Effectiveness of targeted therapy for kidney damage with tuberous sclerosis in a child (clinical case)
https://doi.org/10.36485/1561-6274-2021-25-4-90-94
Abstract
Tuberous sclerosis is a polysystemic, genetically determined, autosomal dominant orphan disease that affects approximately 1 in 10,000 people worldwide. Kidney damage in tuberous sclerosis is the leading cause of death due to serious complications, the most common of which is angiomyolipoma bleeding. A feature of renal angiomyolipomas is that they begin to progress and increase in volume from an early age, leading to the progression of chronic kidney disease, while angiomyolipomas more than 30 mm in diameter are at risk of bleeding. Currently, pharmacotherapy of tuberous sclerosis with mTOR inhibitors is the most effective worldwide. In this article, a clinical case of targeted therapy of tuberous sclerosis is presented, the effectiveness is demonstrated, and the features of the course of tuberous sclerosis are also given on a specific example.
About the Authors
S. L. Morozov
Pirogov Russian National Research Medical University; Veltischev Research and Clinical Institute for Pediatrics
Russian Federation
Russian Federation
Sergey Leonidovich Morozov, MD, PhD, Department of Hospital Pediatrics, No. 2, Faculty of Pediatrics, Associate Professor; Department of Hereditary and Acquired Kidney Diseases , Senior Researcher
117997, Russia, Moscow, st. Ostrovityanova, 1.
Phone: +8(903)138-77-32
O. R. Piruzieva
Veltischev Research and Clinical Institute for Pediatrics; Pirogov Russian National Research Medical University
Russian Federation
Russian Federation
Oksana Rashidovna Piruzieva, MD, Department of Nephrology, nephrologist
117997, Russia, Moscow, st. Ostrovityanova, 1
Phone: +8(903)1387732
V. V. Dlin
Veltischev Research and Clinical Institute for Pediatrics
Russian Federation
Russian Federation
Prof. Vladimir V. Dlin, MD, PhD, DMedSci, Head of the Department of Hereditary and Acquired Kidney Diseases, Director
125412, Russia, Moscow, st. Taldomskaya, 2
Phone: 8(916)634-34-53
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Review
For citations:
Morozov S.L., Piruzieva O.R., Dlin V.V. Effectiveness of targeted therapy for kidney damage with tuberous sclerosis in a child (clinical case). Nephrology (Saint-Petersburg). 2021;25(4):90-94. (In Russ.) https://doi.org/10.36485/1561-6274-2021-25-4-90-94
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