Alport syndrome is an inherited disorder that is characterized by progressive kidney damage, hearing problems, and eye abnormalities. There is currently no cure for Alport syndrome and treatments are limited, but research is ongoing and various therapies are used to help patients manage its symptoms.
These options are described briefly here, and links to sites with more specific information are provided.
Most of the medications used in Alport syndrome aim to slow the progression of kidney damage. Many of these are also either in or needing to enter clinical trials to evaluate their potential benefit in Alport patients.
Alport syndrome patients are commonly prescribed medications that affect the “renin-angiotensin-aldosterone system”, or RAAS, a system of hormones involved in controlling urine levels and blood pressure. Traditionally these medications were given to lower blood pressure, but it is thought that they may also be beneficial in kidney disease by reducing scarring in the kidneys and increasing urine production and blood flow. They are normally prescribed to patients who have proteinuria, or protein in the urine.
The most commonly prescribed medicines are angiotensin-converting enzyme (ACE) inhibitors. These reduce the levels of the enzyme angiotensin II that acts to narrow blood vessels and increase blood pressure. There are a range of different ACE inhibitors, including Lotensin (benazepril), Vasotec (enalapril), and Aceon (perindopril), among others.
Angiotensin receptor blockers (ARBs) inhibit the action angiotensin II and may be prescribed if a patient cannot take ACE inhibitors. These include Atacand (candesartan), Cozaar (losartan), and Teveten (eprosartan), among others.
A third type of medication, called aldosterone inhibitors, may also be prescribed to Alport syndrome patients. These block the action of the hormone aldosterone, which can also contribute to proteinuria in Alport syndrome.
Dialysis is a procedure used to artificially filter waste products from the blood. This may be required in Alport patients whose kidneys can no longer effectively filter the blood, such as when a patient has end-stage kidney disease.
Patients with Alport syndrome whose kidney damage is extensive may be recommended to undergo a kidney transplant. Normally, Alport symptoms should not recur in the transplanted kidney, as long as the donor is not affected by the disease. But around 3 to 5 percent of men with Alport syndrome develop a condition called anti-GBM nephritis, which results in severe inflammation of the kidneys and the subsequent rejection of the transplant.
Some Alport patients may also require surgery to remove cataracts.
Hearing loss can be progressive in Alport, and patients may be advised to use hearing aids to amplify sounds and improve their ability to hear well.
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