32 results on '"Rameev, VV"'
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2. Poster session Wednesday 11 December all day display: 11/12/2013, 09:30-16:00 * Location: Poster area
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Bertrand, PB, Grieten, L, Smeets, C, Verbrugge, FH, Mullens, W, Vrolix, M, Rivero-Ayerza, M, Verhaert, D, Vandervoort, P, Tong, L, Ramalli, A, Tortoli, P, Dhoge, J, Bajraktari, G, Lindqvist, P, Henein, MY, Obremska, M, Boratynska, MB, Kurcz, JK, Zysko, DZ, Baran, TB, Klinger, MK, Darahim, K, Mueller, H, Carballo, D, Popova, N, Vallee, J-P, Floria, M, Chistol, R, Tinica, G, Grecu, M, Rodriguez Serrano, M, Osa-Saez, A, Rueda-Soriano, J, Buendia-Fuentes, F, Domingo-Valero, D, Igual-Munoz, B, Alonso-Fernandez, P, Quesada-Carmona, A, Miro-Palau, V, Palencia-Perez, M, Bech-Hanssen, O, Polte, CL, Lagerstrand, K, Janulewicz, M, Gao, S, Erdogan, E, Akkaya, M, Bacaksiz, A, Tasal, A, Sonmez, O, Turfan, M, Kul, S, Vatankulu, MA, Uyarel, H, Goktekin, O, Mincu, RI, Magda, LS, Mihaila, S, Florescu, M, Mihalcea, D, Enescu, OE, Chiru, A, Popescu, B, Tiu, C, Vinereanu, D, 112/2011, Research grant, Broch, K, Kunszt, G, Massey, R, De Marchi, SF, Aakhus, S, Gullestad, L, Urheim, S, Yuan, L, Feng, JL, Jin, XY, Bombardini, T, Casartelli, M, Simon, D, Gaspari, MG, Procaccio, F, Hasselberg, NE, Haugaa, KH, Brunet, A, Kongsgaard, E, Donal, E, Edvardsen, T, Sahin, TAYLAN, Yurdakul, S, Cengiz, BETUL, Bozkurt, AYSEN, Aytekin, SAIDE, Cesana, F, Spano, F, Santambrogio, G, Alloni, M, Vallerio, P, Salvetti, M, Carerj, S, Gaibazzi, N, Rigo, F, Moreo, A, Group, APRES Collaborative, Wdowiak-Okrojek, K, Michalski, B, Kasprzak, JD, Shim, A, Lipiec, P, Generati, G, Pellegrino, M, Bandera, F, Donghi, V, Alfonzetti, E, Guazzi, M, Marcun, R, Stankovic, I, Farkas, J, Vlahovic-Stipac, A, Putnikovic, B, Kadivec, S, Kosnik, M, Neskovic, AN, Lainscak, M, Iliuta, L, Szymanski, P, Lipczynska, M, Klisiewicz, A, Sobieszczanska-Malek, M, Zielinski, T, Hoffman, P, Gjerdalen, G F, Hisdal, J, Solberg, EE, Andersen, TE, Radunovic, Z, Steine, K, Svanadze, A, Poteshkina, N, Krylova, N, Mogutova, P, Shim, A, Kasprzak, JD, Szymczyk, E, Wdowiak-Okrojek, K, Michalski, B, Stefanczyk, L, Lipiec, P, Benedek, T, Matei, C, Jako, B, Suciu, ZS, Benedek, I, Yaroshchuk, N A, Kochmasheva, V V, Dityatev, V P, Kerbikov, O B, Przewlocka-Kosmala, M, Orda, A, Karolko, B, Mysiak, A, Kosmala, W, Rechcinski, T, Wierzbowska-Drabik, K, Lipiec, P, Chmiela, M, Kasprzak, JD, Aziz, A, Hooper, J, Rayasamudra, S, Uppal, H, Asghar, O, Potluri, R, Zaroui, A, Mourali, MS, Rezine, Z, Mbarki, S, Jemaa, M, Aloui, H, Mechmeche, R, Farhati, A, Gripari, P, Maffessanti, F, Tamborini, G, Muratori, M, Fusini, L, Vignati, C, Bartorelli, AL, Alamanni, F, Agostoni, PG, Pepi, M, Ruiz Ortiz, M, Mesa, D, Delgado, M, Seoane, T, Carrasco, F, Martin, M, Mazuelos, F, Suarez De Lezo Herreros De Tejada, J, Romero, M, Suarez De Lezo, J, Brili, S, Stamatopoulos, I, Misailidou, M, Chrisochoou, C, Christoforatou, E, Stefanadis, C, Ruiz Ortiz, M, Mesa, D, Delgado, M, Martin, M, Seoane, T, Carrasco, F, Ojeda, S, Segura, J, Pan, M, Suarez De Lezo, J, Cammalleri, V, Ussia, GP, Muscoli, S, Marchei, M, Sergi, D, Mazzotta, E, Romeo, F, Igual Munoz, B, Bel Minguez, ABM, Perez Guillen, MPG, Maceira Gonzalez, AMG, Monmeneu Menadas, JVMM, Hernandez Acuna, CHA, Estornell Erill, JEE, Lopez Lereu, PLL, Francisco Jose Valera Martinez, FJVM, Montero Argudo, AMA, Sunbul, M, Akhundova, A, Sari, I, Erdogan, O, Mutlu, B, Cacicedo, A, Velasco Del Castillo, S, Anton Ladislao, A, Aguirre Larracoechea, U, Rodriguez Sanchez, I, Subinas Elorriaga, A, Oria Gonzalez, G, Onaindia Gandarias, J, Laraudogoitia Zaldumbide, E, Lekuona Goya, I, Ding, W, Zhao, Y, Lindqvist, P, Nilson, J, Winter, R, Holmgren, A, Ruck, A, Henein, MY, Attenhofer Jost, C H, Soyka, R, Oxenius, A, Kretschmar, O, Valsangiacomo Buechel, ER, Greutmann, M, Weber, R, Keramida, K, Kouris, N, Kostopoulos, V, Karidas, V, Damaskos, D, Makavos, G, Paraskevopoulos, K, Olympios, CD, Eskesen, K, Olsen, NT, Fritz-Hansen, T, Sogaard, P, Cameli, M, Lisi, M, Righini, FM, Curci, V, Massoni, A, Natali, B, Maccherini, M, Chiavarelli, M, Massetti, M, Mondillo, S, Mabrouk Salem Omar, A, Ahmed Abdel-Rahman, M, Khorshid, H, Rifaie, O, Santoro, C, Santoro, A, Ippolito, R, De Palma, D, De Stefano, F, Muscariiello, R, Galderisi, M, Squeri, A, Censi, S, Baldelli, M, Grattoni, C, Cremonesi, A, Bosi, S, Saura Espin, D, Gonzalez Canovas, C, Gonzalez Carrillo, J, Oliva Sandoval, MJ, Caballero Jimenez, L, Espinosa Garcia, MD, Garcia Navarro, M, Valdes Chavarri, M, De La Morena Valenzuela, G, Ryu, SK, Shin, DG, Son, JW, Choi, JH, Goh, CW, Choi, JW, Park, JY, Hong, GR, Sklyanna, O, Yuan, L, Yuan, L, Planinc, I, Bagadur, G, Ljubas, J, Baricevic, Z, Skoric, B, Velagic, V, Bijnens, B, Milicic, D, Cikes, M, Gospodinova, M, Chamova, T, Guergueltcheva, V, Ivanova, R, Tournev, I, Denchev, S, Ancona, R, Comenale Pinto, S, Caso, P, Arenga, F, Coppola, MG, Calabro, R, Neametalla, H, Boitard, S, Hamdi, H, Planat-Benard, V, Casteilla, L, Li, Z, Hagege, AA, Mericskay, M, Menasche, P, Agbulut, O, Merlo, M, Stolfo, D, Anzini, M, Negri, F, Pinamonti, B, Barbati, G, Di Lenarda, A, Sinagra, G, Stolfo, D, Merlo, M, Pinamonti, B, Gigli, M, Poli, S, Porto, A, Di Nora, C, Barbati, G, Di Lenarda, A, Sinagra, G, Coppola, C, Piscopo, G, Cipresso, C, Rea, D, Maurea, C, Esposito, E, Arra, C, Maurea, N, Nemes, A, Kalapos, A, Domsik, P, Forster, T, Voilliot, D, Huttin, O, Vaugrenard, T, Schwartz, J, Sellal, J-M, Aliot, E, Juilliere, Y, Selton-Suty, C, Sanchez Millan, P J, Cabeza Lainez, P, Castillo Ortiz, J, Chueca Gonzalez, EM, Gheorghe, L, Fernandez Garcia, P, Herruzo Rojas, MS, Del Pozo Contreras, R, Fernandez Garcia, M, Vazquez Garcia, R, Rosca, M, Popescu, BA, Botezatu, D, Calin, A, Beladan, CC, Gurzun, M, Enache, R, Ginghina, C, Farouk, H, Al-Maimoony, T, Alhadad, A, El