Addison's syndrome

Question 1

Definition?

Answer 1

Syndrome of disorder of adrenal glands, creating a primary adrenal insufficiency.

Question 2

RF?

Answer 2

• Female
• Adrenocortical auto antibodies
Adrenal haemorrhage

Question 3

ddx?

Answer 3

• CS therapy
• Secondary or Tertiary adrenal insufficiency
• Haemochromatosis
• Hyperthyroidism
• Occult malignancy
• Anorexia nervosa

Question 4

Epidemiology?

Answer 4

Age:
Sex: Women
Ethnicity:

Question 5

Aetiology?

Answer 5

• Autoimmune adrenalitis
• Infectious adrenalitis-TB, CMV, histoplasmosis
• Adrenal haemorrhage-sepsis, DIC, heparin-induced thrombocytopenia
• Infiltrations
• Adrenalectomy
• Impaired cortisol synthesis
• Vitamin B5 deficiency

Question 6

CP?

Answer 6

see table
also crisis
• Hypotension/shock
• Coma
• Fever
• Diarrhoea and vomiting
• Abdominal pain
Hypoglycaemia, hyponatremia, hyperkalaemia, metabolic acidosis

Question 7

Pathophysiology?

Answer 7

• Adrenal gland disoder so cant produce enough cortisol and aldosterone
• ZG-Aldosterone production-binds to Na/K pump in principle cells in DCT-less potassium, increased sodium and water reabsoprtion and B vol/BP
• Binds to ATPase pumps in a-intercalated cells so more protons are excreted in the urine and bicarbonate into the EC space/blood
• ZF-cortisol/glucocorticoids-stress-CRH-ACTH from pituitary gland-binds to nuclear receptors-gluconeogenesis, proteolysis and lipolysis
• ZR-androgens-DHEA-testosterone production-reproductive tissue development and secondary sex characteristics and libido
• Adrenal cortex gets progressively damaged overtime
• Autoimmune destruction of adrenal cortical tissues
• TB-spreads to adrenal glands-inflammation and immune destruction
• Mets carcinoma-spreads
• High functional reserve-symptoms means up to 90% has been destroyed
• Effects
• Less function of aldosterone pumps-more K and H and less Na and water-salt intake higher and signs of hypovolaemia
• Low blood glucose-fatigue
• Overactive pituitary gland due to NF-more proopiomelanocortin-precursor to adrenocorticotropic hormone and melanocyte stimulating hormone-hyperpigmentation
• Androgen decrease-affects women more as they depend on this source more-loss of hair and low libido

Question 8

I-first line?

Answer 8

• Serum electrolytes-hyponatremia, hyperkalaemia, metabolic acidosis, hypercalcaemia
• Blood urea
• FBC
• Serum glucose-hypoglycaemia
• Morning serum cortisol-low (<3mg/dL)

Question 9

I-second line?

Answer 9

• ACTH -high shows primary
• RAAS activity-high renin, low aldosterone
• DHEA
• DHEAS
• Adrenal antibodies
• CT/MRI
• Overnight single-dose metyrapone test
• Metyraponeinhibits11β hydroxylase→ impaired conversion of11-deoxycortisoltocortisol(last step ofcortisol synthesis)
• Measurement of11-deoxycortisolandcortisolafter administration ofmetyrapone:Adrenalinsufficiency is diagnosed if the11-deoxycortisollevel does not exceed70 ng/mLand thecortisollevel is< 5 μg/dL.
Inprimaryadrenalinsufficiency:metyrapone→↓cortisolsynthesis →↑ inCRH/ACTH→ no increase inadrenalsteroidproduction →↓ 11-deoxycortisoland↓cortisol

Question 10

M-addisonian crisis?

Answer 10

• Glucocorticoid (hydrocortisone) and supportive therapy
• Saline and glucose to correct hypoglycaemia and electrolyte disturbances
The5 S’s ofadrenal crisistreatment are:Salt:0.9% saline,Sugar: 50% dextrose,Steroids:100 mghydrocortisoneIV every8 hours,Support:normal salineto correcthypotensionand electrolyte abnormalities,Searchfor underlying disorder

Question 11

M-stable?

Answer 11

• Glucocorticoid (hydrocortisone) and mineralocorticoid (fludrocortisone)
• Increase in times of stress or infection
Androgen replacement if low libido-DHEA

Question 12

P?

Answer 12

• Need therapy for life
• Non-compliance uncomfortable and life-threatening
Lower quality of life

Question 13

C?

Answer 13

• Secondary Cushing’s syndrome
• Osteopenia/osteoporosis
• HT