by West Medical Ward King Edward Medical University , Lahore, Pakistan.

1. Prof. Tariq Waseem
Prof. Tariq Waseem

3.  A 22 years old male presents with c/o severe
headache off & on, palpitations and sweating
while carrying out his daily activities.
 He also complains of having a sense of
impending doom and generalized weakness
of his body along with trembling of his hands
 His co-workers also noticed marked blushing
of his face during such episodes.
 He was diagnosed HTN one year ago and is
using four antihpertensive drugs but his BP is
rarely on target.
Prof. Tariq Waseem

4. Prof. Tariq Waseem

5.  On physical examination:
 BP= 210/140
 Pulse…92/min regular
 Temp…98.6 F
 Other positive findings include:
 Pallor…+ve
 Tremors…present
 Systemic examination was unremarkable
Prof. Tariq Waseem

6.  CBC.showing Hb..15g/dl
 TLC…18000
 Serum Na+…139meq/l
 Random blood glucose..200 mg/dl
 Normal T3, T4, TSH
 Normal ECG and echo
Prof. Tariq Waseem

7.  Rare but fatal catecholamines producing
tumor
 Incidence: 2-8/million people/year
 Account for 5% of adrenal incidentaloma
 Rule of 10s:
 10% extra-adrenal, 10% bilateral, 10% familial,
10% malignant
 Aside from catecholamines, it can also
secrete dopamine, ACTH, PTH, calcitonin,
VIP
Prof. Tariq Waseem

8.  0.01-0.1% of HTN population
 Found in 0.5% of those screened
 M = F
 3rd to 5th decades of life
 Rare, investigate only if clinically
suspicion:
 Signs or Symptoms
 Severe HTN, HTN crisis
 Refractory HTN (> 3 drugs)
 HTN present @ age < 20 or > 50 ?
 Adrenal lesion found on imaging (ex. Incidentaloma)
Prof. Tariq Waseem

9.  The five P’s:
 Pressure (HTN) 90%
 Pain (Headache) 80%
 Perspiration 71%
 Palpitation 64%
 Pallor 42%
 Paroxysms
 The Classical Triad:
 Pain (Headache), Perspiration, Palpitations
 Lack of all 3 virtually excluded diagnosis of pheo in a
series of > 21,0000 patients
Prof. Tariq Waseem

10.  Plasma Fractionated free metanephrine
levels
 Urinary fractionated metanephrines and
metabolites
 Serum chromogranin A
 Clonidine suppression test
 Urinary VMA levels
 Additional tests
include…erythrocytosis,leukocytosis,hypergly
cemia,hypercalcemia,sometimes raised ESR
Prof. Tariq Waseem

11.  Physical or emotional stress
 Sleep apnea
 Drugs….MAO inhibitors, TCA’s, beta blockers,
amphetamine, levodopa, methyldopa,
labetalol,buspirone, phenoxybenzamine
Prof. Tariq Waseem

12. Prof. Tariq Waseem
Silent 8 cm pheo

13.  CT/ MRI abdomen
 Nuclear imaging….123 meta-iodobenzyl-
Guanidine
 PET scanning…18FDG-PET
 Somatostatin receptor imaging…111in-
labeled octreotide
Prof. Tariq Waseem

14.  SURGERY…treatment of choice for all
pheochromocytomas ( laproscopic
adrenalectomy, open laprotomy)
 Careful pre-operative management to control
blood pressure, correct fluid volume and
prevent intra-operative hypertensive crises
 MEDICATION therapy….for preop
management, acute hypertensive crises and
as a primary therapy for metastatic
pheochromocytoma.
Prof. Tariq Waseem

15.  Adrenergic blocking agents
 Minipress to BP
 Beta blocking agents
 Inderal to Heart Rate, BP & force of contraction
 Diet
 High in vitamin, Mineral, Calorie, No Caffeine
 Sedatives
Prof. Tariq Waseem

