scholarly journals Kidney involvement in monoclonal gammopathies: multidisciplinary approach in oncohematology and nephrology

2020 ◽  
Vol 15 (2) ◽  
pp. 49-60
Author(s):  
A. V. Smirnov ◽  
V. A. Dobronravov ◽  
M. S. Khrabrova ◽  
B. V. Afanasyev
Keyword(s):  
Russian Federation ◽  
Monoclonal Gammopathy ◽  
Specific Treatment ◽  
Health Care Facilities ◽  
International Consensus ◽  
Saint Petersburg ◽  
Consensus Document ◽  
Main Challenge ◽  
Kidney Involvement ◽  
Treatment Consensus

The pathogenetic relationship of kidney damage and an aberrant clone of the B-cell line producing nephrotoxic monoclonal immunoglobulin underlies the concept of “monoclonal gammopathy of renal significance” (MGRS). Herein the aberrant clone does not reach the criteria necessary for initiating antitumor therapy according to oncohematological indications. MGRS is a new nosology in modern nephrology and oncohematology. Monoclonal protein’s 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: i) limited knowledge about the MGRS among hematologists and nephrologists; ii) lack of necessary diagnostic resources in most health-care facilities; iii) lack of approved clinical recommendations and medical economic standards for treatment of this pathological entity. In order to overcome these limitations, leading oncohematologists and nephrologists of the Russian Federation on behalf of professional communities at the end of 2019 published a conciliation document: “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”. 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”, 15–16 of March 2019, Pavlov First Saint Petersburg State Medical University, Saint Petersburg, Russia. Consensus is intended to define the principal practical steps to resolve the problem of MGRS in Russian Federation that are summarized as final clauses which we present here.

scholarly journals 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

2020 ◽  
Vol 92 (7) ◽  
pp. 10-22
Author(s):  
A. V. Smirnov ◽  
B. V. Afanasyev ◽  
I. V. Poddubnaya ◽  
V. A. Dobronravov ◽  
M. S. Khrabrova ◽  
...  
Keyword(s):  
Russian Federation ◽  
Monoclonal Gammopathy ◽  
Treatment Initiation ◽  
Specific Treatment ◽  
Kidney Injury ◽  
Great Majority ◽  
Health Care Facilities ◽  
International Consensus ◽  
Consensus Document ◽  
Main Challenge

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.

scholarly journals Monoclonal gammopathy of renal signifi cance: Consensus of hematologists and nephrologists of Russia on the establishment of nosology, diagnostic approach and rationale for clone specific treatment

2019 ◽  
Vol 23 (6) ◽  
pp. 9-28 ◽  
Author(s):  
A. V. Smirnov ◽  
B. V. Afanasyev ◽  
I. V. Poddubnaya ◽  
V. A. Dobronravov ◽  
M. S. Khrabrova ◽  
...  
Keyword(s):  
Russian Federation ◽  
Monoclonal Gammopathy ◽  
Treatment Initiation ◽  
Specific Treatment ◽  
Kidney Injury ◽  
Great Majority ◽  
Health Care Facilities ◽  
International Consensus ◽  
Consensus Document ◽  
Main Challenge

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 protein’s 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: i) limited knowledge about the MGRS among hematologists and nephrologists; ii) lack of necessary diagnostic resources in most health-care facilities; iii) 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», 15-16 of March 2019, Pavlov First St-Petersburg State Medical University, St-Petersburg, Russia. 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.

scholarly journals 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

2020 ◽  
Vol 10 (2) ◽  
pp. 102-118
Author(s):  
A. V. Smirnov ◽  
B. V. Afanasyev ◽  
I. V. Poddubnaya ◽  
V. A. Dobronravov ◽  
M. S. Khrabrova ◽  
...  
Keyword(s):  
Russian Federation ◽  
Monoclonal Gammopathy ◽  
Treatment Initiation ◽  
Specific Treatment ◽  
Kidney Injury ◽  
Great Majority ◽  
International Consensus ◽  
Healthcare Facilities ◽  
Consensus Document ◽  
Main Challenge

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 protein’s 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: i) limited knowledge about the MGRS among hematologists and nephrologists; ii) lack of necessary diagnostic resources in most healthcare facilities; iii) 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», 15-16 of March 2019, Pavlov First St-Petersburg State Medical University, St-Petersburg, Russia. 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.