Serafi, M, Abdel Ghany, M, Poorzand, H, Mirfeizi, SZ, Javanbakht, A, center, Preventive Cardiovascular care research, center, Lupus Research, sciences, Mashhad university of medical, Tellatin, S, Famoso, G, Dassie, F, Martini, C, Osto, E, Maffei, P, Iliceto, S, Tona, F, Radunovic, Z, Steine, KS, Jedrzejewska, I, Braksator, W, Krol, W, Swiatowiec, A, Sawicki, J, Kostarska-Srokosz, E, Dluzniewski, M, Maceira Gonzalez, A M, Cosin-Sales, J, Diago, JL, Aguilar, J, Ruvira, J, Monmeneu, J, Igual, B, Lopez-Lereu, MP, Estornell, J, Olszanecka, A, Dragan, A, Kawecka-Jaszcz, K, Czarnecka, D, Scholz, F, Gaudron, PD, Hu, K, Liu, D, Florescu, C, Herrmann, S, Bijnens, B, Ertl, G, Stoerk, S, Weidemann, F, Krestjyaninov, M, Razin, VA, Gimaev, RH, Bogdanovic, Z, Burazor, I, Deljanin Ilic, M, Peluso, D, Muraru, D, Cucchini, U, Mihaila, S, Casablanca, S, Pigatto, E, Cozzi, F, Punzi, L, Badano, LP, Iliceto, S, Zhdanova, E, Rameev, VV, Safarova, AF, Moisseyev, SV, Kobalava, ZD, Magnino, C, Omede, P, Avenatti, E, Presutti, D, Losano, I, Moretti, C, Bucca, C, Gaita, F, Veglio, F, Milan, A, Bellsham-Revell, H, Bell, AJ, Miller, OI, Simpson, JM, Hwang, YM, Kim, GH, Jung, MH, Woo, GH, Medicine, Department of Internal, Hospital, St.Vincents, Korea, The Catholic University of, Suwon, Division of Cardiology, Repu, Driessen, MMP, Leiner, T, Schoof, PH, Breur, JMPJ, Sieswerda, GT, Meijboom, FJ, Bellsham-Revell, H, Hayes, N, Anderson, D, Austin, BC, Razavi, R, Greil, GF, Simpson, JM, Bell, AJ, Zhao, XX, Xu, XD, Qin, YW, Szmigielski, C A, Styczynski, G, Sobczynska, M, Placha, G, Kuch-Wocial, A, Ikonomidis, I, Voumbourakis, A, Triantafyllidi, H, Pavlidis, G, Varoudi, M, Papadakis, I, Trivilou, P, Paraskevaidis, I, Anastasiou-Nana, M, Lekakis, I, Kong, WILL, Yip, JAMES, Ling, LH, Milan, A, Tosello, F, Leone, D, Bruno, G, Losano, I, Avenatti, E, Sabia, L, Veglio, F, Zaborska, B, Baran, J, Pilichowska-Paszkiet, E, Sikora-Frac, M, Michalowska, I, Kulakowski, P, Budaj, A, Mega, S, Bono, MC, De Francesco, V, Castiglione, I, Ranocchi, F, Casacalenda, A, Goffredo, C, Patti, G, Di Sciascio, G, Musumeci, F, Kennedy, M, Waterhouse, DF, Sheahan, R, Foley, DF, Mcadam, BF, Ancona, R, Comenale Pinto, S, Caso, P, Arenga, F, Coppola, MG, Calabro, R, Remme, E W, Smedsrud, M K, Hasselberg, N E, Smiseth, O A, Edvardsen, T, Halmai, L, Nemes, A, Kardos, A, Neubauer, S, Degiovanni, A, Baduena, L, Dellera, G, Occhetta, E, Marino, P, Hotchi, J, Yamada, H, Nishio, S, Bando, M, Hayashi, S, Hirata, Y, Amano, R, Soeki, T, Wakatsuki, T, Sata, M, Lamia, B, Molano, LC, Viacroze, C, Cuvelier, A, Muir, JF, Lipczynska, M, Piotr Szymanski, PS, Anna Klisiewicz, AK, Lukasz Mazurkiewicz, LM, Piotr Hoffman, PH, Van T Sant, J, Wijers, SC, Ter Horst, IAH, Leenders, GE, Cramer, MJ, Doevendans, PA, Meine, M, Hatam, N, Goetzenich, A, Aljalloud, A, Mischke, K, Hoffmann, R, Autschbach, R, Sikora-Frac, M, Zaborska, B, Maciejewski, P, Bednarz, B, Budaj, A, Evangelista, A, Torromeo, C, Pandian, NG, Nardinocchi, P, Varano, V, Schiariti, M, Teresi, L, Puddu, PE, Storve, S, Dalen, H, Snare, SR, Haugen, BO, Torp, H, Fehri, W, Mahfoudhi, H, Mezni, F, Annabi, MS, Taamallah, K, Dahmani, R, Haggui, A, Hajlaoui, N, Lahidheb, D, Haouala, H, Colombo, A, Carminati, MC, Maffessanti, F, Gripari, P, Pepi, M, Lang, RM, Caiani, EG, Walker, JR, Abadi, S, Agmon, Y, Carasso, S, Aronson, D, Mutlak, D, Lessick, J, Saxena, A, Ramakrishnan, S, Juneja, R, Ljubas, J, Reskovic Luksic, V, Matasic, R, Pezo Nikolic, B, Lovric, D, Separovic Hanzevacki, J, Quattrone, A, Zito, C, Alongi, G, Vizzari, G, Bitto, A, De Caridi, G, Greco, M, Tripodi, R, Pizzino, G, Carerj, S, Ibrahimi, P, Jashari, F, Johansson, E, Gronlund, C, Bajraktari, G, Wester, P, Henein, MY, Kosmala, W, Marwick, TH, Souza, J R M, Zacharias, L G T, Geloneze, B, Pareja, J C, Chaim, A, Nadruz, W JR, Coelho, O R, Apostolovic, S, Stanojevic, D, Jankovic-Tomasevic, R, Salinger-Martinovic, S, Djordjevic-Radojkovic, D, Pavlovic, M, Tahirovic, E, Musial-Bright, L, Lainscak, M, Duengen, HD, group, CIBIS ELD study, Filipiak, D, Kasprzak, JD, and Lipiec, P
- Abstract
Purpose: With the advent of percutaneous transcatheter device closures in congenital heart defects and the emergence of percutaneous left atrial appendage closure, there is an increasingly important role for echocardiographic guidance and control of device position and function. Disc occluder devices frequently present as an unexplained ‘figure-of-8’ on echocardiography. The aim of this study was to clarify this ‘figure-of-8’ display and to relate its morphology to transducer position and device type. Methods: A mathematical model was developed to resemble disc occluder geometry and to allow a numerical simulation of the echocardiographic appearance. In addition, we developed an in vitro set-up for echocardiographic analysis of various disc occluders and various transducer positions. Results: In the mathematical model of an epitrochoid curve (closely resembling disc occluder geometry) a ‘figure-of-8’ display is obtained when emphasizing points with tangent vector perpendicular to the direction of ultrasound waves. Decreasing imaging depth results in a more asymmetric ‘figure-of-8’, with small upper part and wide lower part. Clinical and in vitro data are in close agreement with these results (Figure 1). Furthermore a ‘figure-of-8’ display is only obtained in a coronal imaging position, and is similar for different commercially available disc occluder types. Conclusions: The ‘figure-of-8’ display in the ultrasound image of a disc occluder is an imaging artifact due to the specific ‘epitrochoidal’ geometry of a deployed device and its interaction with ultrasound waves. The morphology of the ‘figure-of-8’ depends on transducer position, i.e. imaging depth, and is similar for different device types.
