case based approach to adrenal disorders for undergraduates

2. Diseases of
Adrenal Glands

3. Questions& LOs
1. What is adrenal gland and its hormones, what are the roles of
adrenal hormones in the body? Can we live without adrenal
glands?
2. What is adrenal insufficiency and its causes and how to
differentiate between primary and secondary types?
3. Discuss clinical presentation, how to diagnose and treat adrenal
insufficiency and its crisis and follow up of the patient
4. What advice should be given to patients receiving glucocorticoid
replacement therapy? Which measures should be taken if surgery
is indicated?
5. What is Cushing syndrome and what is the difference between its
causes? What is the difference between Cushing’s syndrome and
Cushing’s disease?

4. Questions& Los (2)
6. Discuss the clinical presentation, how to reach diagnosis and
how to manage cases with glucocorticoid excess.
7. What are diseases related to mineralocorticoid excess. What
is the difference between primary and secondary
hyperaldosteronism?
8. Pheochromocytoma, clinical picture and management
We will also discuss
• side effects of Glucocorticoid therapy
• what is congenital adrenal hyperplasia?

5. Adrenal Insufficiency
Cushing Syndrome
Hyperaldosteronism
Congenital adrenal
hyperplasia (CAH)
Pheochromocytoma
Glucocorticoid therapy

7. Adrenal Cortex and Medulla

8. Adrenal Cortical Hormones and Zones

9. Role of Adrenal Hormones

10. Steroid hormone synthesis

11. Steroid hormone synthesis

12. Structure and Function of adrenals

13. Diseases of Adrenal glands

14. Case (1)
• 50 years old female patient, married and has 3 daughters, suffers
from gradual onset of weakness, lethergy and easy fatigability, poor
appetite with loss of weight about 15 Kg in one year.
• No fever , no symptoms suggestive if cardiac, chest or abdominal
troubles.
• She also received treatment for hypothyroidism 8 years ago and
controlled. She has normal ESR, serum Na 124 mmol/l, K 5.7
mmol/l, her fasting blood glucose 55mg/dL and CBC shows
eosinophil 8%
• What is the possible diagnosis?
• What is the relation between this condition and hypothyroidism?
• How to reach diagnosis and what is the complication?
• What advice should be given ?

15. Adrenal Insufficiency
• Adrenal insufficiency results from
inadequate secretion of cortisol and/or
aldosterone.
• It is potentially fatal
 Primary
 secondary

16. Clinical Features of Addison’s disease

18. Pigmentation in Addison's disease

19. Causes of primary hypoadrenalism
Causesofprimary
hypoadrenalism
Autoimmune
Tuberculosis
Surgical removal
Hemorrhage/
infarction
Infiltration
Adrenal
Leukodystrophy

20. Investigations
• Single cortisol measurements
• A 09:00 hours plasma ACTH
level is measured, a high level
(>80 ng/L) with low or low
normal cortisol confirming
primary hypoadrenalism.
• Electrolytes and urea
• Blood glucose
• The short ACTH stimulation
test
• Adrenal antibodies
• Chest and abdominal X-rays

21. CT abdomen in Primary adrenal
insufficiency

22. Features suggesting secondary adrenal
insufficiency
• Lack of pigmentation
• Lack of mineralocorticoid deficiency
• Associated features e.g. visual field defects
• Associated endocrine deficiencies e.g. in
panhypopituitarism.
• Acute onset e.g. in pituitary apoplexy

23. Treatment of Adrenal insufficiency

24. Famous people with Addison’s disease

25. Case (2)
• A 27 year old female patient was diagnosed as lupus nephritis.
She was on 60 mg/d prednisolone, and then decreased to 15
mg/d. Three weeks ago, she developed fever, cough and
expectoration. She interrupted her steroid doses, 4 days ago
she suffered severe weakness, fatigue, nausea and vomiting.
She was comatozed, BP 80/60, and no evidence of lupus
activity.
• What is the diagnosis for the last situation?
• How to treat it?

26. Relative potency of Glucocorticoids

27. Adverse effects of
steroid therapy

28. Management of adrenal Crisis

29. Advice to patients on lifelong cortisone
therapy

30. Surgery in patients with glucocorticoid
replacement

31. Case (3)
• A 42 year-old man presents with recently discovered diabetes
mellitus. On physical examination, there is increased facial
fullness and weight gain, proximal muscle weakness.
Abdominal examination revealed purple striae. His blood
pressure 150/95, fasting blood glucose 250 mg/dl, post-
prandial 385mg/dl, serum Na 150 mEq/L, K 3.3 mEq/L. White
blood cell count greater than 12,000/mm.
• What do you think about the diagnosis?
• What laboratory tests and imaging would you order to reach
the proper diagnosis?

