4,878 results match your criteria Immunoglobulin A Deficiency


Protein-losing Enteropathy as a Complication and/or Differential Diagnosis of Common Variable Immunodeficiency.

J Clin Immunol 2022 Jun 23. Epub 2022 Jun 23.

Université de Lille, UFR Médecine, 59000, Lille, France.

As protein-losing enteropathy (PLE) can lead to hypogammaglobulinemia and lymphopenia, and since common variable immunodeficiency (CVID) is associated with digestive complications, we wondered if (1) PLE could occur during CVID and (2) specific features could help determine whether a patient with antibody deficiency has CVID, PLE, or both. Eligible patients were thus classified in 3 groups: CVID + PLE (n = 8), CVID-only (= 19), and PLE-only (n = 13). PLE was diagnosed using fecal clearance of α1-antitrypsin or 111In-labeled albumin. Read More

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coinfection is associated with improved IgE and IgG3 response against hookworm antigens.

Health Sci Rep 2022 Jul 14;5(4):e672. Epub 2022 Jun 14.

Department of Biomedical Sciences, School of Allied Health Sciences University of Cape Coast Cape Coast Ghana.

Background: and Hookworm infections are prevalent in West Africa and they cause iron deficiency anemia and protein malnutrition in Children. Immune response of these parasites interact and their interactions could have repercussions on vaccine development and efficacy. The current goal of hookworm eradication lies on vaccination. Read More

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Deficiency of leukocyte-specific protein 1 (LSP1) alleviates asthmatic inflammation in a mouse model.

Respir Res 2022 Jun 22;23(1):165. Epub 2022 Jun 22.

Veterinary Biomedical Sciences, University of Saskatchewan, Saskatoon, Canada.

Background: Asthma is a major cause of morbidity and mortality in humans. The mechanisms of asthma are still not fully understood. Leukocyte-specific protein-1 (LSP-1) regulates neutrophil migration during acute lung inflammation. Read More

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Serum Vitamin D Insufficiency in Hospitalized Full-Term Neonates at a Tertiary Hospital in Eastern China.

Front Pediatr 2022 26;10:878992. Epub 2022 May 26.

Department of Intervention, The First Affiliated Hospital of Soochow University, Suzhou, China.

Objective: This study explored the status of serum vitamin D in hospitalized full-term neonates at a tertiary hospital in eastern China.

Methods: A prospective study was conducted among 471 hospitalized full-term neonates at the Children's Hospital of Soochow University between January 1 and June 20, 2020. Perinatal clinical data, serum 25-hydroxyvitamin D (25(OH)D), laboratory examinations, serum calcium levels, and immune function were obtained and analyzed. Read More

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Roles of Secretory Immunoglobulin A in Host-Microbiota Interactions in the Gut Ecosystem.

Front Microbiol 2022 2;13:880484. Epub 2022 Jun 2.

Department of Genomics and Health, Fundación Para el Fomento de la Investigación Sanitaria y Biomédica de la Comunitat Valenciana (FISABIO), Valencia, Spain.

In the gastrointestinal tract (GIT), the immune system interacts with a variety of microorganisms, including pathogens as well as beneficial symbionts that perform important physiological functions for the host and are crucial to sustain intestinal homeostasis. In normal conditions, secretory immunoglobulin A (SIgA) is the principal antibody produced by B cells in the GIT mucosa. Polyreactivity provides certain SIgA molecules with the ability of binding different antigens in the bacterial surface, such as O-antigens and teichoic acids, while cross-species reactivity allows them to recognize and interact with different types of bacteria. Read More

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A Novel BLNK Gene Mutation in a Four-Year-Old Child Who Presented with Late Onset of Severe Infections and High IgM Levels and Diagnosed and Followed as X-Linked Agammaglobulinemia for Two Years.

Case Reports Immunol 2022 10;2022:7313009. Epub 2022 Jun 10.

Department of Pediatrics, Faculty of Medicine, Ege University, Izmir, Turkey.

Agammaglobulinemia is a rare inherited immunodeficiency disorder. Mutations in the BLNK gene cause low levels of mature B lymphocytes in the peripheral blood leading to recurrent infections. We present a four-year-old Turkish boy who had recurrent respiratory tract infections in the last six months. Read More

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Age and gender associated changes in immunoglobulin subclass levels specific to S. pneumoniae, serotype 1.

Comp Immunol Microbiol Infect Dis 2022 Jun 2;87:101834. Epub 2022 Jun 2.