scholarly journals 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

2020 ◽  
Vol 10 (2) ◽  
pp. 102-118
Author(s):  
A. V. Smirnov ◽  
B. V. Afanasyev ◽  
I. V. Poddubnaya ◽  
V. A. Dobronravov ◽  
M. S. Khrabrova ◽  
...  
Keyword(s):  
Russian Federation ◽  
Monoclonal Gammopathy ◽  
Treatment Initiation ◽  
Specific Treatment ◽  
Kidney Injury ◽  
Great Majority ◽  
International Consensus ◽  
Healthcare Facilities ◽  
Consensus Document ◽  
Main Challenge

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 protein’s 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: i) limited knowledge about the MGRS among hematologists and nephrologists; ii) lack of necessary diagnostic resources in most healthcare facilities; iii) 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», 15-16 of March 2019, Pavlov First St-Petersburg State Medical University, St-Petersburg, Russia. 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.

scholarly journals Nephropathic cystinosis: an international consensus document

2014 ◽  
Vol 29 (suppl 4) ◽  
pp. iv87-iv94 ◽  
Author(s):  
F. Emma ◽  
G. Nesterova ◽  
C. Langman ◽  
A. Labbe ◽  
S. Cherqui ◽  
...  
Keyword(s):  
Nephropathic Cystinosis ◽  
International Consensus ◽  
Consensus Document

The image of the Russian Federation on the canvas of social research

2020 ◽  
Vol 18 (3) ◽  
pp. 115-140
Author(s):  
Natalia Sienko
Keyword(s):  
Russian Federation ◽  
Social Research ◽  
Saint Petersburg ◽  
Starting Point ◽  
The Russian Federation ◽  
Principal Analysis ◽  
Depth Interviews

The starting point for this article was research conducted from February to April 2017 and in January and February 2020. The purpose of the work is to show the image and position of the Russian Federation in the eyes of respondents who participated (incl. residents of Moscow, Arkhangelsk, Saint Petersburg and Vladivostok) in in-depth interviews conducted by the author. The principal analysis concerns the results obtained in 2017, and the specific changes observed three years after the initial surveys were completed.

scholarly journals An Unusual Case of Acquired Angioedema and Monoclonal Gammopathy of Renal Significance in a Middle-Aged Caucasian Female

2020 ◽  
Vol 8 ◽  
pp. 232470962091209 ◽  
Author(s):  
Sasmit Roy ◽  
Venu Madhav Konala ◽  
Thurein Kyaw ◽  
Sandipan Chakraborty ◽  
Srikanth Naramala ◽  
...  
Keyword(s):  
Acute Kidney Injury ◽  
B Cell ◽  
Monoclonal Gammopathy ◽  
Cell Clone ◽  
Kidney Injury ◽  
Caucasian Woman ◽  
Acquired Angioedema ◽  
Systemic Involvement ◽  
Kidney Involvement ◽  
Esterase Inhibitor

Acquired angioedema due to deficiency of C1 esterase inhibitor is also called acquired angioedema and is abbreviated as C1INH-AAE. It is a rare syndrome of recurrent episodes of angioedema, without urticaria, and in some patients, it is associated with B-cell lymphoproliferative disorders. Kidney involvement is rare in this condition. The monoclonal immunoglobulin secreted by a nonmalignant or premalignant B-cell or plasma cell clone, causing renal damage that represents a group of disorders which are termed as monoclonal gammopathy of renal significance (MGRS). In this article, we report a rare case of acquired C1 esterase deficiency angioedema and acute kidney injury with renal biopsy-proven MGRS. We present a 64-year-old Caucasian woman who presented with 2 weeks of recurring urticaria and new onset of acute kidney injury. She was diagnosed with monoclonal gammopathy–associated proliferative glomerulopathy through kidney biopsy, and serological workup came back positive for C1 esterase deficiency, implying acquired angioedema. Acquired angioedema is a rare disease with systemic involvement. Recurrent allergic manifestations and acute kidney injury should prompt MGRS as a differential.

scholarly journals European Heart Rhythm Association (EHRA) international consensus document on how to prevent, diagnose, and treat cardiac implantable electronic device infections—endorsed by the Heart Rhythm Society (HRS), the Asia Pacific Heart Rhythm Society (APHRS), the Latin American Heart Rhythm Society (LAHRS), International Society for Cardiovascular Infectious Diseases (ISCVID) and the European Society of Clinical Microbiology and Infectious Diseases (ESCMID) in collaboration with the European Association for Cardio-Thoracic Surgery (EACTS)