34. Causes and Aetiology of Cushing's
syndrome

35. 1- Approach to a suspected case of
Cushing’s syndrome

36. 2-How to reach the root cause of a
confirmed hypercortisolemia

37. Treatment of Hypercortisolism
• According to the cause
• Cortisol hypersecretion should be controlled
prior to surgery or radiotherapy
• Metyrapone, Ketoconazole,
Aminoglutethimide, trilostane & etomidate
infusion (in severe cases).
• Trans-sphenoidal surgery
• Adrenalectomy

38. Improvement after surgery

40. Case (4)
• A 26-year-old man presented with elevated blood pressure
(180/110 seated). Three months ago, his blood pressure was
normal. He was admitted twice in the last month due to
apprehension, severe headache, palpitation and diaphoresis,
flushing. These episodes had an abrupt onset and lasted 10-
15 minutes.
• What do you think the patient primary problem is and explain
the presentation?
• What laboratory tests and imaging would you order to
evaluate this patient?

41. Pheochromocytoma
( clinical features)

42. Pheochromocytoma

43. Synthesis and metabolism of
caticholamines

44. Case (5)

45. Pheochromocytoma

46. Investigation of a suspected case of
pheochromocytoma
• Measurement of urinary catecholamines and
metabolites
• Plasma metanephrines and chromogranin A
• CT and MRI
• Scanning with (131I)metaiodobenzylguanidine
(MIBG)
• Genetic testing

47. Treatment of pheochromocytoma
• Surgery
• Perioperative management
• Phenoxybenzamine then propranolol
• Hydration

49. Endocrine Hypertension
• Acromegaly
• Cushing’s diseasepituitary
• hyperthyroidism
• hypothyroidismThyroid
• Cushing’s syndrome
• Pheochromocytoma
• Primary hyperaldosteronism
• Apparent mineralocorticoid excess
• Some types of Congenital adrenal hyperplasia
Adrenal & related
hormones

50. Endocrine hypertension

51. Mineralocorticoid excess

52. Aldosterone secreting adenoma

53. Investigations of hyperaldosteronism
• Plasma aldosterone : renin ratio (ARR)
• Failure to supress aldosterone with saline or
fludrocortisone
• Decreased ( supressed ) renin
• Inappropriate decrease renal potassium excretion
• CT and MRI
• Adrenal venous sampling

54. Treatment of primary
hyperaldosteronism
• Spironolactone
• Eplerenone
• Amiloride and triamterene
• Calcium channel antagonists
• Surgery( in adenoma)
• Mitotane ( in carcinoma)

55. Congenital adrenal Hyperplasia

56. Ambiguous genitalia

57. Short stature and precocious puberty
due to CAH

58. Non classical CAH

60. Clinical pearls
• Long-term steroid therapy must never be stopped
suddenly
• Any patient receiving steroids, or who has
recently received them (within the last 12
months), requires special control of steroid
medication around the time of surgery.
• Phaeochromocytoma must be excluded before
surgery (for incidentaloma) due to the risk of
perioperative hypertensive or hypotensive crises
• Hirsutism developing before puberty is suggestive
of CAH

61. Clinical pearls (2)
• There is a rare type of hyperaldosteronism that
can be treated with glucocorticoids!
• pigmentation in a case of adrenal insufficiency
point to a primary type.
• Genetic testing for MEN2, VHL, SDHB and SDHD
mutations should be performed in all people with
confirmed phaeochromocytoma or
paraganglioma.
• Patients with pheochromocytoma should be kept
under clinical and biochemical review after
tumour resection, as over 10% of tumours recur

62. Clinical pearls (3)
• pigmentation can occur in subtypes of both
glucocorticoid excess and deficiency .
• The adrenolytic drug mitotane may inhibit
growth of adrenal carcinoma and prolong
survival.
• Nelson syndrome occurs in about 20% of cases
after bilateral adrenalectomy for Cushing's
disease and is characterized by increased
pigmentation.

64. Do not forget that:
1. Adrenal glands are important for life and adrenal crisis may
threaten life
2. Give advice to all adrenal insufficiency patients telling them that
the cortisone tablets are life saving forever and to increase doses
with stress.
3. Cushing disease is commonly of pituitary origin and should be
treated with surgery
4. hyperaldosteronism, is thought to account for 5–10% of all
hypertension.
5. Primary aldosteronism is mostly due to bilateral hyperplasia rather
than adenoma
6. Untreated Cushing syndrome has a very poor prognosis,with
death from hypertension, myocardial infarction, infection and
heart failure.