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Adrenal

Disorders
Dr Jawwad Anis Khan
MBBS,MCPS,FCPS

Adrenal Gland

Two in number
Weighs 3-5 gms each
Rich blood supply
Adrenal consists of outer cortex.
1.

2.

3.

Zona Glomerulosa :
(Mineralocorticoid)
Zona Fasiculata :
(Glucocorticoid)
Zona Reticularis :

Aldosterone
Cortisol
DHEA (Androgen)

Inner Medulla produces catecholamines.

Adrenal gland

Adrenal gland

Adrenal gland (contd..)


Functions of Aldosterone: (Zona
Glomerulosa)
(Acts on renal tubules, intestines, sweat gland)

Sodium reabsorption
K+ excretion
H+ excretion
Water reabsorption Increase ECF volume
Increase blood volume & maintain blood
pressure

Adrenal gland (contd..)


Functions of Cortisol: (Zona Fasciculata)

Effects on Carbohydrates, protein & fat


metabolism.
During acute trauma, exercise, infection,
operations and other stresses increased
cortisol increased glucose & fatty acid
releases for energy.
Anti inflammatory actions
Maintain adequate vascular tone during stress.

Adrenal gland (contd..)


Functions of adrenal androgens:
(Zona Reticularis)

Early development of male sex


organs during childhood.
Growth of pubic & axillary hairs
Promote protein synthesis

Adrenal Disorders
Disorders of adrenal Cortex:
Adrenal hypofunction Addisons

disease
Adrenal hyperfunction :

Cushing syndrome (Mainly hypercortisolism)


Conns syndrome (Hyperaldosteronism)

Congenital Adrenal Hyperplasia (CAH)

Disorders of adrenal medulla:

Pheochromocytoma

Addisons Disease
(Primary Adrenal
Insufficiency)

Primary hypoadrenalism caused by


destruction of adrenal cortex by any
process producing deficiency of
aldosterone, cortisol and adrenal
androgens.
Secondary hypoadrenalism is caused by
deficiency of ACTH.
Uncommon disorder, occurs between 20
50 yrs.
Common in females 3:1.

Addisons Disease
(contd..)
Etiology:

Autoimmune
Tuberculosis
Glucocorticoid treatment
Surgical bilateral adrenalectomy
Secondary tumour deposits
Fungal disorders Histoplasmosis
Haemochromatosis, Amyloidosis,
Sarcoidosis,

Addisons Disease
(contd..)
Etiology (Contd):
Adrenal haemorrhage : septicaemia,
anticoagulant therapy, waterhouse
Friderichsen syndrome, bleeding disorders.
Radiation therapy.

Clinical Features

Generalized weakness, fatigue, lethergy.


Anorexia, weight loss, nausea, vomiting.
Dizziness, postural hypotension,
dehydration, shock.
Muscle, joint pain, fever.
Loss of body hairs, menstrual
abnormalities.
Hyper pigmentation
Depression.

Laboratory
Investigations

Plasma Cortisol Low to absent.


Plasma ACTH High ( in primary
hypoadrenalism)
ACTH Stimulation Test:

Morning plasma cortisol


ACTH ( Cosyntropin) 250 mg I/M or I/V
Second plasma cortisol level after 30, 60 mins.
Cortisol level fail to rise appropriately after
ACTH suggestive of Addison disease.

Laboratory Investigations
(Contd..)

Hyperkalaemia, hyponatraemia.
Increased urea
Hypoglycaemia
Mild anaemia, Neutropenia,
Eosinophilia.
Anti adrenal antibodies.

