What is Connís Syndrome / Primary Hyperaldosteronism?
Primary hyperaldosteronism can be inherited in some people in an autosomal dominant fashion. The basic underlying pathophysiology can be attributed to the increased secretion of the hormone aldosterone from the adrenal glands. Aldosterone acts on the kidneys resulting in an increase in sodium and lowering of potassium levels in the blood, and increases the alkalinity of the blood. Primary hyperaldosteronism has two major subtypes:
- Bilateral adrenal hyperplasia (60-70% of all cases)
- Unilateral aldosterone-producing adenoma (30-40% of all cases)
What are the Causes of Connís Syndrome?
- Tumor present in the adrenal cortex can lead to excess production of aldosterone
- Abnormal functioning of the adrenal cortex
Symptoms and Diagnosis of Connís SyndromeHypertension due to aldosterone-induced sodium and water retention and hypokalemia (low blood potassium levels) are the two main clinical findings in primary aldosteronism. Both the low potassium levels and increased aldosterone acting directly have a role in making the blood more alkaline. Most of the symptoms of primary hyperaldosteronism / Conn's syndrome occur as a result of hypokalemia and alkalosis. Common symptoms include:
- Muscle weakness
- Patients with long-standing hypertension may show the presence of cardiac and neurological problems
- Chronic headaches
- Myalgia (muscle pain)
- Polydipsia (excessive thirst) and Polyuria (increased urination) may be present
Measuring 24-hour urine aldosterone levels and finding out the plasma aldosterone, plasma renin activity ratio are diagnostic tests that need to be carried out for the condition. CT scan is also carried out to determine the subtype of primary aldosteronism.
Other conditions that could resemble Connís syndrome and should be excluded are Liddleís syndrome, renin-secreting tumor, exogenous mineralocorticoid and excessive licorice intake.
Treatment and Prognosis of Connís SyndromeThe most important step before choosing a treatment plan is establishing the correct subtype diagnosis, since the treatment of primary aldosteronism is based upon whether the increased secretion of aldosterone is unilateral (adenoma, unilateral hyperplasia, or carcinoma) or bilateral (Idiopathic HyperAldosteronism or familial hyperaldosteronism). In patients with unilateral disease, surgery is the recommended option for treatment. In fact, surgery will be curative ONLY in patients with unilateral disease.
Hypertension and hypokalemia should be controlled in a patient with Connís syndrome. In patients with hyperplasia of both the adrenals, administration of drugs such as spironolactone or eplerenone can successfully block the effect of the excessively secreted aldosterone. Spironolactone can sometimes cause an increase in breast size in males. Both these drugs must be stopped prior to testing.
Prognosis is excellent if the condition is properly treated. Absence of treatment results in complications and even fatality.
Latest Publications and Research on Connīs SyndromeNeonatal Bartter syndrome associated with ileal atresia and cystic fibrosis. - Published by PubMed
Adrenal incidentalomas: A collection of six interesting cases and brief review of literature. - Published by PubMed
Hormones other than aldosterone may contribute to hypertension in 3 different subtypes of primary aldosteronism. - Published by PubMed
Aldosterone Directly Affects Apelin Expression and Secretion in Adipocytes. - Published by PubMed
Adrenal Venous Sampling for Primary Hyperaldosteronism in Patients with Concurrent Chronic Kidney Disease. - Published by PubMed