Adrenal Disease

Question 1

what is adrenal insufficiency?

Answer 1

is a condition in which there is destruction of the adrenal cortex and subsequent reduction in the output of adrenal hormones, ie glucocorticoids (cortisol) and/or mineralocorticoids (aldosterone).

Question 2

what are the 2 types of adrenal insufficiency?

Answer 2

——Primary insufficiency (Addison’s disease) - there is an inability of the adrenal glands to produce enough steroid hormones. The most common cause for this in the developed world is autoimmune disease.

——-Secondary insufficiency - more common than primary (suppression of the hypothalamic-pituitary axis (of which the most common is exogenous steroid use))——there is inadequate pituitary or hypothalamic stimulation of the adrenal glands.

Question 3

Clinical features of addisons?

Answer 3

NB\_\_\_\_\_
Tiredness (95%)
• Weakness (95%)
• Anorexia (95%)
• Weight loss (90%)
• hyperPigmentation (buccal, palmar creases, new scars—90%) (upto date picture------hyperpigmentation of the skin and increased pigmentation of the distal half of the nails that occurred during the period of adrenal insufficiency. The proximal half of the nails are hypopigmented, a reflection of the reduction in ACTH secretion after the institution of glucocorticoid therapy.)

other______
———-GI complaints—-abdominal pain
postural hypotension
myalgia/arthralgia
——-Electrolyte abnormality- salt craving??
———Psychiatric manifestations — Many patients with severe or long-standing adrenal insufficiency have psychiatric symptoms, including
———–Decreased axillary and pubic hair and loss of libido are common in women, in whom androgen production primarily occurs in the adrenal glands

Question 4

presentation of addisons?

Answer 4

can be dramatic with coma and severe hypoglycaemia (— admit as an emergency) or insidious

Cravings for salt and salty foods such as soy sauce or liquorice

Question 5

associated diseases with addisons?

Answer 5

50% patients with autoimmune Addison’s disease have or will develop another autoimmune disease

(e.g. Graves’ disease, pernicious anaemia)

and 5% of women develop premature ovarian failure

Question 6

investigations for addisons, describe the lab values for 
Na
K
Ca
FBC
Glucose
LFT
Cortisol
ACTH

Answer 6

Sodium - reduced in 90% of newly diagnosed cases of primary adrenal insufficiency.

Potassium - raised in 50% of newly diagnosed cases of primary adrenal insufficiency.

Calcium - raised in 10-20 % of newly diagnosed cases of primary adrenal insufficiency.

FBC - there may be anaemia, mild eosinophilia and lymphocytosis.

Glucose - often low in children.

LFTs - may be raised liver transaminases.

Cortisol - usually reduced:
Levels are highest between 8 am and 9 am when blood test should be taken.

ACTH ———-
Levels are raised in primary insufficiency.
Levels are low or low normal in secondary insufficiency.

Question 7

describe an ACTH stimulation test?

Answer 7

An ACTH stimulation (Synacthen®) test may be required to confirm the diagnosis. ACTH is administered IV or IM, and cortisol levels subsequently measured. The normal response is a rise in cortisol level; in adrenal insufficiency this does not occur.

Question 8

management of addisons?

Answer 8

Management refer to endocrinology.

Treatment usually involves replacing deficient steroids with Both glucocorticoid and mineralocorticoid replacements

Question 9

what advise should you give to patients re addisons and taking steroids?

Answer 9

—Warn patients not to stop steroids abruptly,

—to tell any doctor treating them about their condition and

—-wear Medic-Alert/Medi-Tag bracelet in case of emergency.

—-Double dose of hydrocortisone prior to dental treat- ment or if intercurrent illness (e.g. UrTI)

—–If vomiting, replace hydrocorti- sone po with IM hydrocortisone.

Question 10

Because of the high incidence of other autoimmune disease, those with an autoimmune cause should be screened annually with?

Answer 10

TFTs.
Glucose and HbA1c.
FBC.
Vitamin B12.
Coeliac screen if symptoms suggest.

Question 11

complications of addissons?

Answer 11

adrenal crisis
reduced quality of life (fatigue)
osteoporosis (because of the steroids)

Question 12

prognosos/advise for patients with addisons?

Answer 12

untreated- death
lifelong treatment
if treated- normal health

Question 13

causes of addisons/primary adrenal insufficiency?

causes of anatomical destruction of the gland?

causes if metablic failure in hormone function?

Answer 13

Anatomic destruction of the gland (acute or chronic):

• –Addison’s disease (autoimmune; 85% of cases).
• –Surgical removal.
• —Trauma.
• –Infections - eg, tuberculosis (TB), histoplasmosis, cryptococcosis, HIV, syphilis.
• —Haemorrhage - eg, anticoagulants, Waterhouse-Friderichsen syndrome.
• –Infarction - eg, antiphospholipid syndrome.
• –Invasion - eg, neoplastic, sarcoidosis, amyloidosis, haemachromatosis

Metabolic failure in hormone production:

———-Congenital adrenal hyperplasia - eg, 21-hydroxylase deficiency, 3-beta-hydroxysteroid dehydrogenase deficiency, lipoid hyperplasia.

———-Enzyme inhibition - eg, ketoconazole, fluconazole, etomidate and metapyrone.
Accelerated hepatic metabolism of cortisol - eg, phenytoin, barbiturates, rifampicin.

———–Adrenocorticotropic hormone (ACTH) or glucocorticoid resistance.

———-Cytotoxic agents.

Question 14

functions of Cortisol?

BIG FIB

Answer 14

increases
Blood pressure (upregulates α1-receptors on arterioles􏰇􏰂sensitivity to norepinephrine and epinephrine
􏰂Insulin resistance (diabetogenic) 􏰂Gluconeogenesis, lipolysis, and proteolysis

reduces—–
􏰃Fibroblast activity (causes striae)
􏰃Inflammatory and Immune responses:
—– Inhibits production of leukotrienes and prostaglandins
—- Inhibits leukocyte adhesion 􏰇 neutrophilia
—-Blocks histamine release from mast cells
—-Reduces eosinophils
—–Blocks IL-2 production
Bone formation (decreases osteoblast activity)