Primary immunodeficiencies with predominant impairment of antibody synthesis

2021 ◽  
Vol 90 (4) ◽  
pp. 24-32
Author(s):  
M.V. Vasilyeva ◽  
◽  
I.A. Sai ◽  
Keyword(s):  
Diagnostic Criteria ◽  
Chronic Diseases ◽  
High Mortality ◽  
Allergic Diseases ◽  
Late Diagnosis ◽  
Primary Immunodeficiencies ◽  
Immunocompetent Cells ◽  
Antibody Synthesis ◽  
New Concepts

The urgency of the problem of primary immunodeficiencies (PID) lies in the late diagnosis of this pathology due to the low awareness of doctors of various specialties, the formation of chronic diseases in patients, and high mortality in this group of patients. Currently, the concept of PID includes both traditional concepts - defects leading to the development of quantitative and / or functional insufficiency, and new concepts - uncontrolled activation of the proliferation of immunocompetent cells and the formation of autoimmune, auto-inflammatory and allergic diseases. The article discusses the diagnostic criteria for PID and the main directions in the treatment of this contingent of patients

scholarly journals Update of the Diagnostic Criteria of J-Wave Syndrome: New Concepts and Their Relevance to Cardiology Practice (According to Materials of J-Wave Syndromes Expert Consensus Conference Report: Emerging Concepts and Gaps in Knowledge (2016)

Kardiologiia ◽  
2018 ◽  
Vol 58 (11) ◽  
pp. 41-52 ◽  
Author(s):  
Yu. N. Belenkov ◽  
V. A. Snezhitskiy ◽  
T. P. Gizatulina ◽  
N. V. Shpak ◽  
V. A. Kuznetsov ◽  
...  
Keyword(s):  
Diagnostic Criteria ◽  
Conference Report ◽  
Consensus Conference ◽  
Expert Consensus ◽  
Early Repolarization ◽  
New Concepts ◽  
Comparative Aspect ◽  
Criteria For Diagnosis ◽  
New Criteria ◽  
J Wave

This review includes main positions of the revision of diagnostic criteria of “J-wave syndromes in the J-Wave Syndromes Expert Consensus Conference Report: Emerging Concepts and Gaps in Knowledge” (2016). The article, systematized according to the sections of the above-mentioned document, outlines the questions of terminology, new criteria for diagnosis of the Brugada syndrome (BrS) and early repolarization syndrome (ERS). The section devoted to ERS on the issues of new terminology and standardization of measurements, is supplemented with material from the Consensus Paper - The Early Repolarization Pattern (2015). The article also presents the issues of differential diagnosis in BrS, presents modulating factors, defines acquired Brugada-pattern and Brugada phenocopies. The similarities and differences between BrS and ERS are presented in a comparative aspect.

scholarly journals The Lung in Primary Immunodeficiencies: New Concepts in Infection and Inflammation

2018 ◽  
Vol 9 ◽  
Author(s):  
Ulrich Baumann ◽  
John M. Routes ◽  
Pere Soler-Palacín ◽  
Stephen Jolles
Keyword(s):  
Primary Immunodeficiencies ◽  
New Concepts ◽  
Infection And Inflammation

Fournier's gangrene: Report on two cases

1997 ◽  
Vol 64 (2) ◽  
pp. 266-268
Author(s):  
F. Laganà ◽  
E. Cossaro ◽  
C. Ronconi ◽  
F. Sercia ◽  
M. Marchini
Keyword(s):  
Mortality Rate ◽  
Chronic Diseases ◽  
High Mortality ◽  
Hyperbaric Oxygenation ◽  
Fournier’S Gangrene ◽  
Fournier's Gangrene ◽  
Tissue Ischemia ◽  
First Choice

– Fournier's gangrene is a rare pathology that generally occurs in weakened patients with chronic diseases, like diabetes, which imply tissue ischemia and even today a high mortality rate. Two cases are presented, one of which is interesting for its pathogenesis. The authors confirm that first choice treatment should be surgical associated with antiobiotics and hyperbaric oxygenation whenever possible.

