REN-Related Kidney Disease Information

Friday, January 22, 2010 Article by: D.K. Mangusan Jr., PTRP

REN-related kidney disease is a rare inherited disorder that affects the kidneys. It can cause kidney disease that slowly progresses, which becomes apparent during childhood.

As the disease progresses, the kidneys will slowly be less able to filter out wastes and excess water. Ultimately, the condition can cause kidney failure where the kidneys totally fail to perform its functions. When the kidneys fail to function in people with REN-related kidney disease, they would typically require kidney dialysis or kidney transplantation. Kidney dialysis is performed to remove wastes from the blood.

Signs and symptoms of REN-related kidney disease may include the following:
• Mild anemia (decreased number of red blood cells that carry oxygen to tissues)

• Pale skin (related to anemia)

• Weakness and fatigue (related to anemia)

• Low blood pressure or hypotension

• Gout because of elevated levels of uric acid in the body
Kidneys of people with the condition are not able to excrete wastes. One of these wastes includes uric acid. Normally, uric acid is removed from the blood to become part of the urine. People with REN-related kidney disease are not able to remove this waste product, thus, accumulates in the body. This buildup of uric acid in the body can cause a very painful type of arthritis called gout. The uric acid can form crystals and deposit in joints causing pain.

Experts have identified the abnormal gene that causes REN-related kidney disease. Normal REN gene carries the information for making a hormone called renin, which is produced in the kidneys. Renin plays an important role in the control of blood pressure and water levels in the body.

When the REN gene is altered or undergo changes (mutations), it can cause death to the cells that normally produce renin in the kidneys. The abnormal REN gene produces a substance toxic to the renin-producing cells. These specialized kidney cells gradually die, which can cause progressive kidney disease.

Source: Clinical background provided by the Genetics Home Reference of the U.S. National Library of Medicine.

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