scholarly journals New Insights into Immunological Involvement in Congenital Disorders of Glycosylation (CDG) from a People-Centric Approach

2020 ◽  
Vol 9 (7) ◽  
pp. 2092
Author(s):  
Rita Francisco ◽  
Carlota Pascoal ◽  
Dorinda Marques-da-Silva ◽  
Sandra Brasil ◽  
Fernando M. Pimentel-Santos ◽  
...  
Keyword(s):  
Adverse Reactions ◽  
Food Allergies ◽  
Congenital Disorders ◽  
Healthy Population ◽  
Congenital Disorders Of Glycosylation ◽  
Electronic Questionnaires ◽  
Healthy Participants ◽  
Severe Adverse Reactions ◽  
Allergy Prevalence

Congenital disorders of glycosylation (CDG) are rare diseases with variable phenotypes and severity. Immunological involvement remains a largely uncharted topic in CDG, mainly due to lack of robust data. To better characterize immune-related manifestations’ prevalence, relevance, and quality-of-life (QoL) impact, we developed electronic questionnaires targeting (1) CDG patients and (2) the general “healthy” population. Two-hundred and nine CDG patients/caregivers and 349 healthy participants were included in this study. PMM2-CDG was the most represented CDG (n = 122/209). About half of these participants (n = 65/122) described relevant infections with a noteworthy prevalence of those affecting the gastrointestinal tract (GI) (63.1%, n = 41/65). Infection burden and QoL impact were shown as infections correlated with more severe clinical phenotypes and with a set of relevant non-immune PMM2-CDG signs. Autoimmune diseases had only a marginal presence in PMM2-CDG (2.5%, n = 3/122), all being GI-related. Allergy prevalence was also low in PMM2-CDG (33%, n = 41/122) except for food allergies (26.8%, n = 11/41, of PMM2-CDG and 10.8%, n = 17/158, of controls). High vaccination compliance with greater perceived ineffectiveness (28.3%, n = 17/60) and more severe adverse reactions were described in PMM2-CDG. This people-centric approach not only confirmed literature findings, but created new insights into immunological involvement in CDG, namely by highlighting the possible link between the immune and GI systems in PMM2-CDG. Finally, our results emphasized the importance of patient/caregiver knowledge and raised several red flags about immunological management.

scholarly journals Food Allergies: Current and Future Treatments

Medicina ◽  
2019 ◽  
Vol 55 (5) ◽  
pp. 120 ◽  
Author(s):  
Amelia Licari ◽  
Sara Manti ◽  
Alessia Marseglia ◽  
Ilaria Brambilla ◽  
Martina Votto ◽  
...  
Keyword(s):  
Food Allergy ◽  
Adverse Reactions ◽  
Public Health Problem ◽  
Food Allergies ◽  
Dietary Restrictions ◽  
Related Risk ◽  
Promising Therapeutic Approach ◽  
Significant Burden ◽  
Allergy Treatment

Food allergies are an increasingly public health problem, affecting up to 10% of children and causing a significant burden on affected patients, resulting in dietary restrictions, fear of accidental ingestion and related risk of severe reactions, as well as a reduced quality of life. Currently, there is no specific cure for a food allergy, so the only available management is limited to strict dietary avoidance, education on prompt recognition of symptoms, and emergency treatment of adverse reactions. Several allergen specific- and nonspecific-therapies, aiming to acquire a persistent food tolerance, are under investigation as potential treatments; however, to date, only immunotherapy has been identified as the most promising therapeutic approach for food allergy treatment. The aim of this review is to provide an updated overview on changes in the treatment landscape for food allergies.

scholarly journals A study on self-reported food allergy in young adults from various colleges in Kerala, India

2019 ◽  
Vol 6 (10) ◽  
pp. 4290
Author(s):  
Renata Ann Dias ◽  
Jenyz M. Mundodan ◽  
Sruthi M. V.
Keyword(s):  
Young Adults ◽  
Food Allergy ◽  
Adverse Reactions ◽  
Cross Sectional Study ◽  
Food Allergies ◽  
Cross Sectional ◽  
Life Threatening ◽  
History Of ◽  
Food Fish

