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Rational Pharmacotherapy in Cardiology

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Systemic ATTR-amyloidosis, a Rare Form of Internal Organ Damage

https://doi.org/10.20996/1819-6446-2019-15-3-349-358

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Abstract

The article presents the case report of a rare hereditary form of systemic ATTR-amyloidosis in Russian patient with a discussion of approaches to the diagnosis and treatment of this form, also based on the own experience in the management of such patients. Modern ideas about the pathogenesis of the disease as well as detailed information about the clinical manifestations of amyloid cardiopathy and of other organs are presented. The nature of structural and hemodynamic changes in the heart is discussed on the basis of experience, including own, ultrasound examination of the heart in patients with amyloidosis, especially the article focuses the reader's attention on the true infiltrative nature of transtiretin amyloid cardiopathy in contrast to AL-amyloidosis, in which there is a significant inflammatory component that determines a more unfavorable natural course of AL-amyloidosis of the heart. The article discusses the differential diagnosis of different types of amyloidosis, the diagnostic difficulties associated with weak congophilia of transtiretin amyloidosis and at the same time substantiates the need for morphological verification of the diagnosis. Modern methods of treatment of ATTR-amyloidosis are discussed.

About the Authors

V. V. Rameev
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Russian Federation

MD, PhD, Associate Professor, Chair of Therapy, Occupational Diseases and Rheumatology

Trubetskaya ul. 8-2, Moscow, 119991 Russia



R. P. Myasnikov
National Medical Research Center for Preventive Medicine
Russian Federation

MD, PhD, Senior Researcher, Department of Clinical Cardiology and Molecular Genetics

 Petroverigsky per. 10, Moscow, 101990 Russia



P. P. Vinogradov
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Russian Federation

MD, Post-Graduate Student, Chair of Therapy, Occupational Diseases and Rheumatology

Trubetskaya ul. 8-2, Moscow, 119991 Russia



L. V. Kozlovskaya
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Russian Federation

MD, PhD, Professor, Chair of Therapy, Occupational Diseases and Rheumatology

Trubetskaya ul. 8-2, Moscow, 119991 Russia



S. V. Moiseev
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Russian Federation

MD, PhD, Professor, Head of Chair of Therapy, Occupational Diseases and Rheumatology

Trubetskaya ul. 8-2, Moscow, 119991 Russia



E. I. Fomicheva
National Medical Research Center for Preventive Medicine
Russian Federation

MD, Junior Researcher, Department of Fundamental and Applied Aspects of Obesity

Petroverigsky per. 10, Moscow, 101990 Russia



S. A. Beregovskaya
National Medical Research Center for Preventive Medicine
Russian Federation

MD, Head of the First Cardiology Department

Petroverigsky per. 10, Moscow, 101990 Russia



E. A. Mershina
Lomonosov Moscow State University
Russian Federation

MD, PhD, Head of Radiology Department, Leading Researcher, Medical Research and Education Center

 Leninskie Gory 1, Moscow, 119991 Russia



S. A. Kostina
Eramishanzev Moscow City Clinical Hospital
Russian Federation

MD, PhD, Physician, Functional Diagnostic Department

 Lenskaya ul. 15, Moscow, 129327 Russia



L. A. Strizhakov
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Russian Federation

MD, PhD, Professor, Chair of Therapy, Occupational Diseases and Rheumatology

Trubetskaya ul. 8-2, Moscow, 119991 Russia



A. S. Rameeva
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Russian Federation

MD, Post-Graduate Student, Chair of Therapy, Occupational Diseases and Rheumatology

Trubetskaya ul. 8-2, Moscow, 119991 Russia



P. P. Tao
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Russian Federation

MD, Post-Graduate Student, Chair of Therapy, Occupational Diseases and Rheumatology

Trubetskaya ul. 8-2, Moscow, 119991 Russia



O. M. Drapkina
National Medical Research Center for Preventive Medicine
Russian Federation

MD, PhD, Professor, Corresponding Member of the Russian Academy of Sciences, Director

Petroverigsky per. 10, Moscow, 101990 Russia



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For citation:


Rameev V.V., Myasnikov R.P., Vinogradov P.P., Kozlovskaya L.V., Moiseev S.V., Fomicheva E.I., Beregovskaya S.A., Mershina E.A., Kostina S.A., Strizhakov L.A., Rameeva A.S., Tao P.P., Drapkina O.M. Systemic ATTR-amyloidosis, a Rare Form of Internal Organ Damage. Rational Pharmacotherapy in Cardiology. 2019;15(3):349-358. (In Russ.) https://doi.org/10.20996/1819-6446-2019-15-3-349-358

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