Radiological
Investigations

Chest X-ray
R/O Tuberculosis
Abdomen X-ray
R/O
Tuberculosis
Ultrasound abdomen
CT abdomen

Cushings Syndrome
(Hypercortisolism)

Cushings syndrome refers to


manifestations due to excessive
glucocorticoids in the body.
Generally affect young ( mean age 33
years)
Common in females 5 :1

Cushings Syndrome
(Hypercortisolism)

Etiology:

ACTH hypersecretion by pituitary ( Cushing


disease) (Pituitary adenomas)
ACTH hypersecretion by ACTH producing
tumors (small cell carcinoma lungs, carcinoid
tumors, Thymus, pancreas)
Excessive cortisol secretion by adrenal
neoplasm (Adrenal adenoma, carcinoma)
&
hyperplasia
Prolonged Corticosteroid treatment

Clinical Features of
Cushings Syndrome
Effects of Glucocorticoid Excess

Central obesity, moon face, dorsocervical


fat pad, (buffalo hump), supraclaviculer
fat pad, protuberant abdomen.
Muscle waisting, weakness, fatigue.
Thin skin, easy bruisability, purple striae,
poor wound healing, osteoporosis.
Hypogonadism, menstrual abnormalities,
decrease libido.
Psychosis, depression, impaired memory
Children : Slow growth

Clinical Features of
Cushings Syndrome
(Contd..)

Effects of androgen excess: ( if present)

Hirsuitism, acne, virilization in females.


Pseudoprecocious puberty in children.

Effects of ACTH excess: ( if present)

Hyperpigmentation

Effects of mineralocorticoid excess: ( if


present)

Hypertension
Hypokalaemia

Clinical Features of
Cushings Syndrome
(Contd..)

Effects of pituitary tumour: ( if


present)

Headache
Visual field defects
Hypopituitarism

Laboratory
Investigations
Diagnostic approach:

Establish diagnosis of hypercortisolism

Find out the cause of hypercortisolism

Adrenal source
Pituitary pathology ( Cushing disease)
Ectopic ACTH producing tumors.

Laboratory
Investigations
To establish the diagnosis of
hypercortisolism:

Plasma cortisol:
In normal individuals:

Morning samples at 0800 Hrs:


Evening samples at 2000 Hrs:

10 28 ug/dl
5 10 ug/dl

In Cushing syndrome:

Plasma cortisol high


Loss of diurnal variation
High level also found in stress, obesity,
depression, pregnancy, alcoholism
To differentiate we perform DMX Suppression
test.

Laboratory Investigations
(Contd..)
Overnight Dexamethasone Suppression
Test:
Best Screening Test.
2mg oral dexamethasone given at 2300 Hrs.
Blood specimen for plasma cortisol-next
morning at 0800Hrs
Normal individuals: Plasma Cortisol
Suppressed to < 50% of basal level.
Cushing Syndrome: Failure to suppression
of plasma cortisol levels

Laboratory Investigations
(Contd..)

Prolonged Dexamethasone Suppression


Tests.
Low dose: 0.5 mg six hourly for first two days .
If suppressed
: Normal
No suppression : Go with high dose
High dose: 2.0 mg six hourly for next two
days.
If suppressed
: Cushings disease
No Suppression : - Adrenal tumour
- ACTH secreting tumour

Laboratory Investigations
(Contd..)
To find out the cause of
hypercortisolism:
Plasma ACTH:
High plasma ACTH
Pituitary Adenoma. (Cushings disease)
Ectopic ACTH secretion by tumors.
Low plasma ACTH
Adrenal tumors

Other Laboratory
investigations

Urinary free Cortisol : High


Plasma glucose : High due to anti
Insulin effect
of Cortisol.
Serum K : low due to
mineralocorticoid
effects of
cortisol in more severe
ACTH
dependent disease.
Leukocytosis with lymphopenia.

Radiological
Investigation
For localization of tumors
MRI pituitary.
CT Scan chest, abdomen.
CT Scan Adrenals.

Radio isotopic Studies


Distinguish adrenal adenoma
from hyperplaisa.

Primary Hyperaldosteronism Conns Syndrome

Excessive production of aldosterone


Hypertension (usually mild), polyuria,
fatique and weakness.
Hypokalaemia, metabolic alkalosis.
Elevated plasma & urine aldosterone
Suppressed plasma renin.
Adrenal adenoma (75%), bilateral
adrenocortical hyperplasia (25%).
CT/MRI of adrenal.