16.  Combined  +  blockade
 Phenoxybenzamine
 Selective 1-blocker (ex. Prazosin)
 Propanolol
 Metyrosine
 Calcium Channel Blocker (CCB)
 Nicardipine
Prof. Tariq Waseem

17. GIVE REGITINE AND NIPRIDE
TO PREVENT HYPERTENSIVE
CRISIS
Prof. Tariq Waseem

18.  Most cases can stop all BP meds postop
 Postop hypotension: IV crystalloid
 HTN free: 5 years 74% 10 years 45%
 24h urine collection 2 wk postop
 Surveillance:
 24h urine collections q1y for at least 10y
 Lifelong f/up
Prof. Tariq Waseem

19.  All the complications of severe hypertension
 Catecholamine induced cardiomyopathy
 Severe heart failure and cardiovascular
collapse
 Sudden death due to cardiac arrhythmias
 ARDS
 Hypertensive crises with sudden blindness
and CVA’s…not uncommon
Prof. Tariq Waseem

20.  -blockade
 Selective 1-blockers (Prazosin, Terazosin, Doxazosin)
1st line as less side-effects
 Phenoxybenzamine: more complete -blockade
 -blocker
 CCB, ACE-I, etc.
 Nuclear Medicine Rx:
 Hi dose 131I-MIBG or 111indium-octreotide depending on
MIBG scan or octreoscan pick-up
 Sensitize tumor with Carboplatin + 5-FU
Prof. Tariq Waseem

21.  Diagnosis with 24h urine collections and
MRI
 No stimulation tests, no MIBG if pregnant
 1st & 2nd trimester (< 24 weeks):
 Phenoxybenzamine + blocker prep
 Resect tumor ASAP laprascopically
 3rd trimester:
 Phenoxybenzamine + blocker prep
 When fetus large enough: cesarian section followed by
tumor resection
Prof. Tariq Waseem

23.  A 16 yr old girl was brought to medical OPD
with C/O increased facial hair and acne. Her
mother is concerned about delayed menarche
and not having a smart feminine figure. On her
last visit to physician she was told to watch her
BP which was recorded 140/95 mmHg.
 On examination, pt. is having male type hair
pattern along with ambiguous genitalia.

24.  Congenital adrenal hyperplasia (CAH) is a
group of inherited autosomal-recessive
disorders in which a genetic defect results in
the deficiency of an enzyme essential for
synthesis of cortisol and, at times,
aldosterone.

25.  Reduced cortisol secretion and resulting absence of
feedback pituitary inhibition increases ACTH to
maintain adequate cortisol causing adrenal
hyperplasia.
 There are several forms of CAH, the most common of
which is 21-hydroxylase (21-OH) deficiency, occurring
in over 90% of all cases.

26. Cholesterol
Pregnenolone
17-Hydroxy-
pregnenalone
17-Hydroxy-
progesterone
11-Deoxycortisol
Cortisol
desmolase
17--hydroxylase
11--hydroxylase
21-hydroxylase
3--OH-
dehydrogenase
Androgens
Ambiguous, no androgens, salt-
wasting
Ambiguous, low androgens,
hypertension
Virilized, high androgens,
lethal
Virilized, high androgens, salt-
wasting
Virilized, high androgens,
hypertension
HGSS: Carey: Figure 5.2 Genitalia, androgen level, medical symptomKey:

27. Glucocorticoi
d
Cholesterol
(mitochondrial)
Pregnenolone
17OH-
Pregnenolone
17OH-
Progesterone
Cortisol
Progesterone
11-
deoxycorticosterone
Aldosterone
Mineralocorticoi
d
P-45021
Dehydroepiandrosterone
Androstenedione
(Testosterone
)
Oestradiol DHT
Androgen
ACTH
_
++
11-
deoxycortisol