Figure 1 Impact of imaging depth - Published
- 2013
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3. Schnitzler's Syndrome-Diagnostic Experience, Approaches to Therapy, and Patient Management according to a Multicenter Russian Cohort.
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Salugina SO, Torgashina AV, Borzova EY, Rameev VV, Gorodetsky VR, Fedorov ES, and Muravyova NV
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- Humans, Male, Female, Middle Aged, Adult, Aged, Russia epidemiology, Aged, 80 and over, Retrospective Studies, Cohort Studies, Schnitzler Syndrome drug therapy, Schnitzler Syndrome diagnosis
- Abstract
The objectives of the study were to present the experience of diagnosis, management, and therapy with IL-1 inhibitors in patients with Schnitzler's syndrome (SchS) according to a multicenter Russian cohort. An observational retrospective study for a 10-year period (2012-2022) involved 17 patients with SchS who were admitted to the hospital or were observed on an outpatient basis (eight women and nine men). The diagnosis of all of them corresponded to the Strasbourg diagnostic criteria. The age of patients ranged from 25 to 81 years (Me 53[46; 56]). The age at the time of the onset of the disease ranged from 20 to 72 years (Me 46[39; 54]), the duration of the disease before diagnosis ranged from 1 to 35 years (Me 6.5[3; 6]), in three patients it exceeded 10 years, in the rest it ranged from 1 to 8 years. Infectious and lymphoproliferative diseases, monogenic AIDs (CAPS, TRAPS, and HIDS) were excluded from all patients at the prehospital stage. The referral diagnosis for all of them was Still 's disease in adults. Clinical manifestations of the disease in all patients included fatigue, lethargy, fatigue, rash, and fever. In all patients, skin elements were urticular and were accompanied by itching in 6 (37.5%) patients. Bone pain was observed in 12 (70.6%) patients; arthralgias, in 16 (94.1%); arthritis, in 9 (52.9%); myalgia, in 7 (41.2%); and weight loss, in 4 (23.5%). Lymphadenopathy was detected in 6 (35.3%) patients; enlarged liver, in 6 (35.3%); pericarditis, in 4 (23.5%); angioedema, in 6 (35.3); redness and dryness in the eyes, in 3 (17.6%); sore throat, in 2 (11.8%); abdominal pain, in 1 (5.9%), distal polyneuropathy, in 2 (11.8%); paraesthesia, in 1 (5.9%); and chondritis of the auricles, in 1 (5.9%). Monoclonal gammopathy was detected in all patients with a secretion level of 2.9-15.1 g/L: IgMk (n = 10, 64.7%), less often IgMλ (n = 2), IgGk (n = 2), IgGλ (n = 1), and IgAλ (n = 1). Ben-Jones protein was not detected in any of them. All patients had an increased level of ESR and CRP. Before inclusion in the study, 16 patients received GCs (94.1%) with a temporary effect that disappeared with dose reduction or cancellation. Seven patients received cDMARDs, including methotrexate (5), hydroxychloroquine (2), and cyclophosphamide (1). All patients received NSAIDs and antihistamines, as well as biologics, including the anti-B-cell drug rituximab (1), monoclonal ABs to IgE omalizumab (2, 1 without effect and 1 with partial effect), IL-1i canakinumab (n = 10, 58.8%) subcutaneously once every 8 weeks, and anakinra (n = 4, 23.5%) subcutaneously daily. The duration of taking anakinra, which was prescribed in the test mode, ranged from 1 week to 2.5 months with a further switch to canakinumab in 3 patients. The duration of taking canakinumab at the time of analysis ranged from 7 months to 8 years. Against the background of treatment with IL-1i, 10 out of 11 (90.9%) patients received a complete response in terms of the clinical manifestations of the disease and a decrease in the level of ESR and CRP within a few days. In one patient, a partial response to the administration of anakinra was detected; however, after switching to canakinumab, the effect of treatment was finally lost. One patient received IL-6i for 8 months with an incomplete effect and a positive dynamics after switching to anakinra. Thus, anakinra was initially prescribed to four patients and changed to canakinumab in two of them; canakinumab was started as the first drug in seven patients. Treatment with anakinra was continued in two patients; with canakinumab, in nine patients. In one patient, due to the persistent absence of relapses, the interval between canakinumab injections was increased to 5 months without signs of reactivation; however, subsequently, against the background of stress and relapses of the disease, the intervals were reduced to 4 months. A healthy child was born by the same patient on the background of treatment. The tolerability of therapy was satisfactory in all patients, no SAEs were noted. SchS is a rare multifactorial/non-monogenic AID that should be differentiated from a number of rheumatic diseases and other AIDs. The onset in adulthood, the presence of recurrent urticarial rashes in combination with fever and other manifestations of a systemic inflammatory response are indications for examination for monoclonal secretion. The use of short- or long-acting IL-1i is a highly effective and safe option in the treatment of such patients., (© 2024. Pleiades Publishing, Ltd.)
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- 2024
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4. [Challenges in diagnosing familial Mediterranean fever: exploring atypical clinical features. Clinical case].
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Barsuk MV, Novikov AV, Mikhalina TA, Rameev VV, and Lysenko LV
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- Humans, Male, Female, Adult, Genetic Testing methods, Colchicine therapeutic use, Pyrin genetics, Diagnosis, Differential, Familial Mediterranean Fever diagnosis, Familial Mediterranean Fever genetics
- Abstract
This clinical case series presents descriptions of 3 patients with familial Mediterranean fever (FMF) who have atypical manifestations and abnormal inheritance mechanisms in terms of Gregor Mendel's laws. Although molecular genetic testing can help with disease diagnosis, it is not always conclusive. The primary need for genetic testing in atypical cases is to explain the mechanism of inflammation and to select the optimal therapy. These clinical observations demonstrate the changes in the spectrum of phenotypic manifestations of FMF in the context of the widespread introduction of molecular genetic methods.
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- 2024
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5. [History of the study of amyloidosis: from the Rokitansky's theory to the present day].
