Aim. To describe prognostic meaning of cardiac and other principal clinical manifestations of systemic AL-amyloidosis in their interrelations.Material and methods. It has been made long-time survival analysis of 147 patients with systemic AL-amyloidosis. In the special investigation group (n=58) of AL (n=55) and ATTR (n=3) amyloidotic cardiopathy patients there were evaluated prognostically important structural and functional changes in myocardium with standard and impulse-wave tissue dopplerometric echocardiography in comparison with NTproBNP serum levels.Results. Even though significantly increased nowadays surviving of AL-amylodotic patients (Me=90 months) it has been found that as at previously time orthostatic hypotension and amyloid cardiopathy are being most severe initial syndromes (median 25 months), but after 1 year from diagnosis influence of these syndromes on surviving had decreased and most low surviving was more common in patients with CKD 3-5 (median 28 months). Influence of CKD 3-5 on surviving was associated predominantly with intracardial hemodynamics deterioration. Together with decreased systolic shortening strain rate (48,5%) decreased filtration rate (47,9%) was second of main factors contributing into NTproBNP increasing in effective multiple regression model (R=0,702, F(4,21)=5,095, p=0,005). NTproBNP level in less degree depended on renal clearance.Conclusion. Heart damage is one of the most prognostically unfavorable manifestations of systemic amyloidosis due to a sharp deterioration in the elastic properties of the myocardium, in the process of further development of amyloidosis, the leading factor in progression is the deterioration of the profile of cardiorenal interactions, the marker of which is the level of NTproBNP.
Aim. To investigate an influence of the currently changed etiologic structure of AA-amyloidosis on the diagnosis and treatment tactics.
Materials and methods. In 110 patients with АА-amyloidosis followed during full disease duration (1 month 29 years) etiology, clinical manyfestations and approaches to diagnose and treatment of AA-amyloidosis were evaluated. With ELISA levels of amyloid precursor acute phase inflammation reactant SAA and neutrophil activity marker S100A12 were measured.
Results. Among the most common causes of AA-amyloidosis at the present stage, in addition to RA (40.3%), a significant place is occupied by a group of diseases with a predominantly autoinflammatory mechanism (53.73%). To confirm the autoinflammatory mechanism of the predisposing disease it is recommended to study a highly sensitive parameter serum protein S100A12. An effective marker of the risk of AA-amyloidosis progression, especially in patients with subclinical activity of inflammatory disease, is a high level of production of amyloidogenic protein-a precursor of SAA.
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.
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