4. CLINICAL FEATURES
SYMPTOMS SIGNS
Weight loss
Malaise
Weakness
Fever
Anorexia
Nausea
Diarrhea
Abdominal pain
Constipation
Depression
Confusion
Myalgia
Joint/back pain
Impotence/amenorrhea
sycope
Loss of weight
General wasting
Pigmentation(specially new scars
and palmar creases)
Buccal pigmentation
Postural hypotension
Loss of body hair
Dehydration
5. INVESTIGATIONS
Single cortisol measurements
random cortisol below 100mmol/L during day-highly suggestive
Short ACTH Stimulation test
absent or impaired cortisol response confirms the presence of hypoadrenalism.
9:00 hrs. plasma ACTH level
high level with low or low normal cortisol confirm primary hypoadrenalism
electrolytes and urea
show hyponatremia, hyperkalemia and high urea.
Blood glucose – low
Adrenal antibodies –present
Chest and abdominal X-rays –show evidence of TB and calcification
Plasma renin activity – high ←high aldosterone
hypercalcemia and anaemia
6. MANAGEMENT
ACUTE CASES LONG TERM
Assuming normal CVS Activity
• 1L of 0.9% saline given over 30-60 min with
100mg of i.v. bolus hydrocortisone.
• Subsq req. of several litres of saline within 24h
+ hydrocortisone 100mg IM 6 hourly , until
until patient is stable
• Oral replacement Medication
hydrocortisone 20mg 8 hourly reducing 20-
30 mg in divided doses over few days .
• long term therapy with replacement
glucocorticoid and mineralocorticoid.
7. CONGENITAL ADRENAL HYPERPLASIA
Autosomal recessive deficiency of an enzyme in the cortisol synthetic
pathways.
Six major types ; most common is 21-hydroxylase deficiency
1 in 15000 live birth
Cortisol lvls are reduced → feedback → increased ACTH secretion → adrenal
hyperplasia
Diversion of steroid precursors into androgenic steroid pathways occurs.
17-OH progesterone ,androstenedione and testosterone lvs are increased →
virilization.
Aldosterone synthesis may be impaired → salt wasting .
8. CLINICAL FEATURES
Presents at birth
Cortisol deficiency –
Hypoglycemia ,inability to withstand stress ,vasomotor collapse, hyperpigmentation,
apneic spells, muscle weakness & fatigue
Aldosterone deficiency-
hyponatremia, hyperkalemia, vomiting, urinary sodium wasting, salt craving,
acidosis, failure to thrive, volume depletion, hypotension, dehydration, shock ,
diarrhea.
Androgen excess –
Ambiguous gentilia, virilization of external genitilia, hirsutism, early appearance of
pubic hair, penile enlargement, excessive height gain and skeletal advance.
Late onset CAH – normal genitilia , have acne , hirsutism, irregular menses/
amenorrhoea.
9. INVESTIGATIONS
Profile of adrenocorticoid hormones drawn up before and 1hr after ACTH
Administration
17-OH progesterone levels – increased
Urinary pregnanetriol excretion – increased
Androstenedione levels – raised
Basal ACTH Levels – raised
10. PRIMARY HYPERALDOSTERONISM
Disorder of adrenal cortex
Increased mineralocorticoid secretion from the adrenal cortex.
Excess aldosterone production → sodium retention , potassium loss +
combination of hypokalemia and hypertension.
Account for 5-10% of all hypertension.
Highest chances of detecting in patients :
Under 35 yrs. especially without a family history of HTN
With accelerated HTN
With hypokalemia before diuretic therapy
Resistant to conventional antihypertensive therapy
With unusual symptoms.
11. ENDOCRINE CASES OF HYPERTENSION
Excessive renin , and thus angiotensin II pdtn.
Renal artery stenosis
Renin secreting tumors
Excessive production of catecholamines
pheochromocytoma
Excessive growth hormone(GH) production
Acromegaly
excessive aldosterone production
Adrenal adenoma (Conn Syndrome)
Idiopathic adrenal hyperplasia
Excessive production other mineralocorticoids
cushing syndrome
Congenital adrenal hyperplasia
12. CLINICAL FEATURES
Hypertension
Hypokalemia
Lack of edema
Metabolic alkalosis
Mild hypernatremia, hypomagnesmia
↑ GFR , polyuria , proteinuria, CRF
Muscle weakness & Cramps
LVH, MI, CVA, AF
14. PHAEOCHROMOCYTOMA
Very rare tumors of sympathetic nervous system.
Secrete catecholamines, noradrenaline , adrenaline and their
metabolites
arise in adrenal medulla
Maye be associated with MEN 2 syndromes and the von Hippel –
Lindau syndrome.
Oval groups of cells occur in clusters and stain for chromogranin
A.
25% are multiple and 10% malignant
16. INVESTIGATIONS
Measurement of urinary catecholamines and metabolites
useful screening test, normal levels on three 24h collections of metanephrines
virtually exclude the diagnosis.
Resting plasma metanephrines – raised
Plasma chromogranin A – raised
Clonidine suppression test
CT/MRI Scans – localize the tumours
Scanning with MIBG (meta –iodobenzylguanidine)
Gentic testing – MEN2, VHL, SDHB and SDHD mutations should be performed in all
people with confirmed cases.