EP Europace ◽  
2019 ◽  
Vol 22 (4) ◽  
pp. 515-549 ◽  
Author(s):  
Carina Blomström-Lundqvist ◽  
Vassil Traykov ◽  
Paola Anna Erba ◽  
Haran Burri ◽  
Jens Cosedis Nielsen ◽  
...  
Keyword(s):  
Infectious Diseases ◽  
Latin American ◽  
Electronic Device ◽  
Heart Rhythm ◽  
Asia Pacific ◽  
Cardiac Resynchronization ◽  
Cardiac Implantable Electronic Device ◽  
International Consensus ◽  
Consensus Document ◽  
Cardiac Resynchronization Therapy Devices

Abstract Pacemakers, implantable cardiac defibrillators, and cardiac resynchronization therapy devices are potentially life-saving treatments for a number of cardiac conditions, but are not without risk. Most concerning is the risk of a cardiac implantable electronic device (CIED) infection, which is associated with significant morbidity, increased hospitalizations, reduced survival, and increased healthcare costs. Recommended preventive strategies such as administration of intravenous antibiotics before implantation are well recognized. Uncertainties have remained about the role of various preventive, diagnostic, and treatment measures such as skin antiseptics, pocket antibiotic solutions, anti-bacterial envelopes, prolonged antibiotics post-implantation, and others. Guidance on whether to use novel device alternatives expected to be less prone to infections and novel oral anticoagulants is also limited, as are definitions on minimum quality requirements for centres and operators and volumes. Moreover, an international consensus document on management of CIED infections is lacking. The recognition of these issues, the dissemination of results from important randomized trials focusing on prevention of CIED infections, and observed divergences in managing device-related infections as found in an European Heart Rhythm Association worldwide survey, provided a strong incentive for a 2019 International State-of-the-art Consensus document on risk assessment, prevention, diagnosis, and treatment of CIED infections.

scholarly journals Kidney Involvement in Patients with Type 1 Autoimmune Pancreatitis

2019 ◽  
Vol 8 (2) ◽  
pp. 258
Author(s):  
Miroslav Vujasinovic ◽  
Raffaella Pozzi Mucelli ◽  
Roberto Valente ◽  
Caroline Verbeke ◽  
Stephan Haas ◽  
...  
Keyword(s):  
Autoimmune Pancreatitis ◽  
Lymphocytic Infiltration ◽  
Normal Serum ◽  
International Consensus ◽  
Kidney Involvement ◽  
Type 1 Autoimmune Pancreatitis ◽  
Elevated Creatinine ◽  
Imaging Signs

Introduction: Autoimmune pancreatitis (AIP) type 1 is a special form of chronic pancreatitis with a strong lymphocytic infiltration as the pathological hallmark and other organ involvement (OOI). IgG4-related kidney disease (IgG4-RKD) was first reported as an extrapancreatic manifestation of AIP in 2004. The aim of the present study was to determine the frequency and clinical impact of kidney lesions observed in patients with AIP type 1. Methods: We performed a single-centre retrospective study on a prospectively collected cohort of patients with a histologically proven or highly probable diagnosis of AIP according to the International Consensus Diagnostic Criteria (ICDC) classification. Results: Seventy-one patients with AIP were evaluated. AIP type 1 was diagnosed in 62 (87%) patients. Kidney involvement was present in 17 (27.4%) patients with AIP type 1: 15 (88.2%) males and 2 (11.8%) females. Laboratory and/or imaging signs of kidney involvement were presented at the time of AIP diagnosis in eight (47.1%) patients. In other patients, the onset of kidney involvement occurred between four months and eight years following diagnosis. At the time of the diagnosis of kidney involvement, eight (47.1%) patients showed elevated creatinine, and nine (52.9%) patients showed normal serum creatinine. None of the patients were treated with dialysis. Conclusions: IgG4-RKD was present in 27.4% of patients with AIP type 1, with male gender predominance. In cases of early diagnosis and cortisone treatment, the clinical course was mild in most cases. Regular laboratory control of renal function should be a part of the follow-up of patients with AIP type 1.

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