29.  If severe, CAH presents at birth with sexual
ambiguity or adrenal failure ( collapse,
hypotension, hypoglycemia) sometimes with a
salt losing state( hypotension, hyponatremia).
 In females, clitoral hypertrophy, urogenital
abnormalities, and albioscrotal fusion are
common but syndrome may be unrecognized.
Milder cases present in adult life only with
primary amenorrhea.
 In males. Precocious puberty with hirsutism is
a later presentation,

30.  A profile of adrenocortical hormones is measured
before and 1hr after ACTH administration.
 Basal ACTH levels…are raised
 17-hydroxyprogesterone levels…are increased
 Urinary 17-ketosteroids are increased
 Androstenedione levels…are raised

31. Glucocorticoid replacement / Androgen Suppression
 Adults: usually dexamethasone, larger dose at bed-
time to suppress ACTH
 Beware of over-replacement and monitor :
17--progesterone (testosterone, cortisol, ACTH etc)
growth, bone age, BP, U&E, skin; osteoporosis scans
in adults

32. Correction of salt-wasting
 9-a-fludrocortisone 100-400mcg/day (long-
acting)
 Sodium chloride supplements until able to select
saltier foods
 Monitor BP, U&E, oedema (renin)

33. Corrective surgery to genitalia
Genetic counselling & psychological support

35.  A 34 yr old male presents in ER with C/O body
aches, episodic muscle weakness, numbness
and tingling of the limbs for last 5 days.
Symptoms worsened over 2 days leading to
severe muscle spasms.
 He also has polyuria and polydipsia especially
at night. He has frequent episodic headache
and Palpitation.
 He was diagnosed HTN last year and is on Beta
Blockers with poor control.

36.  B.P 160/100
 Pulse…86/ min, regular
 Respiratory rate..18/min
 No peripheral edema.
 Systemic examination was unremarkable

37.  CBC , LFT’S, RFT’S…Normal
 Serum potassium..2.1meq/L, serum
sodium..156meq/l
 Urinary postassium..> 32mmol/day
 What’s the diagnosis ?

38.  Primary Hyperaldosteronism
 Conn’s Syndrome

39.  Excessive secretion of aldosterone causes
 Increased Sodium reabsorbtion,
 Increased loss of potassium
 Increased loss hydrogen ions
 Hypokalemia and Hypertension.
 Peak incidence between 30-60yrs of age.

40.  May be primary ( autonomous) as in primary
adrenal hyperplasia, adrenal adenoma,
carcinoma
 Secondary hyperaldosteronism…associated
with renovascular hypertension, cirrhosis,
rennin producing tumours, pregnancy.
 Testing for primary hyperaldosteronism should
be done for all hypertensive patients with
hypokalemia.

41.  Frequent urination
 Increased thirst
 Weakness and fatigue
 Headache
 Muscle cramps
 Tingling in fingers
 Transient paralysis
 Palpitations
 Hypertension

42.  Hypokalemia ( Without Diuretic therapy)
 Urinary potassium loss..>30mmol/day
 Plasma aldosterone:rennin ratio
(ARR)…increased
 Elevated plasma aldosterone level (PA…that
are not suppressed with 0.9% saline ( 2L over
4hrs) or fludrocortisone administration.

43.  Suppressed plasma rennin activity
 18-hydroxycorticosterone level..>100ng/dl seen in
adrenal neoplasms.
 Adrenal vein sampling..to differentiate unilateral or
bilateral aldosteronism
 Posture stimulation test
 Imaging…CT abdomen

44.  Control of blood pressure.
 Conn’s syndrome(adenoma ), unilateral
aldosterone secreting adrenal tumours…treated
by laproscopic adrenalectomy though long term
therapy with spironolactone ( aldosterone
antagonist) or eplerenone is an option.

45.  Bilateral adrenal hyperplasia..best treated with
spironolactone or eplerenone.
 Secondary hyperaldosternism…ACE’s and ARB’s,
spironolactone.

47. Questions?
Prof. Tariq Waseem