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Rameev VV and Lysenko LV
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- Humans, History, 20th Century, History, 21st Century, History, 19th Century, Amyloidosis diagnosis, Amyloidosis history, Amyloidosis metabolism
- Abstract
In the history of amyloidosis studying the concept of liquids dyscrasia has been predominated and finally it is resulted in accepting a serum protein-precursor as a leading amyloidogenic factor in the disease pathogenesis. Consequently basic diagnostic and treatment strategy was aimed to find and eliminate this protein from the blood and this approach evidenced high effectiveness in most frequent AA and AL-amyloidosis characterized with anomaly high levels of precursors in the blood. At the same time there are less frequent and slower progressing inheritant forms of systemic amyloidosis including transthyretin induced, which are less depending on amyloidogenecity of amyloid precursor and because of that, in example, the effectiveness of transthyretin stabilizers or blockers of its synthesis is limited comparing with the precursor elimination in AA or AL. Developed in the middle of XX century a theory of local synthesis by macrophages is more preferable to describe the pathogenesis of these forms. And modern proteome analysis using give rise to confirm the key meaning of macrophage in the amyloidogenesis and proves necessity to know deeply mechanisms of macrophagial autophagia - basic tool of maintaining intracellular protein homeostasis. That is why it is difficult to hope on high effectiveness of chemical amyloid solvents in vivo, which being under macrophages regulation never could realize its chemical activities.
- Published
- 2024
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6. First Report of Lysozyme Amyloidosis with p.F21L/T88N Amino Acid Substitutions in a Russian Family.
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Suvorina MY, Stepanova EA, Rameev VV, Kozlovskaya LV, Glukhov AS, Kuznitsyna AA, Surin AK, and Galzitskaya OV
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- Humans, Amino Acid Substitution, Amyloid genetics, Amyloid metabolism, Mutation, Amyloidosis genetics, Muramidase genetics, Muramidase chemistry
- Abstract
Lysozyme amyloidosis is caused by an amino acid substitution in the sequence of this protein. In our study, we described a clinical case of lysozyme amyloidosis in a Russian family. In our work, we described in detail the histological changes in tissues that appeared as a result of massive deposition of amyloid aggregates that affected almost all organ systems, with the exception of the central nervous system. We determined the type of amyloidosis and mutations using mass spectrometry. Using mass spectrometry, the protein composition of tissue samples of patient 1 (autopsy material) and patient 2 (biopsy material) with histologically confirmed amyloid deposits were analyzed. Amino acid substitutions p.F21L/T88N in the lysozyme sequence were identified in both sets of samples and confirmed by sequencing of the lysozyme gene of members of this family. We have shown the inheritance of these mutations in the lysozyme gene in members of the described family. For the first time, we discovered a mutation in the first exon p.F21L of the lysozyme gene, which, together with p.T88N amino acid substitution, led to amyloidosis in members of the studied family.
- Published
- 2023
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7. [The analysis of secondary AA-amyloidosis current etiology and its influence on the approaches for diagnosis and treatment].
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Rameev VV, Kozlovskaya LV, Rameeva AS, Novikov AV, and Barsuk MV
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- Humans, S100A12 Protein, Inflammation, Serum Amyloid A Protein metabolism, Amyloidosis
- Abstract
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.
- Published
- 2021
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8. [Monoclonal gammopathy of renal significance: consensus of hematologists and nephrologists of Russia on the establishment of nosology, diagnostic approach and rationale for clone specific treatment].
- Author
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Smirnov AV, Afanasyev BV, Poddubnaya IV, Dobronravov VA, Khrabrova MS, Zakharova EV, Nikitin EA, Lysenko Kozlovskaya LV, Bobkova IN, Rameev VV, Batyushin MM, Moiseev IS, Darskaya EI, Pirogova OV, Mendeleeva LP, and Biryukova LS
- Subjects
- Clone Cells, Consensus, Humans, Kidney, Nephrologists, Russia, Kidney Diseases, Paraproteinemias diagnosis, Paraproteinemias therapy
- Abstract
Monoclonal gammopathy of renal significance (MGRS) is a new nosology in modern nephrology and oncohematology. MGRS is defined as kidney injury due to nephrotoxic monoclonal immunoglobulin produced by the B-cell line clone which does not reach the hematological criteria for specific treatment initiation. Monoclonal proteins pathological effects on kidney parenchyma result in irreversible decline of kidney function till the end stage renal disease that in line with the position of International Consensus of hematologists and nephrologists determinates critical necessity for clone specific treatment in patients with MGRS despite the absence of hematological indications for treatment initiation. Main challenge of MGRS in Russian Federation is an inaccessibility of an in-time diagnostic and appropriate treatment for the great majority of patients due to the following reasons: 1) limited knowledge about the MGRS among hematologists and nephrologists; 2) lack of necessary diagnostic resources in most health-care facilities; 3) lack of approved clinical recommendations and medical economic standards for treatment of this pathological entity. Consensus document comprises the opinion of experts leading nephrologists and hematologists of Russian Federation on the problem of MGRS including the incoherence in nosology classification, diagnostics approach and rationale for clone specific treatment. Consensus document is based on conclusions and agreements reached during the conference of leading nephrologists and hematologists of Russia which was held in the framework of symposia Plasma cell dyscrasias and lymphoproliferative diseases: modern approaches to therapy, 1516 of March 2019, Pavlov First Saint Petersburg State Medical University. The present Consensus is intended to define the principal practical steps to resolve the problem of MGRS in Russian Federation that are summarized as final clauses.
- Published
- 2020
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9. [Monoclonal gammapathy of renal significance (MGRS) at the current state: terminology, diagnosis and treatment].
- Author
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Lysenko Kozlovskaya LV, Rameev VV, and Androsova TV
- Subjects
- Humans, Kidney, Retrospective Studies, Kidney Diseases diagnosis, Kidney Diseases etiology, Kidney Diseases therapy, Monoclonal Gammopathy of Undetermined Significance, Paraproteinemias complications, Paraproteinemias diagnosis, Paraproteinemias therapy
- Abstract
In this article we discussed the current state of monoclonal gammapathy of renal significance (Monoclonal Gammopathy of Renal Significance MGRS) and revealed problems of B-cell clone secreting nephrotoxic monoclonal immunoglobulin identification. We followed 276 patients with monoclonal gammapathy including patients with non-amyloid nephropathy. The majority of patients had systemic AL-amyloidosis. We established better survival of the treated patients with systemic AL-amyloidosis in comparison with retrospective untreated cohort. We considered current treatment of patients with non-amyloid nephropathy and focused on the crucial role of multidisciplinary approach in management of these patients.
- Published
- 2020
- Full Text
- View/download PDF
10. [Modern approaches to the detection of monoclonal gammopathy of undetermined significance (MGUS) in patients with kidney diseases].
- Author
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Kozlovskaya Lysenko LV, Chebotareva NV, Mrykhin NN, Rameev VV, Androsova TV, Roshchupkina SV, Maryina SA, Kogarko IN, and Kogarko BS
- Abstract
Monoclonal gammopathy (MG) is not only the state preceding of hematological neoplasms, but also associated with non - hematological diseases, in particular kidney damage., Aim: To assess the diagnostic value of "Freelite" methods in addition to electrophoresis (EF) and immunofixation (IF) of serum and urine proteins for detecting MG in patients with kidney diseases., Materials and Methods: 87 patients with kidney damage, in which MG was established using the method of electrophoresis of serum proteins (EF), immunofixation (IF) and the method of free light chains determination - FLC "Freelite" were selected. The diagnostic value of three - component serum panel was compared with EF and IF., Results and Discussion: AL-amyloidosis with kidney involvement was diagnosed in 41% patients, cryoglobulinemic glomerulonephritis (cryo GN) - in 18%, chronic glomerulonephritis (CGN) - in 35%, also there was small number of patients with light chain disease and cast - nephropathy. Determination of MG using EP was possible only in 38 (44%). Adding to the serum electrophoretic methods instead of the "Freelite" method, the urine EF and IF reduced the number of missed patients with monoclonal gammopathy from 24 (27%) to 11 (13%), including in the subgroup of patients with AL-amyloidosis but did not reach the sensitivity of the three - component serum screening panel. In 10 (11.5%) MG was represented only by intact mIg with one type of light chain, either κ or λ. Most often - in 25% of patients, intact monoclonal gammopathy was observed in HCV (+) cryo GN. A combination of intact mIgM, mIgG or mIgA with mFLC, was detected in 37 (42.5%). In almost half (46%) of the patients, only mFLC was detected - an abnormal κ/λ ratio., Conclusion: The serum screening panel EF + IF + "Freelite" spreads the low - grade monoclonal gammopathy recognition (MGUS) and should be included in the algorithm of examining patients with kidney disease.
