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Is your kid actin out? A series of six patients with inherited ARPC1B deficiency and review of the literature
CONCLUSION: ARPC1B deficiency has a variable and heterogeneous clinical spectrum, expanded by these cases to include keloid scars and Epstein-Barr virus chronic hepatitis. A novel deletion in exon 8 is shared by three unrelated families and might be the result of a founder effect.PMID:36708766 | DOI:10.1016/j.jaip.2022.12.045
Source: Clinical Lung Cancer - January 28, 2023 Category: Cancer & Oncology Authors: Estefan ía Vásquez-Echeverri Marco Antonio Yamazaki-Nakashimada Edna Venegas Montoya Selma Cecilia Scheffler Mendoza Lina Maria Castano-Jaramillo Edgar Alejandro Medina-Torres Maria Edith Gonz ález-Serrano Melissa Espinosa-Navarro Juan Carlos Bustamant Source Type: research

Late-onset enteric virus infection associated with hepatitis (EVAH) in transplanted SCID patients
Transplanted SCID patients without B-cell reconstitution are at risk of developing late-onset hepatitis related to a chronic enteric viral infection (norovirus/sapovirus/human Aichi virus) and associated with effector memory CD8+ T cells expansion.
Source: Journal of Allergy and Clinical Immunology - January 9, 2023 Category: Allergy & Immunology Authors: Quentin Riller, Jacques Fourgeaud, Julie Bruneau, Suk See De Ravin, Grace Smith, Mathieu Fusaro, Samy Meriem, Aude Magerus, Marine Luka, Ghaith Abdessalem, Ludovic Lhermitte, Anne Jamet, Emmanuelle Six, Alessandra Magnani, Martin Castelle, Romain L évy, Source Type: research

High Frequency of Concomitant Food Allergy Development and Autoantibody Formation in Children Who Have Undergone Liver Transplantation
Conclusions. In contrast to respiratory allergy, food allergy and autoantibody formation occurred together in the same children who underwent liver transplantation at a frequency higher than would be expected by chance. This may reflect an underlying immune dysregulation that impairs immune tolerance to both food allergens and autoantigens.
Source: Transplantation - October 29, 2019 Category: Transplant Surgery Tags: Original Clinical Science–Liver Source Type: research

Myeloid-Derived Suppressor Cells: Ductile Targets in Disease
Discussion MDSCs violently emerge in pathological conditions in an attempt to limit potentially harmful immune and inflammatory responses. Mechanisms supporting their expansion and survival are deeply investigated in cancer, in the perspective to reactivate specific antitumor responses and prevent their contribution to disease evolution. These findings will likely contribute to improve the targeting of MDSCs in anticancer immunotherapies, either alone or in combination with immune checkpoint inhibitors. New evidence indicates that the expansion of myeloid cell differentiation in pathology is subject to fine-tuning, as its...
Source: Frontiers in Immunology - May 2, 2019 Category: Allergy & Immunology Source Type: research

Hepatic Natural Killer Cells: Organ-Specific Sentinels of Liver Immune Homeostasis and Physiopathology
Joanna Mikulak1,2, Elena Bruni1,2, Ferdinando Oriolo1,2, Clara Di Vito1 and Domenico Mavilio1,2* 1Unit of Clinical and Experimental Immunology, Humanitas Clinical and Research Center, Milan, Italy 2Department of Medical Biotechnologies and Translational Medicine, University of Milan, Milan, Italy The liver is considered a preferential tissue for NK cells residency. In humans, almost 50% of all intrahepatic lymphocytes are NK cells that are strongly imprinted in a liver-specific manner and show a broad spectrum of cellular heterogeneity. Hepatic NK (he-NK) cells play key roles in tuning liver immune response in b...
Source: Frontiers in Immunology - April 29, 2019 Category: Allergy & Immunology Source Type: research

Neurological Involvement in Primary Systemic Vasculitis
Conclusion Neurological involvement is a common complication of PSV (Table 1), and neurologists play an important role in the identification and diagnosis of PSV patients with otherwise unexplained neurological symptoms as their chief complaint. This article summarizes the neurological manifestations of PSV and hopes to improve neuroscientists' understanding of this broad range of diseases. TABLE 1 Table 1. Common CNS and PNS involvements of primary systemic vasculitis. Author Contributions SZ conceived the article and wrote the manuscript. DY and GT reviewed and edited the manuscript. All authors ...
Source: Frontiers in Neurology - April 25, 2019 Category: Neurology Source Type: research

The Emerging Epigenetic Role of CD8+T Cells in Autoimmune Diseases: A Systematic Review
The Emerging Epigenetic Role of CD8+T Cells in Autoimmune Diseases: A Systematic Review Qiancheng Deng1, Yangyang Luo1,2, Christopher Chang3, Haijing Wu1, Yan Ding4* and Rong Xiao1* 1Hunan Key Laboratory of Medical Epigenetics, Department of Dermatology, The Second Xiangya Hospital, Central South University, Changsha, China 2Department of Dermatology, Hunan Children's Hospital, Changsha, China 3Division of Rheumatology, Allergy and Clinical Immunology, University of California, Davis, Davis, CA, United States 4Department of Dermatology, Hainan Provincial Dermatology Disease Hospital, Haikou, China A...
Source: Frontiers in Immunology - April 17, 2019 Category: Allergy & Immunology Source Type: research