Institute of Virology, Vaccines and Sera, Torlak, Vojvode Stepe 458, 11000 Belgrade, Serbia; Institute for Medical Research, National Institute of Republic of Serbia, University of Belgrade, Dr. Subotića 4, POB 39, 11129 Belgrade 102, Serbia. Electronic address:

S. pneumoniae is an important human pathogen which has a polysaccharide capsule with virulent properties. This work aims to estimate the titres of S. Read More

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Defining Clinical and Immunological Predictors of Poor Immune Responses to COVID-19 mRNA Vaccines in Patients with Primary Antibody Deficiency.

J Clin Immunol 2022 Jun 17. Epub 2022 Jun 17.

Section of Rheumatology, Allergy & Immunology, Department of Internal Medicine, Yale University School of Medicine, 300 Cedar Street, New Haven, Connecticut, 06520, USA.

Immune responses to coronavirus disease 2019 (COVID-19) mRNA vaccines in primary antibody deficiencies (PADs) are largely unknown. We investigated antibody and CD4 T-cell responses specific for SARS-CoV-2 spike protein (S) before and after vaccination and associations between vaccine response and patients' clinical and immunological characteristics in PADs. The PAD cohort consisted of common variable immune deficiency (CVID) and other PADs, not meeting the criteria for CVID diagnosis (oPADs). Read More

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Berardinelli Seip Syndrome: A rare case report.

J Pak Med Assoc 2022 May;72(5):969-971

Department of Pediatric Medicine, The Children's Hospital and Institute of Child Health, Lahore, Pakistan.

Berardinelli Seip Congenital Lipodystrophy (BSCL) or Congenital Generalized Lipodystrophy (CGL) is one of the four subgroups of lipodystrophy syndrome which is characterized by varying degrees of loss of adipose mass in the body. It is an extremely rare autosomal recessive disorder and commonly reported clinical presentations include muscular hypertrophy, gigantism, hepatomegaly, impaired glucose tolerance, acanthosis nigricans, hypertriglyceridaemia, cardiomyopathy, intellectual impairment, bone cysts and phlebomegaly. We present a case of a 4. Read More

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Dynamic monitoring and a clinical correlation analysis of the serum vitamin A, D, and E levels in children with recurrent respiratory tract infections.

Am J Transl Res 2022 15;14(5):3533-3538. Epub 2022 May 15.

Department of Pediatrics, Cangzhou Central Hospital Cangzhou, China.

Objective: To investigate the correlation of the serum vitamin A, D, and E (VA, VD, and VE) levels with the occurrence and development of recurrent respiratory tract infections (RRTIs).

Methods: A total of 129 children with respiratory tract infections (RTIs) treated in our hospital from January 2018 to February 2020 (the RTIs group) and 50 healthy children undergoing physical examinations (the control group) in our hospital were recruited as the study cohort. The serum VA, VD, and VE levels were measured upon admission (the active phase) and at two weeks after discharge (the stable phase). Read More

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Pediatric Celiac Disease and Selective IgA Deficiency: Unexpected Sequence of Events.

J Clin Immunol 2022 Jun 14. Epub 2022 Jun 14.

Hans Christian Andersen Children's Hospital, Odense University Hospital, Kloevervaenget 23C, 5000, Odense C, Denmark.

Purpose: Selective IgA deficiency (IgAD) is the most common primary immunodeficiency, frequently leading to only minor clinical complaints. IgAD may be associated with autoimmune diseases such as celiac disease (CeD). Although IgAD is thought to precede CeD and autoimmunity, the association between the two conditions has not been clarified. Read More

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Selective Immunoglobulin A Deficiency and the Microbiome.

Crit Rev Immunol 2021 ;41(6):1-12

Department of Medicine, Division of Allergy & Clinical Immunology, National Jewish Health, 1400 Jackson St., Denver, CO 80206, USA.

Selective immunoglobulin A (IgA) deficiency (SIgAD) is the most common primary immunodeficiency disease with a prevalence of about 1:500 individuals. SIgAD is heterogeneous, though thought to be due to a defect in the differentiation of IgA-bearing B lymphocytes into IgA-secreting plasma cells which provide a first line of defense against bacterial and viral pathogens. Although SIgAD was for a long time considered asymptomatic, longitudinal studies have revealed that about 80% of patients are symptomatic and can present with a range of phenotypes including allergic disease, recurrent bacterial respiratory tract infections, gastrointestinal disorders, and autoimmune diseases. Read More

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Preface Special Issue: Microbiome-Immune System Interactions.