scholarly journals IgE-Related Chronic Diseases and Anti-IgE-Based Treatments

2016 ◽  
Vol 2016 ◽  
pp. 1-12 ◽  
Author(s):  
Arnau Navinés-Ferrer ◽  
Eva Serrano-Candelas ◽  
Gustavo-J Molina-Molina ◽  
Margarita Martín
Keyword(s):  
Allergic Asthma ◽  
Chronic Diseases ◽  
Chronic Urticaria ◽  
Inhaled Corticosteroids ◽  
Systemic Mastocytosis ◽  
Airway Remodeling ◽  
Clinical Manifestations ◽  
Allergic Diseases ◽  
Severe Allergic Asthma ◽  
Prophylactic Use

IgE is an immunoglobulin that plays a central role in acute allergic reactions and chronic inflammatory allergic diseases. The development of a drug able to neutralize this antibody represents a breakthrough in the treatment of inflammatory pathologies with a probable allergic basis. This review focuses on IgE-related chronic diseases, such as allergic asthma and chronic urticaria (CU), and on the role of the anti-IgE monoclonal antibody, omalizumab, in their treatment. We also assess the off-label use of omalizumab for other pathologies associated with IgE and report the latest findings concerning this drug and other new related drugs. To date, omalizumab has only been approved for severe allergic asthma and unresponsive chronic urticaria treatments. In allergic asthma, omalizumab has demonstrated its efficacy in reducing the dose of inhaled corticosteroids required by patients, decreasing the number of asthma exacerbations, and limiting the effect on airway remodeling. In CU, omalizumab treatment rapidly improves symptoms and in some cases achieves complete disease remission. In systemic mastocytosis, omalizumab also improves symptoms and its prophylactic use to prevent anaphylactic reactions has also been discussed. In other pathologies such as atopic dermatitis, food allergy, allergic rhinitis, nasal polyposis, and keratoconjunctivitis, omalizumab significantly improves clinical manifestations. Omalizumab acts in two ways: by sequestering free IgE and by accelerating the dissociation of the IgE-Fcεreceptor I complex.

Diagnostic Criteria for Primary Immunodeficiencies

1999 ◽  
Vol 93 (3) ◽  
pp. 190-197 ◽  
Author(s):  
Mary Ellen Conley ◽  
Luigi D. Notarangelo ◽  
Amos Etzioni
Keyword(s):  
Diagnostic Criteria ◽  
Primary Immunodeficiencies

scholarly journals Aggressive course of bronchial cancer

2020 ◽  
pp. 119-122
Author(s):  
A. V. Pronevich ◽  
P. N. Kovalchuk
Keyword(s):  
Mortality Rate ◽  
High Mortality ◽  
Clinical Case ◽  
Late Diagnosis ◽  
Bronchial Cancer

The article describes a clinical case of bronchial cancer without radiologic changes. This is often associated with late diagnosis of the disease and a high mortality rate of this pathology.

scholarly journals ROLE OF MACROPHAGES IN IMMUNOLOGICAL MATURATION

1968 ◽  
Vol 128 (3) ◽  
pp. 459-467 ◽  
Author(s):  
Bertie F. Argyris
Keyword(s):  
Antigen Processing ◽  
Processing System ◽  
Peritoneal Macrophages ◽  
Immunocompetent Cells ◽  
Critical Ratio ◽  
Spleen Cells ◽  
Antibody Synthesis ◽  
Newborn Mice ◽  
C3h Mice ◽  
High Doses