- Published
- 2019
- Full Text
- View/download PDF
11. [Autoinflammatory diseases and kidney involvement].
- Author
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Mukhin NA, Bogdanova MV, Rameev VV, and Kozlovskaya LV
- Subjects
- Humans, Autoimmune Diseases immunology, Inflammation immunology, Kidney Diseases immunology
- Abstract
Autoinflammatory disease (AID) is a new concept formulated from the results of studying the pathogenesis of familial periodic fevers, a heterogeneous group of genetically determined diseases characterized by causelessly recurrent exacerbations of the inflammatory process due to genetically determined disorders of innate immunity and accompanied by uncontrolled hypersecretion of interleukin-1 (IL-1). These mechanisms were a basic model for understanding a wide range of rheumatologic and other inflammatory diseases of the internal organs. The late diagnosis of AIDs and their ineffective treatment increase the risk for the development and progression of secondary AA amyloidosis. Elaboration of both clinical and effective laboratory criteria for diagnosing autoinflammation is of great importance for determining the tactics of anti-inflammatory therapy and prevention of complications.
- Published
- 2017
- Full Text
- View/download PDF
12. [Oligosecretory monoclonal gammopathy with renal involvement].
- Author
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Kozlovskaya LV, Rameev VV, Androsova TV, Kogarko IN, Kogarko BS, Mrykhin NN, and Rekhtina IG
- Subjects
- Diagnosis, Differential, Disease Management, Disease Progression, Humans, Prognosis, Glomerulonephritis, Membranoproliferative diagnosis, Glomerulonephritis, Membranoproliferative etiology, Glomerulonephritis, Membranoproliferative therapy, Kidney pathology, Monoclonal Gammopathy of Undetermined Significance complications, Monoclonal Gammopathy of Undetermined Significance diagnosis, Monoclonal Gammopathy of Undetermined Significance immunology, Monoclonal Gammopathy of Undetermined Significance therapy
- Abstract
The article deals with the so-called monoclonal gammopathy of undetermined significance (MGUS), which is being actively explored in the world and has been recently investigated in Russia. It indicates the principles of identifying the phenotypes of MGUS and criteria for assessing the risk of its progression to cancer. There is an update on the possible involvement of monoclonal proteins in the pathogenesis of certain non-neoplastic kidney diseases, renal injuries in particular. The paper gives their classification and enumerates differential diagnostic techniques, including the Freelite method, a highly sensitive one to determine free light chains (FLC), prognostic criteria, and approaches to treating each separate form in relation to the phenotype of a monoclonal protein. The authors present their own data on detection rates for MGUS at a multidisciplinary hospital and a clinical case of MGUS-associated membranoproliferative glomerulonephritis, by justifying a treatment regimen containing bortezomib (velcade).
- Published
- 2016
- Full Text
- View/download PDF
13. [Serum calgranulin C is a highly sensitive autoinflammation activity indicator in patients with familial periodic fevers].
- Author
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Bogdanova MV, Rameev VV, Kozlovskaya LV, Fedorov ES, and Salugina SO
- Subjects
- Adolescent, Biomarkers blood, Blood Sedimentation, C-Reactive Protein analysis, Child, Preschool, Female, Fibrinogen analysis, Humans, Leukocyte Count, Male, Middle Aged, Patient Acuity, Reproducibility of Results, Sensitivity and Specificity, Familial Mediterranean Fever blood, Familial Mediterranean Fever diagnosis, Familial Mediterranean Fever physiopathology, Inflammation blood, Inflammation physiopathology, S100A12 Protein blood
- Abstract
Aim: To determine the possibility of using the serum proinflammatory calcium-binding protein, or calgranulin C (S100A12), to assess activity and therapeutic efficiency in patients with periodic disease (PD) and other familial periodic fevers (FPFs)., Subjects and Methods: Thirty-five patients with PD and other FPDs, which were verified by molecular genetic study, were examined. In accordance with the disease activity, the patients were divided into 2 groups. The investigators determined the concentration of S100A12 by solid-phase enzyme immunoassay and that of other acute-phase inflammatory markers (erythrocyte sedimentation rate (ERT), neutrophil counts, and fibrinogen and C-reactive protein (CRP) concentrations)., Results: The serum concentration of S100A12 in the stage of disease activity was 466.7 (265.22--851.7) ng/ml, which was significantly higher than in remission (244.29 (118.93--409.85) ng/ml (p=0.000002). The highest S100A12 concentrations were noted in the patients with PD; these were 758.95 (434.80--1035.95) ng/ml; the S100A12 level in the majority of PD patients even during remission remained moderately higher. An investigation of the relationship of A100A12 to genetic variants found no differences between the patients homozygous for M694V and those with other genotypes (p=0.37). Estimation of the time course of therapy-induced changes in the serum S100A12 concentration revealed its considerable reduction (р=0.0018). However, normalization of S100A12 levels was not achieved in PD. The remaining increased S100A12 concentration in these patients may be suggestive of the activity of PD despite the absence of its clinical manifestations. S100A12 as a highly sensitive marker allows more exact evaluation of the anti-inflammatory effect of therapy. The S100A12 identification of the subclinical activity of autoinflammatory diseases made all the more important since traditional inflammatory markers, such as ERT, CRP, fibrinogen, and leukocyte counts, are less sensitive for these purposes. In our study, these markers were within the reference range in remission. No differences were found in the S100A12 levels between the groups with and without amyloidosis (p=0.62)., Conclusion: S100A12 is a highly sensitive marker for the activity of autoinflammatory diseases and the efficiency of their therapy. The serum level of S100A12 in PD may be used to diagnose the subclinical activity of inflammation, which is of importance in monitoring the risk of amyloidosis.