Crit Rev Immunol 2021 ;41(6)

Laboratory of Immune Regulation, Department of Medicine, University of California at San Diego, La Jolla, CA 92093-0637, United States.

Body homeostasis, immune response to microbial infections or vaccination, control of cancer onset or autoimmune or inflammatory diseases, as well as autism or other behavioral disorders, among other examples, are now recognized to be associated with the complex constitution of the body's microbiome. Recent findings demonstrate that the microbial composition, i.e. Read More

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Nomenclature and diagnosis of seronegative coeliac disease and chronic non-coeliac enteropathies in adults: the Paris consensus.

Gut 2022 Jun 8. Epub 2022 Jun 8.

Gastroenterology Unit, IRCCS Pavia, ICS Maugeri, University of Pavia, Pavia, Italy.

Objective: Differential diagnosis of villous atrophy (VA) without coeliac antibodies in adults includes seronegative coeliac disease (CD) and chronic enteropathies unrelated to gluten, ie. non-coeliac enteropathies (NCEs). There is currently no international consensus on the nomenclature and diagnostic criteria for these enteropathies. Read More

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Prevalence of Coeliac Disease in Omani Adults with Iron Deficiency Anaemia of Unknown Cause: Case-finding study.

Sultan Qaboos Univ Med J 2022 May 26;22(2):262-267. Epub 2022 May 26.

Department of Family Medicine & Public Health, Sultan Qaboos University Hospital, Muscat, Oman.

Objectives: This study aimed to estimate the serological prevalence of coeliac disease in patients with iron deficiency anaemia (IDA) of unknown cause at a primary healthcare facility in Oman.

Methods: This prospective case-finding study was conducted at the primary care clinics in Sultan Qaboos University Hospital, Muscat, Oman from September 2018 to June 2020. Patients aged 18 to 55 years, with a haemoglobin (Hb) level <11. Read More

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Cernunnos defect in an Iranian patient with T B NK severe combined immunodeficiency: A case report and review of the literature.

Mol Genet Genomic Med 2022 Jun 2:e1990. Epub 2022 Jun 2.

Immunology and Allergy Department, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Background: Defective Cernunnos gene in nonhomologous end-joining (NHEJ) pathway of the DNA repair is responsible for radiosensitive severe combined immunodeficiency (SCID). Herein, presented a new patient with Cernunnos deficiency and summarized the clinical, immunological, and molecular features of reported patients in the literature.

Case: The patient was a 6-month-old female born to consanguineous parents. Read More

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Isolated coagulopathy without classic CRAB symptoms as the initial manifestation of multiple myeloma: A case report.

World J Clin Cases 2022 Apr;10(12):3822-3827

Department of Hematology, Mianyang Central Hospital, Mianyang 621000, Sichuan Province, China.

Background: Multiple myeloma patients usually present with CRAB symptoms (hypercalcemia, renal disease, anemia and bone diseases) as initial manifestations. Bleeding symptoms are less common, most of which result from thrombocytopenia or infiltration of plasmacytoma. Relatively, coagulopathy is not so common, especially isolated coagulopathy without CRAB manifestations, which is very rare. Read More

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Persistent Hyper IgA as a Marker of Immune Deficiency: A Case Report.

Antibodies (Basel) 2022 Apr 25;11(2). Epub 2022 Apr 25.

Children's Hospital and Medical Center, Department of Pediatrics, University of Nebraska Medical Center, Omaha, NE 68198, USA.

An elevated IgA level obtained in a 10-year-old male a year after an episode of pneumococcal sepsis led to the discovery of a broad-based IgG-specific antibody deficiency syndrome. The specifics of the case and pertinent literature are presented, including a discussion of the hyper-IgD syndrome. An elevated IgA, greater than two standard deviations above the expected age range should prompt a complete workup for selective antibody deficiency syndrome and adds an additional associated marker of an indolent hyper-IgD syndrome in a different clinical circumstance, although the lack of antibody response to vaccines is atypical of the hyper-IgD syndrome. Read More

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[Antibody deficiencies in adults. Forty years of follow up].

Medicina (B Aires) 2022 ;82(3):361-369

Unidad Inmunología e Histocompatibilidad, Hospital Dr. Carlos G. Durand, Buenos Aires, Argentina.