Background: Food allergy is defined as a reproducible adverse immune reaction to food proteins. Food allergies can result in life threatening reactions and diminish quality of life. In the last several decades, prevalence of food allergy has increased in several regions throughout the world.Methods: A cross sectional study was conducted among 321 young adults of the age 17 to 26 years from various colleges in Kerala. The participants were asked to fill out a pretested questionnaire. The data was collected and entered in MS Excel and analyzed.Results: Adverse reactions following food consumption was experienced by 82 respondents (25.5%). Out of these 82 respondents, 38 (46%) had their condition diagnosed by a doctor. 23 respondents had only one episode of such an attack. 41 respondents took medications for the attack. 14 respondents had been hospitalized at least once for such an attack. The most common symptoms following the consumption of food were as follows: vomiting, itching and eczema. The symptoms occur after the consumption of sea food/fish and eggs mostly. 27 respondents had a family history of food allergy.Conclusions: The symptoms of food allergy may vary from minor itching to even anaphylaxis. It is very important to know the causative allergen in the diet to prevent the occurrence of an episode of food allergy. 

Side Effects of the Therapy With Peginterferon and Ribavirin in Chronic Hepatitis C

2011 ◽  
Vol 25 (1) ◽  
pp. 85-88 ◽  
Author(s):  
José Barreto Cruz Nogueira ◽  
Laís Cristina Santana Sena ◽  
Jullyana de Souza Siqueira Quintans ◽  
Jackson Roberto Guedes da Silva Almeida ◽  
Alex Vianey Callado França ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Chronic Hepatitis ◽  
Hepatitis C ◽  
Chronic Hepatitis C ◽  
Adverse Reactions ◽  
Treatment Compliance ◽  
Potential Drug ◽  
Peginterferon And Ribavirin ◽  
Severe Adverse Reactions

This study sought to decribe, quantify, and classify any adverse reactions occurring in patients with chronic hepatitis C treated with peginterferon and ribavirin, as well as verify the occurrence of potential medication interactions. The most prevalent reactions were fatigue (84.8%), fever (83%), weight loss (80%), irritability (74%), and body pain (72%). Most of the reactions were classified as mild (95%), whereas 4.5% were classified as moderate and 0.4% as severe. Adverse reactions led to the rearrangement of therapy for 9 patients (20%), where there was a reduction in dose for 7 (15%), temporary interruption of treatment for 5 (11%), and permanent discontinuation for 3 patients (7%). A total of 11 potential medication interactions were identified in 9 patients (20%), with the most frequent between peginterferon-α2a and captopril (45%). Given the above, it is observed that the treatment of chronic hepatitis C is marked by several adverse reactions of varying severity, which can interfere with the patient’s quality of life or in treatment compliance, and that can be aggravated by potential drug–drug interactions.

Does Infliximab Short Infusion have a Beneficial Impact on the Quality of Life in Patients with Inflammatory Bowel Diseases? A Single Centre Prospective Evaluation

2015 ◽  
Vol 24 (2) ◽  
pp. 165-170 ◽  
Author(s):  
Mariabeatrice Principi ◽  
Giuseppe Losurdo ◽  
Rosa Federica La Fortezza ◽  
Pasquale Lopolito ◽  
Rosa Lovero ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Inflammatory Bowel Diseases ◽  
Adverse Reactions ◽  
Severe Disease ◽  
Sexual Life ◽  
Necrosis Factor Alpha ◽  
Short Infusion ◽  
Bowel Diseases ◽  
Inflammatory Bowel

