What is the amyloidosis of the kidneys?

Amyloidosis diseases are a group of diseases with certain characteristics. The main characteristic of these diseases is the presence of abnormally shaped proteins. In the kidney amyloidosis, malformed proteins are stored in the kidneys, causing progressive kidney disease and renal failure. There are more than 20 known proteins that can cause amyloidosis. Most of them are associated with rare hereditary forms of disease. All proteins have a three -dimensional structure that is dictated by chemical interactions between different amino acids in the protein chain. Amyloidosis diseases develop due to genetic mutation, diseases such as lymphoma, chronic inflammation or environmental factor that causes mis or more proteins. Therefore, these mutated or poorly composed proteins cannot work normally. In the census, as these abnormally structured proteins accumulate, they are stored in spaces between the cells and form a matrix that gradually disrupte function of healthy organs and tissues.

Renal amyloidosis does not generally occur separately, but it is a common effect in people with systemic amyloidosis. The systemic form of the disease results in storage of abnormal proteins in organs and tissues throughout the body. When the kidneys are affected, the final result is kidney failure, which can only be treated by kidney transplant. The amyloidosis of kidney often develops as part of inherited syndrome. Familiary amyloidosis of kidney is sometimes known as Otertag amyloidosis, named for Berthold Otertag, a doctor who first characterized this disease in 1932.

All forms of hereditary renal amyloidosis are caused by mutations in one protein. The most common type of kidneyloidosis is called amyloidosis of transthyretin (TTR) and occurs due to mutations in proteins such as lysozy, fibrinogen or apolipoprotein. Symptoms of amyloidosis of kidneys developbecause the abnormal deposits of proteins increase and begin to disrupt the kidney function. One of the most common symptoms is the presence of slightly enlarged kidneys and a very large amount of protein in the urine. This protein is present because the affected kidneys are unable to filter protein from waste water.

Renal amyloidosis is usually a systemic disease, so these symptoms are accompanied by symptoms related to the abnormal function of other organs. These include liver and enlargement, heart failure, nervous disease, thyroid enlargement and gastrointestinal tract problems. Systemic amyloidosis is often a fatal disease due to multiple organ involvement, but the progression of the disease and available treatment may vary depending on protein abnormality. For examps, when abnormal protein is produced in the liver, the progression of the disease may sometimes be stopped by successful liver transplantation.