- Published
- 2016
- Full Text
- View/download PDF
14. Predictors of AA amyloidosis in familial Mediterranean fever.
- Author
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Mukhin NA, Kozlovskaya LV, Bogdanova MV, Rameev VV, Moiseev SV, and Simonyan AKh
- Subjects
- Adult, Amyloidosis blood, Amyloidosis diagnosis, Amyloidosis genetics, Armenia, Chi-Square Distribution, Cytoskeletal Proteins genetics, DNA Mutational Analysis, Familial Mediterranean Fever diagnosis, Familial Mediterranean Fever genetics, Female, Genetic Markers, Genetic Predisposition to Disease, Heterozygote, Homozygote, Humans, Male, Mutation, Odds Ratio, Phenotype, Prognosis, Pyrin, Retrospective Studies, Risk Factors, Russia, Young Adult, Amyloidosis etiology, Familial Mediterranean Fever complications, Serum Amyloid A Protein analysis
- Abstract
The aim of the study was to evaluate the clinical and genetic predictors of AA amyloidosis in patients with familial Mediterranean fever (FMF). We retrospectively studied 170 Armenian patients who were admitted to the two tertiary centers in 2003-2014. The diagnosis of amyloidosis that was suspected clinically (new proteinuria or nephrotic syndrome) was confirmed histologically. Screening for MEFV gene mutations was performed in 70 patients. The most common genotype was M694V/M694V (in 36 % of patients). Biopsy-proven AA amyloidosis was found in 102 (60 %) of 170 patients. AA amyloidosis was diagnosed in 17 (68 %) of 25 patients with homozygous M694V mutation, 17 (53 %) of 32 patients with heterozygous M694V allele and 4 (31 %) of 13 patients with other MEFV gene mutations. The M694V homozygosity and heterozygosity were associated with increased risk of AA amyloidosis, but this association did not reach statistical significance (odds ratio 2.43; 95 % CI 0.87-6.76, and 3.33; 0.91-12.1, respectively). Male gender, early onset of disease, severity of FMF, frequent attacks, peritonitis, pleuritis and erysipelas-like erythema also did not predict AA amyloidosis development. Recurrent arthritis was the only clinical finding that was significantly associated with AA amyloidosis (odds ratio 2.28; 95 % CI 1.17-4.42). Involvement of the joint synovial membrane, that is capable of active serum amyloid A production, is the main predictor of renal amyloidosis in FMF.
- Published
- 2015
- Full Text
- View/download PDF
15. [Kidney injury associated with monoclonal gammopathies: Perspectives on diagnosis and treatment].
- Author
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Kozlovskaya LV, Rameev VV, Mrykhin NN, Kogarko IN, and Kogarko BS
- Subjects
- Humans, Acute Kidney Injury diagnosis, Acute Kidney Injury etiology, Acute Kidney Injury therapy, Disease Management, Paraproteinemias complications, Paraproteinemias diagnosis, Paraproteinemias therapy
- Abstract
It is currently well justified that monoclonal gammopathies are the most important predictor for kidney diseases, including glomerulonephritis. To determine a correlation of nephropathy with oligosecretory gammopathy is of fundamental importance, as the treatment of these patients necessitates the use of special chemotherapy regimens to eliminate a pathological clone of lymphocytes or plasmocytes. If this clone is not eliminated, injury of the organ may recur to develop its failure. The principles of this therapy have been presently tried out by the example of AL-amyloidosis and showed its efficiency and relatively low toxicity.
- Published
- 2015
- Full Text
- View/download PDF
16. [Cardiorenal links in systemic amyloidosis].
- Author
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Rameev VV, Kozlovskaia LV, Zhdanova EA, and Gudkova KV
- Subjects
- Adult, Aged, Diagnosis, Differential, Fatal Outcome, Humans, Male, Amyloidosis classification, Amyloidosis diagnosis, Amyloidosis therapy, Heart Diseases classification, Heart Diseases diagnosis, Heart Diseases therapy, Kidney Diseases classification, Kidney Diseases diagnosis, Kidney Diseases therapy
- Abstract
The paper gives current general data on the structure of amyloid fibril and the principles in the classification of amyloidosis, information on the clinical course of cardiac and renal involvements in systemic AL and AA amyloidosis, and that on diagnostic and prognostic criteria and the specific features of cardiorenal links. The authors draw the conclusion that the identification of acute and chronic cardiorenal links is of practical value for systemic amyloidosis. Cardiorenal and renocardiac syndromes are not always differentiated clearly in the systemacy of involvement.
- Published
- 2013
17. [Modern conceptions of cardiac amyloidosis].
- Author
-
Zhdanova EA, Gudkova KV, Rameev VV, Safarova AF, and Moiseev SV
- Subjects
- Amyloid, Diagnosis, Differential, Heart Function Tests methods, Humans, Prognosis, Amyloidosis diagnosis, Amyloidosis etiology, Amyloidosis physiopathology, Amyloidosis therapy, Cardiovascular Diseases diagnosis, Cardiovascular Diseases etiology, Cardiovascular Diseases physiopathology, Cardiovascular Diseases therapy, Myocardium pathology
- Abstract
Cardiac amyloidosis is accumulation in the heart of a pathologic fibrillar protein amyloid. It represents a heterogeneous group of states from clinically insignificant amyloid accumulation in isolated atrial amyloidosis to severe involvement of the heart in primary amyloidosis when mean duration of life equals to 6 months. Insufficient awareness of physicians of this pathology leads to erroneous and belated diagnosis of cardiac amyloidosis. This paper contains contemporary data of pathophysiology, clinical manifestation, diagnosis, treatment, and prognosis of various variants of cardiac amyloidosis.
- Published
- 2013
18. [Muckle-Wells syndrome caused by a new cryopyrin mutation: effective treatment with interleukin-1 antagonist].
- Author
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Rameev VV, Kozlovskaia LV, and Bogdanova MV
- Subjects
- Adult, Cryopyrin-Associated Periodic Syndromes diagnosis, Female, Humans, Interleukin 1 Receptor Antagonist Protein administration & dosage, NLR Family, Pyrin Domain-Containing 3 Protein, Treatment Outcome, Carrier Proteins genetics, Cryopyrin-Associated Periodic Syndromes drug therapy, Cryopyrin-Associated Periodic Syndromes genetics, Interleukin 1 Receptor Antagonist Protein therapeutic use, Interleukin-1 antagonists & inhibitors, Mutation, Missense
- Abstract
A significant progress in the field of molecular-biological investigations resulted in definition of a new group of systemic diseases referred to as autoinflammatory. This group comprises familial periodic fevers: periodic disease (mediterranean fever), Muckle-Wells syndrome, others cryopirinopathy, TRAPS-syndrome. As shown by case reports, Muckle-Wells syndrome is not a rare disease, its sporadic forms are encountered as well as a less severe variant of cryopirinopathy - nonallergic cold urticaria. Awareness of the physicians in respect of this pathology is essential especially because early diagnosis enables control of this disease with use of biological preparations the spectrum of which tends to expansion. Moreover, arrest of inflammation is necessary for prevention of development and progression of such prognostically poor complication as AA-amyloidosis.
- Published
- 2012
19. [Current methods of systemic amyloidosis diagnosis and monitoring of its course].
- Author
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Rameev VV, Kozlovskaia LV, Malinina EA, Serova AG, Kogarko IN, Kogarko BS, and Liubimova NV
- Subjects
- Adolescent, Adult, Aged, Amyloidosis complications, Amyloidosis diagnosis, Arthritis, Rheumatoid complications, Arthritis, Rheumatoid diagnosis, Case-Control Studies, Enzyme-Linked Immunosorbent Assay, Female, Humans, Male, Middle Aged, Prognosis, Severity of Illness Index, Young Adult, Amyloidosis blood, Arthritis, Rheumatoid blood, Immunoglobulin Light Chains blood, Serum Amyloid A Protein analysis
- Abstract
Aim: To determine clinical significance of measuring blood levels of protein precursors of AA- and AL-amyloidosis - SAA and immunoglobulin free light chains (ILC), respectively., Material and Methods: SAA concentrations were studied with ELISA in 43 rheumatoid arthritis (RA) patients including complicated with reactive AA-amyloidosis (n = 31). Inflammation activity and its severity were studied (indices Li, richi, HAQ, DAS4). A modern quantitative nephelometric method Freelite estimated ILC levels in 31 patients with AL-amyloidosis., Results: Patients with RA complicated with AA-amyloidosis and free of it had a strong correlation between blood serum SAA concentration and activity of joint disease. Elevated SAA concentrations to 160 mg/l (normal 10 mg/l) were detected in many patients with clinical remission of the joint syndrome. Significal inhibition of AA-amyloidosis progression was seen only in SAA concentration drop under 60 mg/l. For AL-amyloidosis patients ILC fall by less than 3 normal value means a 6-time increase in chances of a favourable outcome., Conclusion: Monitoring of blood levels of proteins precursors of AA- and AL-amyloidosis is a key factor in prognosis of the disease and treatment efficacy.