Congenital Adrenal
Hyperplasia - CAH

1.
2.
3.

In born error of steroid synthesis due to


enzyme deficiency.
Autosomal recessive
21 hydroxylase deficiency & 11 Beta
hydroxylase deficiency.
Decreased Cortisol increased ACTH
increased adrenal activity & hyperplasia.
Deficiency of cortisol & other steroids e.g
Aldosterone.
Clinical effects of increased precursors
virulization.

Congenital Adrenal
Hyperplasia - CAH

May present in three forms.


Simple virulism at birth/neonatal period
(mild deficiency of enzyme & salt wasting
not a feature)
Salt wasting & virulism at birth (severe
enzyme deficiency)
Late onset with hirsutism and infertility.

Elevated ACTH, 17-0H progesterone,


progesterone, testosterone, DHEA
(dehydroepi- androsterone)

Pheochromocytoma

Catacholamine secreting tumors of adrenal


medulla.
Rough rule of ten.
10% cases Extra adrenal (along
sympathetic nervous chain)
10% cases Children
10% cases Familial
10% cases Bilateral adrenal involvement
10% cases Metastatic
10% cases Extra abdominal (Thorax,
Bladder, brain)
10% cases No hypertension.

Pheochromocytoma

Paroxysmal or sustained hypertension.


Sometimes postural hypotension
Episodes of perspiration, palpitation.
Headache, Anxiety, nausea, chest or
abdominal pain, dyspnea, visual
disturbances.
Hypermetabolism with normal thyroid tests.
Hyperglycaemia due to anti insulin effect of
catacholamines.
Elevated urinary catecholamines & their
metabolites e.g. metanephrines & VMA.
CT/MRI can confirm & localize the tumour.

SUMMARY

Adrenal gland consist of outer cortex and


inner medulla
Adrenal cortex secrete Aldosterone,
Cortisone and DHEA (Androgen)
Addisons disease is hypofunction of
adrenal gland. Causes may include
Autoimmune, Tuberculosis of adrenals,
Glucocorticoid treatment, Surgical
adrenalectomy, Secondary tumour
deposits, Haemochromatosis, Amyloidosis
and Fungal disorders of adrenal gland.

SUMMARY

Helpful investigations include plasma


cortisol and ACTH levels, ACTH
stimulation test, Antiadrenal antibodies,
Ultrasonography & CT scan of adrenal.
Cushings syndrome is hypercortisolism.
Causes may include ACTH hypersecretion
by pituitary (Cushings disease due to
pituitary adenoma is the commonest
cause), ACTH producing tumours in the
body, Adrenal adenoma or carcinoma and
prolonged corticosteroid treatment.

SUMMARY

Helpful investigations include plasma Cortisol


and ACTH levels, Dexamethasone suppression
tests, Ultrasonography & CT scan of adrenal,
CT & MRI of pituitary gland, CT scan of chest
Conns syndrome is due to hyper aldosteronism
Congenital adrenal hyperplasia CAH is inborn
error of steroid synthesis due to enzyme
deficiency resulting into decreased Cortisol,
Aldosterone levels and increased ACTH & 17
OH progesterone levels
Pheochromocytomas are Catacholamine
secreting tumours of adrenal glands

Interactive Session

QUESTIONS

What are functions of Aldosterone,


Cortisol and Androgens

QUESTIONS

What is Addisons disease. Tell its


causes and clinical features

QUESTIONS

Tell relevant investigations for


diagnosis of Addisons disease

QUESTIONS

What is Cushings syndrome

QUESTIONS

What is the etiology of Cushings


syndrome

QUESTIONS

What are the clinical features of


Cushings Syndrome

QUESTIONS

Tell relevant investigations for


diagnosis of Cushings syndrome

THANK YOU