Page 814 - Textbook of Pathology, 6th Edition

798 CLINICAL FEATURES. Conn’s syndrome is more frequent ETIOPATHOGENESIS. Causes of acute insufficiency are
in adult females. Its principal features are as under: as under:
1. Hypertension, usually mild to moderate diastolic 1. Bilateral adrenalectomy e.g. in the treatment of cortical
hypertension. hyperfunction, hypertension and in selected cases of breast
2. Hypokalaemia and associated muscular weakness, cancer.
peripheral neuropathy and cardiac arrhythmias. 2. Septicaemia e.g. in endotoxic shock and meningococcal
3. Retention of sodium and water. infection producing grossly haemorrhagic and necrotic
4. Polyuria and polydipsia due to reduced concentrating adrenal cortex termed adrenal apoplexy. The acute condition
power of the renal tubules. so produced is called Waterhouse-Friderichsen’s syndrome.
3. Rapid withdrawal of steroids.
Adrenogenital Syndrome (Adrenal Virilism) 4. Any form of acute stress in a case of chronic insufficiency
i.e. in Addison’s disease.
Adrenal cortex secretes a smaller amount of sex steroids than
the gonads. However, adrenocortical hyperfunction may CLINICAL FEATURES. Clinical features of acute adreno-
occasionally cause sexual disturbances. cortical insufficiency are due to deficiency of mineralo-
ETIOPATHOGENESIS. Hypersecretion of sex steroids, corticoids and glucocorticoids. These are as follows:
mainly androgens, may occur in children or in adults: 1. Deficiency of mineralocorticoids (i.e. aldosterone
1. In children, it is due to congenital adrenal hyperplasia in deficiency) result in salt deficiency, hyperkalaemia and
which there is congenital deficiency of a specific enzyme. dehydration.
2. In adults, it is caused by an adrenocortical adenoma or a 2. Deficiency of glucocorticoids (i.e. cortisol deficiency)
carcinoma. Cushing’s syndrome is often present as well. leads to hypoglycaemia, increased insulin sensitivity and
vomitings.
CLINICAL FEATURES. The clinical features depend upon
the age and sex of the patient. B. Primary Chronic Adrenocortical Insufficiency
1. In children, there is distortion of the external genitalia in (Addison’s Disease)
girls, and precocious puberty in boys.
2. In adults, the features in females show virilisation (e.g. Progressive chronic destruction of more than 90% of adrenal
hirsutism, oligomenorrhoea, deepening of voice, hyper- cortex on both sides results in an uncommon clinical
trophy of the clitoris); and in males may rarely cause condition called Addison’s disease.
feminisation. ETIOPATHOGENESIS. Any condition which causes
SECTION III
3. There is generally increased excretion of 17-ketosteroids marked chronic adrenal destruction may produce Addison’s
in the urine. disease. These include: tuberculosis, autoimmune or
idiopathic adrenalitis, histoplasmosis, amyloidosis,
ADRENOCORTICAL INSUFFICIENCY
(HYPOADRENALISM) metastatic cancer, sarcoidosis and haemochromatosis.
However, currently the first two causes—tuberculosis and
Adrenocortical insufficiency may result from deficient autoimmune chronic destruction of adrenal glands, are
synthesis of cortical steroids from the adrenal cortex or may implicated in majority of cases of Addison’s disease.
be secondary to ACTH deficiency. Three types of Irrespective of the cause, the adrenal glands are bilaterally
adrenocortical hypofunction are distinguished: small and irregularly shrunken. Histologic changes,
1. Primary adrenocortical insufficiency caused primarily by the depending upon the cause, may reveal specific features as
disease of the adrenal glands. Two forms are described: acute in tuberculosis and histoplasmosis, or the changes may be
Systemic Pathology
or ‘adrenal crisis’, and chronic or ‘Addison’s disease’. in the form of nonspecific lymphocytic infiltrate as in
2. Secondary adrenocortical insufficiency resulting from idiopathic (autoimmune) adrenalitis.
diminished secretion of ACTH.
3. Hypoaldosteronism characterised by deficient secretion of CLINICAL FEATURES. Clinical manifestations develop
aldosterone. slowly and insidiously. The usual features are as under:
1. Asthenia i.e. progressive weakness, weight loss and
PRIMARY ADRENOCORTICAL INSUFFICIENCY lethargy as the cardinal symptoms.
Primary adrenal hypofunction occurs due to defect in the 2. Hyperpigmentation, initially most marked on exposed
adrenal glands and normal pituitary function. It may develop areas, but later involves unexposed parts and mucous
in 2 ways: membranes as well.
A. Acute primary adrenocortical insufficiency or ‘adrenal 3. Arterial hypotension.
crisis’. 4. Vague upper gastrointestinal symptoms such as mild loss
B. Chronic primary adrenocortical insufficiency or of appetite, nausea, vomiting and upper abdominal pain.
‘Addison’s disease’.
5. Lack of androgen causing loss of hair in women.
A. Primary Acute Adrenocortical Insufficiency 6. Episodes of hypoglycaemia.
(Adrenal Crisis)
7. Biochemical changes include reduced GFR, acidosis,
Sudden loss of adrenocortical function may result in an acute hyperkalaemia and low levels of serum sodium, chloride and
condition called adrenal crisis. bicarbonate.