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Berger's disease

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Kidneys and adrenal glands

Alternative Names
IgA nephropathy, Berger's nephropathy

Definition
Berger's disease is a disorder of unknown cause that results in kidney damage, and may lead to kidney failure.

What is going on in the body?
The job of the kidneys is to make urine by filtering the blood. In Berger's disease, the filtering parts of the kidney, called the glomeruli, become damaged. The damage is due to deposits of proteins called antibodies. Antibodies are normally made by the immune system to fight infections. For unknown reasons, these antibodies, called IgA antibodies, clog up the filtering system of the kidney in Berger's disease.

Berger's disease usually occurs in people less than 30 years old, often in children. Within 20 years after the first symptoms, roughly 20% of affected people develop kidney failure.

What are the signs and symptoms of the disease?
A person with Berger's disease may have no symptoms at all, or may have symptoms that include: What are the causes and risks of the disease?
The cause of Berger's disease is unknown. It is more common in Asia and Australia than in the US. Males are more often affected than women. This condition is usually first seen in children and young adults.

What can be done to prevent the disease?
There are no known ways to prevent Berger's disease.

How is the disease diagnosed?
Diagnosis of Berger's disease begins with the history and physical examination. A urine test called a urinalysis will show blood, and often protein, in the urine. This often makes the doctor suspect Berger's disease. A blood test can be done to measure the level of IgA antibodies, which is often higher than normal.

A kidney biopsy is usually needed to confirm the diagnosis. This involves inserting a special needle through the skin of the back and into the kidney. A small piece of kidney is removed with the needle. This piece can then be sent to the laboratory for examination.

What are the long-term effects of the disease?
Some people with Berger's disease, especially children, suffer no long-term effects. In other cases, the disease slowly gets worse over time. A person may develop high blood pressure due to the kidney damage.

Roughly 20% of people with Berger's disease will slowly develop kidney failure, which is the most feared long-term effect. Those with kidney failure need dialysis or a kidney transplant to survive. Dialysis is a procedure to filter the blood. It usually requires the person to be hooked up to a special blood-filtering machine 3 times a week. This continues for life or until the person gets a kidney transplant.

What are the risks to others?
Berger's disease is not contagious and poses no risks to others.

What are the treatments for the disease?
Treatment of Berger's disease usually focuses on controlling blood pressure and reducing protein loss in the urine. This is usually done with one of the blood pressure medications called ACE inhibitors. Examples include enalapril and captopril. Fish oil, and corticosteroids such as prednisone, may help slow down the kidney damage in some cases.

A person who develops kidney failure will need dialysis or a kidney transplant.

What are the side effects of the treatments?
ACE inhibitor medications may cause cough, salt imbalances, and kidney problems in some cases. Fish oil capsules can cause burping and a fishy aftertaste. Corticosteroids can cause osteoporosis.

Both dialysis and kidney transplant require surgery. All surgery carries a risk of bleeding, infection, and allergic reaction to anaesthesia. Dialysis has possible many side effects, including infection, salt imbalances, and even death. After a kidney transplant, the person must take powerful medications to prevent rejection. These medications can lead to infections, and also may damage the new kidney.

What happens after treatment for the disease?
Treatment does not cure Berger's disease. Affected people, especially children, may go into remission and need no further treatment. Others will have occasional flares of the disease, requiring closer monitoring and treatment. Kidney failure from Berger's disease is usually not reversible. A person with kidney failure usually needs treatment for life.

How is the disease monitored?
The doctor will order regular kidney function tests, and check the person's blood pressure periodically. The person can watch for blood in the urine at home. Any new or worsening symptoms should be reported to the doctor

Author:
Reviewer: HealthAnswers Australia Medical Review Panel
Editor: Dr David Taylor, Chief Medical Officer HealthAnswers Australia
Last Updated: 1/10/2001
Contributors
Potential conflict of interest information for reviewers available on request


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