Conn syndrome
Conn's syndrome Classification and external resources |
|
Aldosterone | |
ICD-10 | E26.0 |
ICD-9 | 255.1 |
DiseasesDB | 3073 |
MedlinePlus | 000330 |
eMedicine | med/432 |
MeSH | D006929 |
Conn's syndrome is characterized by the overproduction of the mineralocorticoid hormone aldosterone by the adrenal glands. Aldosterone causes sodium and water retention and potassium excretion in the kidneys, leading to arterial hypertension (high blood pressure). It is a rare but recognised cause of hypertension.
Contents |
Signs, symptoms and findings
Conn's syndrome is also known as primary hyperaldosteronism. Apart from high blood pressure, the symptoms may include muscle cramps (due to hyperexcitability of neurons), muscle weakness (due to hypoexcitability of skeletal muscles) and headaches (due to the low potassium), metabolic alkalosis (due to increased secretion of H+ ions by the kidney). The high pH of the blood makes calcium less available to the tissues and causes symptoms of hypocalcemia (low calcium levels).
It can be mimicked by liquorice ingestion (glycyrrhizin) and Liddle syndrome.
Diagnosis
Measuring aldosterone alone is not considered adequate to diagnose Conn's syndrome. Rather, both renin and aldosterone are measured, and the ratio is diagnostic.[1][2]
Usually, renin levels are suppressed, leading to a very low renin-aldosterone ratio (<0.0005). This test is confounded by antihypertensive drugs, which have to be stopped up to 6 weeks.
If there is biochemic proof of hyperaldosteronism, CT scanning can confirm the presence of an adrenal adenoma.
Causes
The syndrome is due to:
- aldosterone-secreting adrenal adenoma (benign tumor, 50-60%)
- hyperplasia of the adrenal gland (40-50%)
- rare forms
Therapy
The treatment for hyperaldosteronism depends on the underlying cause. In patients with a single benign tumor (adenoma), surgical removal (adrenalectomy) is 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 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.[3]
References
- ^ Tiu S, Choi C, Shek C, Ng Y, Chan F, Ng C, Kong A (2005). "The use of aldosterone-renin ratio as a diagnostic test for primary hyperaldosteronism and its test characteristics under different conditions of blood sampling". J Clin Endocrinol Metab 90 (1): 72-8. PMID 15483077.
- ^ http://www.ubht.nhs.uk/pathology/ChemicalPathology/TestProtocols/16Renin.html
- ^ http://www.cumc.columbia.edu/dept/cs/pat/adrenal/hyperaldosteronism.html/ref>
Eponym
It is named after Dr Jerome W. Conn (1907-1994), the American endocrinologist who first described the condition at the University of Michigan in 1955. <ref>Conn JW, Louis LH. ''Primary aldosteronism: a new clinical entity.'' Trans Assoc Am Physicians 1955;68:215-31; discussion, 231-3. PMID 13299331.</li></ol></ref>