S.L. MOROZOV^1,2, O.R. PIRUZIEVA¹, M.YU. DOROFEEVA¹, O.V. TARASOVA², V.V. DLIN¹

 ¹Veltischev Research and Clinical Institute for Pediatrics, Moscow

²Pirogov Russian National Research Medical University, Moscow

Contact details:

Morozov S.L. — PhD (medicine), Leading Researcher of the Department of Hereditary and Acquired Kidney Diseases named after M.S. Ignatova, Associate Professor of the Department of Hospital Therapy No. 2 of the Pediatrics Faculty

Address: 2 Taldomskaya St., Moscow, Russian Federation, 125412, tel.: +7-903-138-77-32, e-mail: mser@list.ru

Tuberous sclerosis is a rare multisystem disease with autosomal dominant inheritance, characterized by the formation of hamartomas in many organs and tissues, including skin, brain, kidneys, lungs and heart, with a variety of clinical manifestations. One of the largest problems in patients with tuberous sclerosis is renal angiomyolipomas, which are the most common manifestation of the disease. An important point in the course of tuberous sclerosis with kidney damage is the formation of secondary complications that occur against the background of the underlying disease. The present review shows the relevance of the problem of angiomyolipomas in tuberous sclerosis, considers the pathogenesis of the disease, and presents the basic principles of treatment.

Key words: children, tuberous sclerosis, angiomyolipomas, vascular tumor, rapamycin, everalimus.

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