Alport syndrome is a genetic condition caused by mutations in the genes encoding for a structural protein called type 4 collagen. These mutations cause type 4 collagen to be faulty and leads to kidney disease, hearing loss, and eye abnormalities.
What are aldosterone inhibitors?
Aldosterone inhibitors are a class of medications that affect the secretion of fluid and the retention of salt in the kidneys. This class of medication often is prescribed to control blood pressure.
Aldosterone is a signaling hormone which binds to a protein receptor in the kidneys. Once bound, the aldosterone/receptor complex signals to increase the production of ion channel proteins in the kidneys. More channels mean that more sodium and water are retained from urine before it is excreted. The same pumps that remove sodium from the urine excrete potassium at the same time, so aldosterone receptors also cause an increase in potassium excretion.
Aldosterone inhibitors block the function of aldosterone by binding to the aldosterone receptor, which means that the receptor is not available to bind aldosterone. By blocking the binding of aldosterone to its receptor, the amount of water and sodium excreted by the kidneys is increased, and potassium is retained.
Aldosterone inhibitors in Alport syndrome
Aldosterone inhibitors are recommended as a second-line treatment after ACE-inhibitors in supporting kidney function. Some ACE-inhibitors also can cause increased protein in the urine, which may be related to an increase in aldosterone. Therefore, combining ACE-inhibitors with aldosterone inhibitors may be more effective than either treatment on its own.
Two aldosterone inhibitors are currently available: Aldactone (spironolactone) and Inspra (eplerenone).
Other information
Aldosterone inhibitors can cause side effects, including tiredness, nausea, diarrhea, and stomach cramps.
Aldactone also may cause an increase in blood levels of potassium, so it is recommended that patients ensure they are eating a “renal healthy” diet that is low in potassium.
While taking aldosterone inhibitors, it is recommended that patients weigh themselves at the same time of day every day. If a patient gains more than three pounds in a single day, or more than five pounds in a week, he or she should contact their physician immediately.
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