Background & Aims: Infliximab (IFX) is an anti-tumor necrosis factor alpha agent used in inflammatory bowel diseases (IBD) therapy. Usually, it is administered over a 2-hour intravenous infusion. However, shortening the infusion duration to 1 hour has proved to be feasible and safe. In the present study we evaluated whether shortening the IFX infusion could affect the patients' quality of life (QoL) compared to the standard protocol.Methods: Subjects affected by IBD receiving IFX were prospectively recruited. The main criterion to shorten the infusion was the absence of IFX-related adverse reactions during the previous three 2-h infusions. For each patient, demographic, clinical and anthropometric data were collected. A questionnaire investigating their overall/job/social/sexual QoL was administered. Ordinal regression was performed with odds ratios (OR) for significant independent variables.Results: Eighty-one patients were included (46 with ulcerative colitis - UC, 35 with Crohn's disease - CD). Sixteen received the 2-h infusion due to previous adverse reactions, and the remaining 65 underwent the 1-h schedule. Shortening the infusion to 1 hour determined a better QoL (OR=0.626). However, the QoL was negatively influenced by age (OR=1.023), female sex (OR=2.04) and severe disease activity (OR=7.242). One-hour IFX infusion induced a better outcome on work (OR=0.588) and social (OR=0.643) QoL. Long-standing disease was correlated with a slightly better sexual QoL (OR=0.93). Conversely, older age (OR=1.046), severe clinical score (OR=15.579), use of other immunomodulators (OR=3.693) and perianal CD (OR=3.265) were related to an unsatisfactory sexual life. The total number of infusions (OR=0.891), proctitis (OR=0.062) or pancolitis (OR=0.1) minimized the perception of infusion-related side effects.Conclusion: The 1-h short infusion improves overall, social and job QoL, so that, when indicated, it should be recommended.

ОЦЕНКА ЭФФЕКТИВНОСТИ И БЕЗОПАСНОСТИ 10% ВНУТРИВЕННОГО ИММУНОГЛОБУЛИНА ПРИВИДЖЕН В РЕАЛЬНОЙ КЛИНИЧЕСКОЙ ПРАКТИКЕ

2019 ◽  
Author(s):  
L.G. Khludova ◽  
I.A. Manto ◽  
E.A. Latysheva ◽  
T.V. Latysheva ◽  
M.R. Khaitov
Keyword(s):  
Replacement Therapy ◽  
Primary Immunodeficiency ◽  
Adverse Reactions ◽  
Efficacy And Safety ◽  
High Efficacy ◽  
Antibody Synthesis ◽  
Human Immunoglobulins ◽  
Severe Adverse Reactions ◽  
Common Variable ◽  
Real Clinical Practice

Актуальность. Заместительная терапия иммуноглобулинами человека является ведущим патогенетическим методом лечения первичных иммунодефицитов с нарушением синтеза антител. В настоящее время в России доступно несколько препаратов иммуноглобулинов человека нормальных для внутривенного введения. Цель. Оценить эффективность и безопасность препарата Привиджен (10 раствор иммуноглобулина для внутривенного введения) в реальной клинической практике в течение 12 клинических месяцев. Материалы и методы. 20 взрослых с диагнозом общая вариабельная иммунная недостаточности и Х-сцепленная агаммаглобулинемия получали внутривенный иммуноглобулин Привиджен к интервалом 243 дня в течение 12 мес. Первичными критериями оценки была частота инфекционных осложнений и нежелательных явлений. Результаты. У большинства пациентов в ходе исследования достигнут удовлетворительный претранс-фузионный уровень IgG. Тяжелых нежелательных явлений, связанных с введением препарата, не зарегистрировано. Заключение. В ходе исследования препарат продемонстрировал высокую эффективность и безопасность у пациентов, нуждающихся в ежемесячной заместительной терапииRelevance. Replacement therapy with human immunoglobulins is the leading pathogenetic method of treatment of primary immunodeficiency with impaired antibody synthesis. Currently, several preparations of human immunoglobulins for intravenous administration are available in Russia. Purposes. Evaluation of the efficacy and safety of Privigen immunoglobulin intravenous 10 liquid in real clinical practice within 12 clinical months. Methods. Twenty adults diagnosed with common variable immunodeficiency or X-linked agammaglobulinemia received intravenous Privigen infusions (0.2-0.4 mg/kg) at 243 intervals over a 12-month period. The primary endpoint was the annual rate of infections and adverse events. Results. Sufficient level of IgG was achieved in most patients during the study. Severe adverse reactions during the treatment were not registered. Conclusions. High efficacy and safety of monthly replacement therapy in patients with primary immunodeficiency with impaired antibody synthesis has been demonstrated.

