Amyloidosis Primary

Amyloidosis Primary: Understanding and Diagnosis

Primary amyloidosis, also known as genuine amyloidosis or idiopathic amyloidosis, is a rare and serious disease associated with the abnormal accumulation of amyloid in various tissues of the body. Amyloid is an unusual protein substance that can form insoluble deposits in organs and tissues, interfering with their normal function.

In primary amyloidosis, amyloid is formed from certain proteins that are normally present in the body. However, as a result of genetic or acquired disorders, they fold incorrectly, which leads to the formation of amyloid plaques. These plaques accumulate in various organs, causing damage and dysfunction.

Primary amyloidosis can affect various organs and systems of the body, including the heart, kidneys, liver, nervous system and others. Symptoms and manifestations of primary amyloidosis can vary depending on the organ affected, but common symptoms may include fatigue, weight loss, organ dysfunction, swelling and pain.

Diagnosis of primary amyloidosis can be difficult because symptoms may be nonspecific and similar to other diseases. However, there are several methods that can help make the diagnosis. One of them is a biopsy of the affected tissue, which allows the presence of amyloid to be examined. Various laboratory tests and instrumental methods, such as MRI, computed tomography and electrocardiography, can also be used.

Treatment of primary amyloidosis is aimed at managing symptoms, slowing disease progression, and preventing complications. This may include medications such as anti-inflammatory drugs or chemotherapy, and organ transplantation in some cases.

However, it is important to note that primary amyloidosis is a chronic and progressive disease, and its treatment can be difficult. Therefore, early detection and diagnosis play an important role in managing this condition and improving the prognosis for patients.

In conclusion, primary amyloidosis is a rare disease characterized by abnormal accumulation of amyloid in various organs and tissues. Understanding this condition and its diagnosis is essential to ensure timely treatment and improve the prognosis of patients. It is necessary to continue research and development of new methods for diagnosing and treating primary amyloidosis to help those suffering from this serious disease.

Amyloidosis (syn.: amyloid syndrome, amyloid disease), a hereditary disease from the group of systemic dysproteinemias, associated with the formation of specific masses in tissues - amyloids - and leading to their massive decay.

A.P. Grazhdankova, L.S. Gitelzon, Z.V. Varaksa indicate that pathognomonic for this disease is the deposition in tissues and organs of inert substances with a β-conformation, similar to fibrils. When a special type of fibril is formed, amyloid is deposited; they consist of polypeptide chains and aggregates