- Published
- 2011
20. [Determination of urinary markers of proteolysis/fibrinolysis and fibroangiogenesis in the kidney in hypertensive patients].
- Author
-
Nanchikeeva ML, Kozlovskaia LV, Rameev VV, Fomin VV, and Bulanov NM
- Subjects
- Adolescent, Adult, Aged, Albuminuria etiology, Biomarkers urine, Collagen Type IV urine, Female, Glomerular Filtration Rate, Humans, Hypertension complications, Kidney Diseases etiology, Male, Middle Aged, Monitoring, Physiologic, Neovascularization, Pathologic etiology, Plasminogen Activator Inhibitor 1 urine, Transforming Growth Factor beta urine, Vascular Endothelial Growth Factor A urine, Young Adult, Albuminuria urine, Fibrinolysis, Hypertension physiopathology, Kidney Diseases urine, Neovascularization, Pathologic urine
- Abstract
Aim: To determine clinical significance of urinary biomarkers of proteolysis/fibrinolysis and fibroangiogenesis in essential hypertension (EH)., Material and Methods: Examination of the kidneys was made in 71 patients with EH degree 1-3. Renal function was assessed by 24-h albuminuria, calculated glomerular filtration rate (GFR) by Cockroft-Golt. Early signs of renal damage were microalbuminuria--MAU (diurnal albuminuria 30-300 mg/day), reduction of GFR (< 90 ml/min/1.73 m2). EH patients with hypercreatininemia and GFR under 60 ml/min/1.73m2 corresponding to stage III of chronic kidney disease were not included in the study. An additional nephropathy marker was an elevated index of resistance of interlobular renal arteries (RI > 0.65) as shown by dopplerometry. ELISA examined urinary biomarkers of intercellular and cell-matrix interactions in the kidney in EHpatients and healthy controls (n = 12)., Results: MAU was detected in 54 (76%) of 71 EH patients, elevated RI > 0.65--in 37 (52%) patients. Urinary biomarkers of proteolysis/fibrinolysis and fibroangiogenesis were higher in EH patients then in the controls. Urinary excretion of PAI-1, TGF-beta1, VEGF and collagen of type IV in EH patients with MAU was significantly higher than in patients with normoalbuminuria. A strong direct correlation between MAU and the rest above urinary biomarkers was found as well as between urinary excretion of collagen IV and RI. An inverse negative relationship was seen between RI and GFR., Conclusion: Renal impairment in EHpatients is a progressive disorder. Each stage of this process has its own clinicodiagnostic markers. Urinary biomarkers ofproteolysis/fibrinolysis and fibroangiogenesis in the kidney are informative for monitoring of early HNP.
- Published
- 2011
21. [New possibilities of the diagnosis and monitoring of the course of AL-amyloidosis].
- Author
-
Rameev VV, Kozlovskaia LV, Kogarko IN, and Kogarko BS
- Subjects
- Amyloidosis blood, Amyloidosis drug therapy, Amyloidosis epidemiology, Humans, Immunoglobulin kappa-Chains blood, Immunoglobulin lambda-Chains blood, Prognosis, Severity of Illness Index, Amyloidosis diagnosis, Immunoglobulin Light Chains blood
- Abstract
Aim: to define the clinical value of various concentrations of immunoglobulin light chains (ILCs) in patients with AL amyloidosis., Subjects and Methods: The content of free ILCs was studied by a nephelometric technique after their fixation in the blood of 31 patients with AL amyloidosis; monoclonal gammapathy was associated with the hyperproduction of monoclonal ILCs of lambda- and kappa-type in 14 and 17 patients, respectively. The obtained value was compared with the data of physical examination and laboratory and instrumental studies indicating lesions to target organs and with the course of the disease., Results: In patients with the good course of AL amyloidosis, the average level of free ILCs was 1.8 (range 0.77-3) times greater than the normal values (the range of ILCs of lambda and kappa-type was 20.24 to 67.4 and 20.14 to 81.38 mg/l, respectively); in those with the poor course, the excess of ILCs was 5.8 (range 3.7-13) times higher than the normal values (the range of lLCs of lambda and kappa-type was 54.32 to 286.7 and 117.06 to 2606.0 mg/l, respectively). The optimal range of diagnostic sensitivity (75%) and specificity (75%) in the estimation of prognosis was determined at the ILC levels that were three times greater (64.5 mg/l for kappa-ILCs and 80 mg/l for lambda-ILCs). Among the patients with a blood free ILC level of < 3 times more than the normal values, the good and poor courses of AL amyloidosis were noted in 13 and 4 cases, respectively., Conclusion: The determination of serum ILC concentration by the Freelite method may be used to diagnose AL amyloidosis and to specify the presence of appropriate organ dysfunction; this study over time makes it possible to monitor the course of the disease and to estimate its response to therapy.
- Published
- 2010
22. [Left ventricular noncompaction concurrent with bronchoectatic disease complicated by secondary AA-amyloidosis with renal involvement].
- Author
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Fomin VV, Rameev VV, Sedov VP, Severova MM, Svet AV, Miroshnichenko NG, and Kozlovskaia LV
- Subjects
- Humans, Amyloid Neuropathies complications, Bronchiectasis complications, Isolated Noncompaction of the Ventricular Myocardium complications
- Abstract
The paper describes a clinical case of congenital cardiomyopathy (left ventricular noncompaction) concurrent with secondary amyloidosis and renal involvement that develops at the outcome of long existing brochoectatic disease.
- Published
- 2010
23. [An AA-amyloidosis course in patients with rheumatoid arthritis].
- Author
-
Sarkisova IA, Rameev VV, Varshavskiĭ VA, Golitsyn EP, and Kozlovskaia LV
- Subjects
- Adult, Age of Onset, Aged, Amyloidosis metabolism, Arthritis, Rheumatoid metabolism, Biopsy, C-Reactive Protein metabolism, Female, Humans, Kidney pathology, Male, Middle Aged, Prevalence, Severity of Illness Index, Amyloidosis epidemiology, Arthritis, Rheumatoid epidemiology
- Abstract
Aim: To characterize renal amyloidosis in patients with rheumatoid arthritis and stages of amyloid nephropathy., Material and Methods: The trial covered 30 patients (6 males and 24 females) with documented rheumatoid arthritis (RA) complicated with secondary AA-amyloidosis. Amyloidosis diagnosis was confirmed in all the patients morphologically, the samples were studied with the peroxidase immunohistochemical method using specific monoclonal antibodies to SAA. Clinical manifestations of RA were assessed by the disease activity, functional impairment of the joints, x-ray alterations, extraarticular signs of RA, etc. All the patients were examined clinically, total blood count and biochemical tests were made., Results: In 23 (77%) of 30 examinees with RA, proteinuria as the first clinical symptom of AA-amyloidosis emerged with the first 15 years of RA. RA of the second-third degree of activity were diagnosed in 25 (83%) patients, 21 (70%) patients had apparent destructive changes in the joints (x-ray stage III-IV). Severe functional insufficiency of the joints was observed in 25 (83%) patients, deformation of the joints - in 27 (90%) patients. Clinically, renal amyloidosis was characterized by change of stages - from moderate proteinuria to nephrotic syndrome and renal failure. Prognosis of amyloid nephropathy in RA depends on duration of the proteinuric stage: if this stage is short (3 years maximum), the prognosis is worse than in its long duration., Conclusion: RA ranks first among causes of secondary AA-amyloidosis. Development of AA-amyloidosis in RA patients is most probable in the first 15 years of the course of the articular process. Amyloidosis is more frequent in patients with severe clinical manifestations of RA.