Editorial: Shaping of Human Immune System and Metabolic Processes by Viruses and Microorganisms
Conclusions In conclusion, articles in this Research Topic made a very significant contribution to our understanding of the role played by environmental factors, dysbiotic conditions, and infections in triggering diseases. Since this is a rapidly expanding area of research, many other factors contributing to the onset of these diseases are not covered here. We are confident, however, that further studies will expand the list as well as bring a better understanding of mechanisms involved in the onset of autoimmune and autoinflammatory diseases. Author Contributions All authors listed have made a substantial, direct and i...
Source: Frontiers in Microbiology - April 16, 2019 Category: Microbiology Source Type: research

The Antidepressant Mirtazapine Inhibits Hepatic Innate Immune Networks to Attenuate Immune-Mediated Liver Injury in Mice
Conclusion: Our data suggest that mirtazapine can attenuate hepatic innate immune responses that critically regulate the subsequent development of autoimmune liver injury. Therefore, given that it is a safe and widely used medication, mirtazapine may represent a novel therapeutic approach to autoimmune liver disease. Introduction Classically, autoimmune disease was considered a disorder of adaptive immunity (1). However, early innate immune responses are clearly important for driving subsequent adaptive immune responses in autoimmunity. In numerous autoimmune disease models, activation of resident tissue macrophages,...
Source: Frontiers in Immunology - April 11, 2019 Category: Allergy & Immunology Source Type: research

NIH scientists illuminate causes of hepatitis b virus-associated acute liver failure
(NIH/National Institute of Allergy and Infectious Diseases) National Institutes of Health scientists and their collaborators found that hepatitis B virus (HBV)-associated acute liver failure (ALF) -- a rare condition that can turn fatal within days without liver transplantation -- results from an uncommon encounter between a highly mutated HBV variant and an unusual immune response in the patient's liver that is mainly sustained by antibody-producing B cells.
Source: EurekAlert! - Medicine and Health - November 13, 2018 Category: International Medicine & Public Health Source Type: news

How Common is α -1-Antitrypsin Deficiency?
Discussion α-1-Antitrypsin Deficiency (A1AT) is a common single-gene mutation disease that is homozygous recessive. The normal allele is called M and the most common abnormal allele is Z. There are other alleles though. The gene codes for one of the primary protease inhibitors in the serum, thus those who are homozygous for the Z gene are sometimes referred to as “PIZZ” or “PIZ.” α-1-Antitrypsin is found in all body tissues but is especially important in the serum and lung. As noted it is one of the primary neutrophil protease inhibitors in the serum, and acts to neutralize these enzymes...
Source: PediatricEducation.org - April 24, 2017 Category: Pediatrics Authors: pediatriceducationmin Tags: Uncategorized Source Type: news

Liver transplantation in patients with primary antibody deficiency
Common variable immunodeficiency (CVID) and X-linked agammaglobulinemia (XLA) are the most common symptomatic primary antibody deficiencies (PADs). Patients with PAD may develop severe liver disease as part of their immunodeficiency syndrome.1 There has been a reluctance to perform liver transplantation (LTX) in these patients due to the risks associated with lifelong immunosuppression and fear of severe infections. Previously, iatrogenic chronic hepatitis C virus (HCV) infection was a leading cause of end-stage liver disease in patients with PAD and the lack of eradication of the virus after transplantation added to the r...
Source: Journal of Allergy and Clinical Immunology - December 5, 2016 Category: Allergy & Immunology Authors: Silje F. J ørgensen, Magnhild E. Macpherson, Kristian Bjøro, Tom H. Karlsen, Henrik M. Reims, Krzysztof Grzyb, Bjarte Fosby, Børre Fevang, Pål Aukrust, Ingvild Nordøy Tags: Letter to the Editor Source Type: research

The Management of Autoimmune Hepatitis Patients with Decompensated Cirrhosis: Real-World Experience and a Comprehensive Review
In this study, we sought to determine the therapeutic effect of corticosteroids in this special group of AIH patients. Eighty-two AIH patients with decompensated cirrhosis were included through a retrospective analysis from January 2009 to September 2015. Sixty-four patients were treated with corticosteroids while 18 patients did not receive any corticosteroids. Clinical, laboratory, and histological characteristics and outcomes were analyzed comparing corticosteroid-treated and untreated groups. Patients that did not receive corticosteroids were older than corticosteroid-treated patients and had a worse survival. In corti...
Source: Clinical Reviews in Allergy and Immunology - August 10, 2016 Category: Allergy & Immunology Source Type: research

What determines treatment success and future perspectives?
Hepatocellular carcinoma (HCC) is the second most common cause of cancer-related death in most Asian countries due to the high prevalence of hepatitis B viral infection.1 Surgical resection and liver transplantation are regarded as the main curative treatments for HCC. Many patients, however, are not eligible for resection due to locally advanced tumour, underlying liver cirrhosis with suboptimal liver reserve or metastatic disease on presentation.1 Puppala et al2 concisely summarise the modern image-guided therapy options for patients with advanced HCC, such as radiofrequency ablation (RFA), microwave ablation, percutaneo...
Source: Postgraduate Medical Journal - February 25, 2016 Category: Journals (General) Authors: Yau, T., Chiu, J., Cheung, T. T. Tags: Liver disease, Immunology (including allergy), Hepatic cancer, Radiology, Clinical diagnostic tests, Gastrointestinal surgery, Surgical oncology, Transplantation Editorials Source Type: research

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