Conn's syndrome

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Main article: Primary aldosteronism

Conn's syndrome
Classification and external resources

ICD-10
ICD-9
DiseasesDB
MeSH

Aldosterone
E26.0
255.12
3073
D006929

Conn's syndrome is an aldosterone-producing adenoma. Conn's syndrome is named

after Jerome W. Conn (19071994), the American endocrinologist who first described the
condition at the University of Michigan in 1955.[1]

Contents

1 Causes
2 Pathogenesis
3 Diagnosis
4 Differential diagnosis
5 Therapy
6 Prognosis
7 See also

8 References

Causes
Primary hyperaldosteronism has many causes, including adrenal hyperplasia and adrenal
carcinoma.[2]

The syndrome is due to:

bilateral idiopathic adrenal hyperplasia 70%

unilateral idiopathic adrenal hyperplasia 20%
aldosterone-secreting adrenal adenoma (benign tumor, < 5%)
rare forms, including disorders of the renin-angiotensin system

Pathogenesis
Aldosterone enhances exchange of sodium for potassium in the kidney, so increased
aldosteronism will lead to hypernatremia (elevated sodium level) and hypokalemia (low
blood potassium). Once the potassium has been significantly reduced by aldosterone, a
sodium/hydrogen pump in the nephron becomes more active, leading to increased
excretion of hydrogen ions and further exacerbating the elevated sodium level resulting in
a further increase in hypernatremia. The hydrogen ions exchanged for sodium are
generated by carbonic anhydrase in the renal tubule epithelium, causing increased
production of bicarbonate. The increased bicarbonate and the excreted hydrogen combine
to generate a metabolic alkalosis.
The high pH of the blood makes calcium less available to the tissues and causes
symptoms of hypocalcemia (low calcium levels).
The sodium retention leads to plasma volume expansion and elevated blood pressure. The
increased blood pressure will lead to an increased glomerular filtration rate and cause a
decrease in renin release from the granular cells of the juxtaglomerular apparatus in the
kidney. If a patient is thought to suffer from primary hyperaldosteronism, the
aldosterone:renin activity ratio is used to assess this. The decreased renin levels and in
turn the reactive down-regulation of angiotensin II are thought to be unable to downregulate the constitutively formed aldosterone, thus leading to an elevated [plasma
aldosterone:plasma renin activity] ratio (lending the assay to be a clinical tool for
diagnostic purposes).
Aside from hypertension, other manifesting problems include myalgias, weakness, and
chronic headaches. The muscle cramps are due to neuron hyperexcitability seen in the
setting of hypocalcemia, muscle weakness secondary to hypoexcitability of skeletal
muscles in the setting of low blood potassium (hypokalemia), and headaches which are
thought to be due to both electrolyte imbalance (hypokalemia) and hypertension.
Secondary hyperaldosteronism is often related to decreased cardiac output, which is
associated with elevated renin levels.

Diagnosis
Measuring aldosterone alone is not considered adequate to diagnose primary
hyperaldosteronism. The screening test of choice for diagnosis is the plasma

aldosterone:plasma renin activity ratio. Renin activity, not simply plasma renin level, is
assayed. Both renin and aldosterone are measured, and a ratio greater than 30 is
indicative of primary hyperaldosteronism.[3][4]

Differential diagnosis
Hyperaldosteronism can be mimicked by Liddle syndrome, and by ingestion of liquorice
and other foods containing glycyrrhizin. In one case report, hypertension and
quadriparesis resulted from intoxication with a nonalcoholic pastis (an anise-flavored
aperitif containing glycyrrhizinic acid).[5]

Therapy
In patients with a single benign tumor (adenoma), surgical removal (adrenalectomy) may
be curative. This is usually performed laparoscopically, through several very small
incisions. For patients with hyperplasia of both glands, successful treatment is often
achieved with spironolactone or eplerenone, drugs that block the effect of aldosterone. In
males, one common side effect of spironolactone drug therapy sometimes seen is
gynecomastia. Gynecomastia usually does not occur with eplerenone drug therapy.[citation
needed]

Prognosis
In the absence of proper treatment, individuals with hyperaldosteronism often suffer from
poorly controlled high blood pressure, which may be associated with increased rates of
stroke, heart disease, and kidney failure. With appropriate treatment, the prognosis is
excellent.[6]