- Published
- 2006
24. [The role of endothelial dysfunction in progression of chronic glomerulonephritis, current tools of its correction].
- Author
-
Bobkova IN, Chebotareva NV, Rameev VV, Plieva OK, and Kozlovskaia LV
- Subjects
- Chronic Disease, Disease Progression, Humans, Endothelium, Vascular physiopathology, Glomerulonephritis drug therapy, Glomerulonephritis physiopathology
- Published
- 2005
25. [Amyloidosis in the elderly].
- Author
-
Kozlovskaia LV, Rameev VV, and Sarkisova I
- Subjects
- Age Factors, Aged, Aged, 80 and over, Global Health, Humans, Incidence, Risk Factors, Amyloidosis diagnosis, Amyloidosis epidemiology, Amyloidosis etiology
- Published
- 2005
26. [The key role of tubulointerstitium remodeling in progression of chronic renal diseases].
- Author
-
Mukhin NA, Kozlovskaia LV, Bobkova IN, Rameev VV, Chebotareva NB, Plieva OK, Shcherbak AV, Varshavskiĭ VA, and Golitsina EP
- Subjects
- Amyloidosis drug therapy, Amyloidosis pathology, Chemokines metabolism, Fibronectins metabolism, Fibronectins urine, Humans, Kidney Failure, Chronic drug therapy, Nephritis, Interstitial drug therapy, Plasminogen Activators metabolism, Proteinuria pathology, Tetrazoles pharmacology, Valine pharmacology, Valsartan, Kidney Failure, Chronic pathology, Kidney Tubules pathology, NF-kappa B metabolism, Nephritis, Interstitial pathology, Valine analogs & derivatives
- Abstract
Tubulointerstitial fibrosis (TIF) is thought now to be a key factor in progression of renal failure in chronic nephropathies. A similar pattern of changes in glomerulonephritis and amyloidosis suggests common mechanisms operating in progression of renal failure in these nephropathies. Of importance in the process of interstitial inflammation is activation of the nuclear factor of transcription (NFkB) in tubular cells due to components of proteinuria and their secretion of some proinflammatory mediators, first of all chemokines with formation of inflammatory infiltrate and accumulation of interstitial myofibroblasts--the main source of extracellular matrix (ECM) components. Our findings are of interest in the light of current ideas that among ECM components the number of fibronectin deposites most of all reflects the severity of structural renal tissue damage including TIF and correlates with severity of renal failure.
- Published
- 2004
27. [Improved prognosis of generalized AL-amyloidosis in a patient with multiple myeloma treated according to the VAD protocol].
- Author
-
Sarkisova IA, Rameev VV, Kozlovskaia LV, and Andreeva NE
- Subjects
- Amyloidosis complications, Antineoplastic Combined Chemotherapy Protocols administration & dosage, Dexamethasone administration & dosage, Doxorubicin administration & dosage, Humans, Male, Middle Aged, Multiple Myeloma complications, Treatment Outcome, Vincristine administration & dosage, Amyloidosis drug therapy, Antineoplastic Combined Chemotherapy Protocols therapeutic use, Dexamethasone therapeutic use, Doxorubicin therapeutic use, Multiple Myeloma drug therapy, Vincristine therapeutic use
- Published
- 2003
28. Potentiated antibodies to tumor necrosis factor-alpha in the therapy of patients with rheumatoid arthritis.
- Author
-
Kozlovskaya LV, Mukhin NA, Rameev VV, Sarkisova IA, and Epstein OI
- Subjects
- Adult, Aged, Antibodies, Monoclonal immunology, Antibodies, Monoclonal therapeutic use, Arthritis, Rheumatoid metabolism, Arthritis, Rheumatoid pathology, Female, Humans, Male, Middle Aged, Treatment Outcome, Antibodies therapeutic use, Antirheumatic Agents therapeutic use, Arthritis, Rheumatoid drug therapy, Tumor Necrosis Factor-alpha immunology
- Abstract
We studied the efficiency and safety of a new homeopathic preparation Artrofoon containing affinely purified antibodies to tumor necrosis factor-alpha in the therapy of patients with rheumatoid arthritis. Artrofoon produced a positive antiinflammatory effect on the course of rheumatoid arthritis. This preparation reduced the severity of arthralgia (indexes of Li and Ritchie) and morning stiffness and decreased the erythrocyte sedimentation rate and contents of rheumatoid factor and C-reactive protein. One-month therapy improved the state of patients. Artrofoon was well tolerable. The preparation did not cause the ulcerogenic and nephrotoxic effects. Artrofoon holds much promise for combination therapy of patients with rheumatoid arthritis (including severe articular-and-visceral forms) and complications after treatment with nonsteroid antiinflammatory preparations.
- Published
- 2003
- Full Text
- View/download PDF
29. [Genetic aspects of periodic diseases and associated amyloidosis].
- Author
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Rameev VV, Kozlovskaia LV, Sarkisova IA, and Simonian AKh
- Subjects
- Amyloidosis diagnosis, Amyloidosis drug therapy, Colchicine therapeutic use, Cytoskeletal Proteins, Familial Mediterranean Fever diagnosis, Familial Mediterranean Fever drug therapy, Humans, Proteins genetics, Pyrin, Serum Amyloid A Protein analysis, Amyloidosis genetics, Familial Mediterranean Fever genetics
- Published
- 2002
30. [Systemic AL-amyloidosis with extensive pulmonary and vascular lesions].
- Author
-
Kozlovskaia LV, Rameev VV, Proskurneva EP, Osipenko VI, Sarkisova IA, and Onoprienko AA
- Subjects
- Amyloidosis complications, Female, Humans, Lung Diseases complications, Middle Aged, Pneumothorax etiology, Amyloidosis pathology, Lung Diseases pathology, Vascular Diseases pathology
- Abstract
Here is an observation data on systemic AL-amiloidosis with primary lesion of lungs and vessels (a false hemorrhagic syndrome, ischemic insult in the region of middle cerebral artery). At the same time lesions of kidneys and heart became of minor significance. Amiloid lesion of lungs was characterized by unusual combination of symptoms: a galloping hydrothorax, a vast focus in the consolidation of pulmonary tissue with focuses of calcification, relapsing pneumothorax.
- Published
- 2002
31. [AA-amyloidosis in Muckle-Wells syndrome].
- Author
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Kozlovskaia LV, Rameev VV, Ianushkevich TN, Proskurneva EP, Konovalov DV, Simonian AKh, and Sarkisova IA
- Subjects
- Adult, Female, Humans, Male, Nephrotic Syndrome genetics, Pedigree, Syndrome, Adhesins, Bacterial analysis, Amyloidosis genetics, Deafness genetics, Escherichia coli Proteins analysis, Urticaria genetics
- Published
- 2002
32. [Effective long-term use of colchicine in secondary AA-amyloidosis].
- Author
-
Rameev VV, Chegaeva TV, and Proskurneva EP
- Subjects
- Adenocarcinoma, Follicular complications, Adenocarcinoma, Follicular diagnosis, Amyloidosis diagnosis, Amyloidosis etiology, Amyloidosis metabolism, Female, Follow-Up Studies, Humans, Middle Aged, Retrospective Studies, Rheumatic Heart Disease complications, Rheumatic Heart Disease diagnosis, Thyroid Neoplasms complications, Tuberculosis, Pulmonary complications, Tuberculosis, Pulmonary diagnosis, Amyloidosis drug therapy, Colchicine therapeutic use, Gout Suppressants therapeutic use, Serum Amyloid A Protein metabolism
- Published
- 1999
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