Abstract

The aimof the work was to study the clinical case of systemic amyloidosis.Materials and methods. Patient Ch., 63 уears old, have admitted to the regional cardiological dispensary with complaints of short stabbing pains in the heart, without reaction to nitroglycerin, interruptions in the work of the heart, mainly at night, shortness of breath with little exertion, weakness, swelling of the legs and feet in September 2018. Sick from April 2016, when was dyspnea on exertion. The high level of creatinine, normochromic anemia have detected. In June 2016 chronic pyelonephritis was diagnosed. In August 2018, a right-sided hydrothorax was diagnosed, a pleural puncture was performed.Results. Laboratory and instrumental research methods were performed. Based on anamnesis data (normochromic anemia, proteinuria, increased creatinine in the blood), clinical picture, data of instrumental studies (restrictive cardiomyopathy, bilateral hydrothorax) clinical diagnosis was made: systemic amyloidosis the kidneys and heart with chronic kidney disease C5 and chronic heart failure. Histological evidence of amyloid and determination of the variant of amyloidosis by immunohistochemical method was recommended. But the patient died before the study. The histological verification of the diagnosis was carried out at the autopsy.Conclusion.This clinical observation is an example of late diagnosis of systemic amyloidosis and postmortal verification of the diagnosis, which is associated with the nonspecific symptoms and the rapidly progressing course of the disease. This case is interesting because amyloidosis proceeded under the guise of other diseases, quickly led to the development of severe heart and renal failure and to the death of the patient before verifying the disease.

Highlights

  • The aim of the work was to study the clinical case of systemic amyloidosis

  • This clinical observation is an example of late diagnosis of systemic amyloidosis and postmortal verification of the diagnosis, which is associated with the nonspecific symptoms and the rapidly progressing course of the disease

  • Заподозрить наличие амилоидоза у данного пациента позволило выявление периорбитальных геморрагий, рецидивирующих в одних и тех же местах, в сочетании с концентрической гипертрофией левого желудочка (ЛЖ), по данным эхокардиографии, при отсутствии артериальной гипертензии, с нарушением диастолической функции по рестриктивному типу, наличием ярких включений в структуре миокарда, а также клиникой декомпенсации хронической сердечной недостаточности с сохраненной или сниженной фракцией выброса ЛЖ, плохо поддающейся терапии, кроме того – поражение почек (гипоальбуминемия, протеинурия, отеки) [13, 14]

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Summary

Описание случая

Цель исследования – описать клинический случай системного амилоидоза. На основании данных анамнеза (нормохромная анемия, протеинурия, повышение креатинина в крови до 246 мкмоль / л), клинической картины, результатов инструментальных исследований (рестриктивная кардиомиопатия, двусторонний гидроторакс) было предполо­ жено системное поражение в виде амилоидоза почек и сердца с развитием хронической болезни почек С5 и хронической сердечной недостаточности стадии IIБ, III функционального класса. Данное клиническое наблюдение является примером поздней диагностики системного амилоидоза и посмертной верификации диагноза, что связано с неспецифичностью симптомов, а также быстро прогрессирующим течением заболевания. The aim of the work was to study the clinical case of systemic amyloidosis. Based on anamnesis data (normochromic anemia, proteinuria, increased creatinine in the blood), clinical picture, data of instrumental studies (restrictive cardiomyopathy, bilateral hydrothorax) clinical diagnosis was made: systemic amyloidosis the kidneys and heart with chronic kidney disease C5 and chronic heart failure. The histological verification of the diagnosis was carried out at the autopsy

Conclusion
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