1. Transplantation of peritoneal macrophages from thioglycollate-stimulated adult C3H donor mice, into 3-day-old C3H mice results in an enhanced antibody response to simultaneously injected SRBC. The increase in immuno-competence is even more pronounced when 1-day-old C3H mice are pretreated with adult macrophages and sensitized 3 days later with SRBC. Nonviable macrophages or nonviable spleen cells are ineffective. 2. There is a critical period in the development of the neonatal mouse during which the spleen cells benefit from the addition of adult macrophages. Treatment before or beyond this stage is ineffective. 3. Very high doses (20 million) of macrophages are less effective in stimulating antibody synthesis to SRBC than doses of 5 or 10 million, suggesting that a critical ratio of macrophages to immunocompetent cells may be required for enhancing antibody synthesis in young mice. 4. The results are discussed in the light of the hypothesis that newborn mice are immunologically deficient not because they lack immunocompetent cells but because they lack an antigen recognition or antigen-processing system in the form of functional macrophages.

scholarly journals On the Criteria of Activity-Based Education

2019 ◽  
Vol 15 (3) ◽  
pp. 105-116 ◽  
Author(s):  
G.A. Zuckerman ◽  
T.M. Bilibina ◽  
O.M. Vinogradova ◽  
O.L. Obukhova ◽  
N.A. Shibanova
Keyword(s):  
Diagnostic Criteria ◽  
Subject Matter ◽  
Personal Meaning ◽  
Learning Activity ◽  
Education Student ◽  
Children’S Learning ◽  
New Concepts ◽  
The Subject ◽  
Expressed Emotions ◽  
Children's Learning

The goal of this article is to discuss diagnostic criteria that are directly observable and at the same time allow to judge with sufficient reliability whether learning is activity-based not only in its project, but also in its implementation - in a cooperative child-adult action. Only one criterion is proposed here: children's initiative aimed at discovering and appropriating new concepts. In an attempt to elucidate the blurred meaning of such terms as “activity-based education”, “student as an agency (initiator) of learning activity” the authors suggest revising the subject matter behind these words, and for this purpose to visit the lessons and highlight the events that manifest children's learning initiative. The evidence from the literacy lessons taught according to the Elkonin’s ABC primer shows how the guesses and questions by individual students reveal to the observer that the students relate the concept mastered here and now and the concepts that belong to the same system but have not been discussed as yet in the classroom. Such transcending beyond the scope of the task as initially set by the teacher, discloses children's efforts to construct the concepts that outstrip the teacher's plan by several hours, weeks, and even months. As a rule, such initiatives are accompanied by expressed emotions of joy, surprise, pleasure, inspiration, interest, signifying their personal meaning for children.

scholarly journals Polyspecific antibodies without persisting antigen in multiple sclerosis, neurolupus and Guillain-Barré syndrome: immune network connectivity in chronic diseases

2017 ◽  
Vol 75 (8) ◽  
pp. 580-588 ◽  
Author(s):  
Hansotto Reiber
Keyword(s):  
Multiple Sclerosis ◽  
Chronic Diseases ◽  
Network Connectivity ◽  
Guillain Barre Syndrome ◽  
Immune Network ◽  
Continuous Change ◽  
Antibody Synthesis ◽  
Varicella Zoster ◽  
Guillain Barré Syndrome ◽  
Guillain Barré

ABSTRACT The polyspecific antibody synthesis in multiple sclerosis (MS) gained diagnostic relevance with the frequent combination of measles-, rubella- and varicella zoster antibodies (MRZ-antibody reaction) but their pathophysiological role remains unknown. This review connects the data for intrathecal polyspecific antibody synthesis in MS and neurolupus with observations in the blood of patients with Guillain-Barré syndrome (GBS). Simultaneously increased antibody and autoantibody titers in GBS blood samples indicate that the polyspecific antibodies are based on a general property of an immune network, supported by the deterministic day-to-day concentration variation of antibodies in normal blood. Strongly correlated measles- and rubella- antibody variations point to a particular connectivity between the MRZ antibodies. The immune network, which provides serological memory in the absence of an antigen, implements the continuous change of the MRZ pattern in blood, not followed by the earlier immigrated B cells without corresponding connectivity in the brain. This may explain the different antibody patterns in cerebrospinal fluid, aqueous humor and blood of the individual MS patient. A complexity approach must implement a different view on causation in chronic diseases and causal therapies.

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