Angiotensin-converting-enzyme (ACE) inhibitors, with help to relax blood vessels, are medications to treat high blood pressure.

They are used to slow the damage that can lead to kidney failure and improve life expectancy in Alport syndrome patients. They also can reduce proteinuria, the presence of proteins in the urine, in these patients. Proteinuria is associated with kidney damage.

ACE inhibitors may be used in combination with angiotensin receptor blockers (ARBs).

How ACE inhibitors work

As the name suggests, ACE inhibitors inhibit an enzyme called angiotensin-converting-enzyme (ACE). ACE acts to convert the hormone angiotensin 1 into another hormone called angiotensin 2.

Angiotensin 2 is known to increase blood pressure by three different mechanisms:

  • It acts as a vasoconstrictor, a molecule that causes blood vessels to narrow so as to increase blood pressure.
  • It stimulates the release of a hormone called aldosterone that promotes the retention of sodium and chloride ions. Higher sodium chloride or salt concentrations in the blood increase the volume of the blood, subsequently leading to a rise in blood pressure. Aldosterone also increases the excretion of potassium.
  • It breaks down a protein called bradykinin, which is known to dilate or widen blood vessels. Bradykinin’s loss can cause blood pressure to rise.

By lowering the levels of angiotensin 2, ACE inhibitors reduce blood pressure. Their effect on blood pressure by stabilizing bradykinin, however, remains to be proven.

Other blood-pressure-lowering medications do not show the same protective benefits to the kidneys as ACE inhibitors. Scientist for this reason suggest that ACE inhibitors may aid kidney function by additional mechanisms. For example, they are known to lower the glomerular filtration pressure. They also decrease the permeability of the glomeruli, the small blood vessels in the kidneys the filter waste products from the blood. Both mechanisms are thought to prevent proteinuria.

ACE inhibitors for Alport patients

ACE inhibitors that are used to treat Alport syndrome patients include Lotensin (benazepril), cilazapril, enalapril, Monopril (fosinopril), lisinopril, Aceon (perindopril), Altace (ramipril), Accupril (quinapril), and Mavik (trandolapril).

Additional information

Because ACE inhibitors work to retain potassium, potassium-rich foods, such as dairy products, salt substitutes, nuts, and certain fruits and vegetables should be avoided while taking these medications.

ACE inhibitors are usually well-tolerated and can be safely used in children. Possible side effects include fatigue, dry cough, headaches, dizziness, and loss of taste.

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