Vacuolar nephropathy

Vacuolar nephropathy - (nephropathy vacuole - literally “a disease characterized by abnormal focal accumulations of vacuoles resulting from mutations of several transcription factors”) is a rare hereditary disease of children and adults. The disease belongs to the category of idiopathic progressive glomerulonephritis. Despite significant progress made in studying the genetic and molecular mechanisms of nephropathy, its etiology has not been definitively established. The disease is based on a mutation or loss of one of the proteins of the V-ATPase and PtdIns3K family.

Clinically, nephropathy occurs with pronounced variations, but invariably with periods of remission and exacerbation. With the development of the disease in young children, nephritis develops rapidly, which raises the question of kidney transplantation in the chronic form of the disease. Experts often consider hematuria as the main symptom. The appearance of the latter is associated with mechanical damage to the glomerular capillaries with a violation of their structure. Earlier symptoms are edema, proteinuria and hypertension. Dehydration