Systemic transtiretin amyloidosis in the elderly patient with recurrent pleural effusions

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Systemic amyloidosis is a group of diseases associated with extracellular deposition of fibrillar proteins, which leads to a loss of normal organ structure and function. Transthyretin amyloidosis occurs with the deposition of amyloid, consisting of transthyretin transport protein, and can be a genetic or degenerative disease of senility (acquired from the deposition of wild-type transthyretin).

The article describes the clinical case of transthyretine amyloidosis in elderly patient, manifested by recurrent pleural effusions and biventricular heart failure demonstrating the complexity of timely diagnosis of wild-type transthyretin amyloidosis.

作者简介

Svetlana Boldueva

North-Western State Medical University named after I.I. Mechnikov

Email: svetlanaboldueva@mail.ru
ORCID iD: 0000-0002-1898-084X
Scopus 作者 ID: 6506877971

MD, Dr. Sci. (Med.), Professor

俄罗斯联邦, 41 Kirochnaya St., Saint Petersburg, 191015

Dmitriy Evdokimov

North-Western State Medical University named after I.I. Mechnikov

编辑信件的主要联系方式.
Email: kasabian244@gmail.com
ORCID iD: 0000-0002-3107-1691

аспирант кафедры факультетской терапии

俄罗斯联邦, 41 Kirochnaya St., Saint Petersburg, 191015

Natalia Shvets

North-Western State Medical University named after I.I. Mechnikov

Email: natashashvets@yandex.ru

MD, Cand. Sci. (Med.)

俄罗斯联邦, 41 Kirochnaya St., Saint Petersburg, 191015

Anahit Shahbazyan

North-Western State Medical University named after I.I. Mechnikov

Email: shahbazyananahit13@gmail.com
ORCID iD: 0000-0002-6923-5321

врач-кардиолог

俄罗斯联邦, 41 Kirochnaya St., Saint Petersburg, 191015

Elena Kalinina

Saint-Petersburg State Pediatric Medical University

Email: drkalinina@yandex.ru

MD, Cand. Sci. (Med.)

俄罗斯联邦, Saint Petersburg

Lyubov Mitrofanova

Almazov National Medical Research Centre

Email: lubamitr@yandex.ru

MD, Dr. Sci. (Med.), Assistant Professor

俄罗斯联邦, Saint Petersburg

参考

  1. Quarta CC, Solomon SD, Uraizee I, et al. Left ventricular structure and function in transthyretin-related versus light-chain cardiac amyloidosis. Circulation. 2014;129(18):1840–1849. doi: 10.1161/CIRCULATIONAHA.113.006242
  2. Connors LH, Sam F, Skinner M, et al. Heart failure resulting from age-related cardiac amyloid disease associated with wild-type transthyretin: a prospective, observational Cohort Study. Circulation. 2016;133(3):282–290. doi: 10.1161/CIRCULATIONAHA.115.018852
  3. Boldueva SA, Petrova VB, Khavinson VKh, et al. Problems in the diagnostics and treatment of transtiretinum amyloidosis with heart disease in the elderly: clinical experience. Advances in Gerontology. 2019;32(1–2):137–144. (In Russ.)
  4. Castaño A, Drachman BM, Judge D, Maurer MS. Natural history and therapy of TTR-cardiac amyloidosis: emerging disease-modifying therapies from organ transplantation to stabilizer and silencer drugs. Heart Fail Rev. 2015;20(2):163–178. doi: 10.1007/s10741-014-9462-7
  5. Sultan AM, Edwards WD, Mohammed SF, et al. Cardiac amyloid deposition is common in elderly patients with heart failure and preserved ejection fraction. Circulation. 2010;122:A17926.
  6. Tanskanen M, Peuralinna T, Polvikoski T, et al. Senile systemic amyloidosis affects 25% of the very aged and associates with genetic variation in alpha2-macroglobulin and tau: a population-based autopsy study. Ann Med. 2008;40(3):232–239. doi: 10.1080/07853890701842988
  7. Poliakova AA, Semernin EN, Sitnikova MY, et al. Transthyretin amyloidosis in a cohort fold and very old patients with chronic heart failure. Kardiologiia. 2018;58(S2):12–18. (In Russ.). doi: 10.18087/cardio.2390
  8. Dyudina IA. Transtyretin amyloidosis: the current state of the problem. Heart failure and comorbid conditions.2017;1:63–68. (In Ukraine)
  9. Dubrey SW, Hawkins PN, Falk RH. Amyloid diseases of the heart: assessment, diagnosis, and referral. Heart. 2011;97(1):75–84. doi: 10.1136/hrt.2009.190405
  10. Fernandes A, Caetano F, Almeida I, et al. Diagnostic approach to cardiac amyloidosis: A case report. Amiloidose cardíaca – a bordagem diagnóstica, a propósito de um casoclínico. Rev Port Cardiol. 2016;35(5):305.e1–305.e7. doi: 10.1016/j.repc.2016.01.004
  11. Lysenko (Kozlovskaya) LV, Rameev VV, Moiseev S, et al. Clinical guidelines for diagnosis and treatment of systemic amyloidosis. Klinicheskaya farmakologiya i terapiya. 2020;(1):13–24.(In Russ.). DOI: 10.32756/ 0869-5490-2020-1-13-24
  12. Berk JL. Pleural effusions in systemic amyloidosis. Curr Opin Pulm Med. 2005;11(4):324–328. doi: 10.1097/01.mcp.0000162378.35928.37
  13. Mansalis KA, Klein DA, Demartini SD, et al. Pleural findings in a patient with persistent pulmonary effusions from systemic amyloidosis. Amyloid. 2011;18(1):29–31. doi: 10.3109/13506129.2010.537156

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2. Fig. 1. The electrocardiogram at the admission

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3. Fig. 2. The echocardiography at the admission

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4. Fig. 3. Chest X-ray data: a — 05.02; b — 11.02; c — 13.02

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5. Fig. 4. Magnification ×400. Congo-red. Deposition of amyloid under the endocardium

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6. Fig. 5. Magnification ×400. Congo-red. Polarized light microscopy. Amyloid in the artery wall

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7. Fig. 6. Magnification ×400. Congo-red. Polarized light microscopy. Myocardium. Perivascular amyloid deposition

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8. Fig. 7. Magnification ×200. Congo-red. Deposition of amyloid in the myocardium

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9. Fig. 8. Magnification ×200. Congo-red. Myocardium. Deposition of amyloid in the stroma

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10. Fig. 9. Magnification ×400. Microscopy in polarized light. Amyloid on the tongue

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11. Fig. 10. Gross specimen of the heart, left ventricular (LV) section. There is a significant thickening of the LV walls, small size of the LV cavity

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版权所有 © Boldueva S., Evdokimov D., Shvets N., Shahbazyan A., Kalinina E., Mitrofanova L., 2021

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