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?
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
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
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
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?
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?
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
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?
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
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.
63.
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.