insufficiency, but can be low-normal in partial

insufficiency. Thus, unsuitable for screening.

!  Short ACTH stimulation test

Diagnosis – Level of defect ! Need to measure basal plasma ACTH, renin and

aldosterone conc. !  If primary, ACTH high. Will have high renin, low

aldosterone, raised K+ and decreased Na+.

!  If secondary/tertiary, ACTH low. Renin and aldosterone usually unaffected.

!  Prolonged ACTH test will help distinguish between primary and secondary/tertiary.

! Differentiation between secondary and tertiary by ACTRH – although not really important.

Diagnosis - Aetiology !  Pituitary CT or MRI.

! Abdominal CT.

! CXR, urine culture for TB.

! CT directed percutaneous fine needle aspiration of enlarged adrenal glands.

Treatment – Adrenal Crisis !  Emergency Measures

!  Large IV access !  Bloods – U&E, glucose, cortisol, ACTH. !  Saline 2000 – 3000ml !  Dexamethasone 4mg IV BD (does not affect cortisol measurement)

or hydrocortisone 100mg QDS. !  Supportive measures

!  Subacute Measures !  Continue saline for 24 – 48 hours !  Treat precipitants !  Perform short ACTH stimulation test !  Determine type of insufficiency. !  Taper glucocorticoids over 1-3 days !  Begin mineralocorticoid replacement with fludrocortisone.

Treatment – Chronic Adrenal Insufficiency !  Glucocorticoid Replacement

!  Dexamethasone 0.25-0.75mg or prednisolone 2.5-7.5mg PO, supplemented with hydrocortisone 5-10mg in afternoon PRN.

!  Alternatively, hydrocortisone 15-20mg OM & 5-10mg in afternoon.

!  Monitor ACTH.

!  Mineralocorticoid Replacement !  Fludrocortisone 0.05 – 0.2mg PO. !  Liberal salt intake. !  Monitor postural BPs, HR, oedema, K+, and renin.

!  Androgen Replacement !  Dehydroepiandrosterone 25-50mg PO in women.

!  Other !  Patient education !  Medic–alert bracelet

Thank you!