Insect Allergens on the Dining Table

2020 ◽  
Vol 21 (2) ◽  
pp. 159-169 ◽  
Author(s):  
Kyoung Yong Jeong ◽  
Jung-Won Park
Keyword(s):  
Food Processing ◽  
Adverse Reactions ◽  
Arginine Kinase ◽  
House Dust Mites ◽  
Food Allergies ◽  
Edible Insects ◽  
Dust Mites ◽  
Edible Insect ◽  
Respiratory Allergies ◽  
Inhalant Allergens

Edible insects are important sources of nutrition, particularly in Africa, Asia, and Latin America. Recently, edible insects have gained considerable interest as a possible solution to global exhaustion of the food supply with population growth. However, little attention has been given to the adverse reactions caused by insect consumption. Here, we provide an overview of the food allergens in edible insects and offer insights for further studies. Most of the edible insect allergens identified to date are highly cross-reactive invertebrate pan-allergens such as tropomyosin and arginine kinase. Allergic reactions to these allergens may be cross-reactions resulting from sensitization to shellfish and/or house dust mites. No unique insect allergen specifically eliciting a food allergy has been described. Many of the edible insect allergens described thus far have counterpart allergens in cockroaches, which are an important cause of respiratory allergies, but it is questionable whether inhalant allergens can cause food allergies. Greater effort is needed to characterize the allergens that are unique to edible insects so that safe edible insects can be developed. The changes in insect proteins upon food processing or cooking should also be examined to enhance our understanding of edible insect food allergies.

scholarly journals Congenital disorders of glycosylation in children - histopathological and ultrastructural changes in the liver

2021 ◽  
Author(s):  
Patryk Lipiński ◽  
Joanna Cielecka-Kuszyk ◽  
Elżbieta Czarnowska ◽  
Anna Bogdańska ◽  
Piotr Socha ◽  
...  
Keyword(s):  
Ultrastructural Changes ◽  
Congenital Disorders ◽  
Congenital Disorders Of Glycosylation

scholarly journals Characteristics associated with clinical response to Comano thermal spring water balneotherapy in pediatric patients with atopic dermatitis

2021 ◽  
Vol 47 (1) ◽  
Author(s):  
Davide Geat ◽  
Mattia Giovannini ◽  
Ezio Gabriele Barlocco ◽  
Riccardo Pertile ◽  
Stefania Farina ◽  
...  
Keyword(s):  
Atopic Dermatitis ◽  
Clinical Response ◽  
Disease Severity ◽  
Early Onset ◽  
Pediatric Patients ◽  
Thermal Spring ◽  
Spring Water ◽  
Food Allergies ◽  
Thermal Spring Water

Abstract Background Several studies have investigated the efficacy of balneotherapy in atopic dermatitis (AD), including a pediatric open randomized clinical trial conducted at the Comano thermal spring water center, which showed a significant reduction in AD severity and an improvement of the quality of life. However, so far many studies on balneotherapy in pediatric AD have included relatively small populations without identifying patients’ characteristics associated with their response. The aim of the present study was to identify any features associated with the clinical response to the Comano thermal spring water balneotherapy in a large cohort of pediatric AD patients. Methods An observational study was conducted on 867 children aged ≤16 years (females 50.5%, mean patient’s age 5.9 years, standard deviation ±3.6 years) with mild to severe AD who underwent balneotherapy at the Comano thermal spring water center (Comano, Trentino, Italy) from April to October 2014. Patients were stratified according to their disease severity, which was evaluated using five SCORing Atopic Dermatitis (SCORAD) categories before and immediately after a thermal spring water balneotherapy course. Potential characteristics associated with the patients’ clinical response to Comano thermal spring water balneotherapy were investigated. Results A statistically significant improvement in AD severity was observed after Comano thermal spring water balneotherapy (p < 0.0001). A significantly higher percentage of patients achieving improvement in AD severity was reported among children ≤4 years old (p < 0.0001) with early-onset AD (p < 0.0001), severe AD (p < 0.0001) or coexistent reported food allergies (p < 0.01). The therapy was well tolerated, and no relevant adverse effects were reported during the treatment course. Conclusions Comano thermal spring water balneotherapy is a safe complementary treatment for pediatric patients with AD, as it was able to reduce the disease severity, especially in children ≤4 years old, with early onset AD, severe AD or concomitant food allergies.

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