Kidney Big Sebaceous

Origin of the name The word “big greasy” (white amyloid kidney) first arose as a result of a mistake by laboratory technicians who mixed up the structure of the kidneys of the deceased Pavlovsky sisters - the elder Tatyana and the younger Sophia - in the morgue (there are several possible versions of the origin of this nickname; see, for example, comments by P M. Zinoviev for the film “Sofya Petrovna”).

Large sebaceous kidney (large white amyloid kidney, polycystic sebaceous glands) is a rare disease that is associated with enlargement and hardening of the large sebaceous glands in the kidneys.

The causes of the disease are not exactly known, but scientists associate it with metabolic and protein disorders in the body. The increase in a large volume of white amyloid deposits in the lumen of blood vessels is presumably due to improper outflow of blood from the microvascular endothelium. Possible influence of hereditary factors from Wilson-Konovalov disease, Hurler syndrome and benign familial hyperlipoproteinemic syndrome.

In order to avoid the formation of polycystic cysts, timely treatment is necessary after making an accurate diagnosis. The main goal of treatment is to reduce the growth of sebaceous nuclei in size. Chemotherapy treatment has no effect on the growth of large structures of amloidosis, and the use of anticoagulants, glucocorticosteroids and interferon is useful only for relapses and massive damage to the parenchyma. Myelostabilizing chemotherapy with simultaneous administration of angiotensin-converting enzyme inhibitors is often used. In 2017, randomized studies of the combined administration of the drug bortezomi were conducted