Antibody deficiencies (AD) are characterized by low or absent immunoglobulin levels or the inability to develop a specific antibody response. They are classified in primary (PAD) when there is an intrinsic immune defect, or secondary (SAD) to other diseases or drugs. The aim of our study was to review the evolutio n of AD assisted at the Immunology Unit, Hospital Durand between 1982 and 2020, divided into two periods: Period I (1982-2009) and Period II (2010-2020); to evaluate their growth, epidemiologic features and treatment options. Read More

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Clearance of Persistent SARS-CoV-2 RNA Detection in a NFκB-Deficient Patient in Association with the Ingestion of Human Breast Milk: A Case Report.

Viruses 2022 05 13;14(5). Epub 2022 May 13.

Laboratory of Pediatric Immunology, Center for Investigation in Pediatrics, Faculty of Medical Sciences, University of Campinas, Campinas 13083-887, Brazil.

Currently, there are no evidence-based treatment options for long COVID-19, and it is known that SARS-CoV-2 can persist in part of the infected patients, especially those with immunosuppression. Since there is a robust secretion of SARS-CoV-2-specific highly-neutralizing IgA antibodies in breast milk, and because this immunoglobulin plays an essential role against respiratory virus infection in mucosa cells, being, in addition, more potent in neutralizing SARS-CoV-2 than IgG, here we report the clinical course of an NFκB-deficient patient chronically infected with the SARS-CoV-2 Gamma variant, who, after a non-full effective treatment with plasma infusion, received breast milk from a vaccinated mother by oral route as treatment for COVID-19. After such treatment, the symptoms improved, and the patient was systematically tested negative for SARS-CoV-2. Read More

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Computational Analysis Reveals the Characteristics of Immune Cells in Glomerular and Tubulointerstitial Compartments in IgA Nephropathy Patients.

Front Genet 2022 4;13:838863. Epub 2022 May 4.

Department of Nephrology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China.

The commonalities and differences regarding immune states between glomerular and tubulointerstitial compartments of IgA nephropathy (IgAN) remains largely undetermined. We aim to perform bioinformatic analysis for providing a comprehensive insight into the characteristics of immune cells and associated molecular mechanisms in IgAN. We performed integrated bioinformatic analyses by using IgAN-related datasets from the Gene Expression Omnibus database. Read More

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Intranasal administration of a recombinant RBD vaccine induces long-term immunity against Omicron-included SARS-CoV-2 variants.

Signal Transduct Target Ther 2022 05 17;7(1):159. Epub 2022 May 17.

Laboratory of Aging Research and Cancer Drug Target, State Key Laboratory of Biotherapy and Cancer Center, National Clinical Research Center for Geriatrics, West China Hospital, Sichuan University, 610041, Chengdu, China.

The outbreak of coronavirus disease 2019 (COVID-19) has posed great threats to global health and economy. Several effective vaccines are available now, but additional booster immunization is required to retain or increase the immune responses owing to waning immunity and the emergency of new variant strains. The deficiency of intramuscularly delivered vaccines to induce mucosal immunity urged the development of mucosal vaccines. Read More

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Evaluation of Celiac Disease by Minimally Invasive Biomarkers in a Spanish Pediatric Population.

Int J Environ Res Public Health 2022 04 20;19(9). Epub 2022 Apr 20.

SNL Laboratory, School of Medicine and Dentistry, 15782 A Coruña, Spain.

Background: The diagnosis of celiac disease (CD) has been substantially improved with the availability of highly sensitive CD-specific IgA-TG2, Ig-GDP, and IgA-EMA. The European Society for Pediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN) published (2012) and updated (2020) diagnostic criteria for CD in order to simplify CD diagnosis and to avoid biopsies in selected patients.

Methods: A prospective study including 5641 pediatric patients (0-16 years old) from January 2012 to January 2019 was performed. Read More

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To DGP-IgG or not? a comparison of TTG-IgA and DGP-IgG.

Clin Chim Acta 2022 Jun 10;531:382-385. Epub 2022 May 10.

Department of Laboratories, Seattle Children's Hospital, Seattle, WA, United States; Department of Laboratory Medicine and Pathology, University of Washington, Seattle, WA, United States.

Background: We assessed the diagnostic utility of deamidated gliadin peptide immunoglobulin G (DGP-IgG) in pediatric patients without immunoglobulin A deficiency who underwent tissue transglutaminase immunoglobulin A (TTG-IgA) screening and biopsy.

Methods: Patients who had TTG-IgA performed in our laboratory had sample frozen over 1.5 y. Read More

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Factor H-Related Protein 1 Drives Disease Susceptibility and Prognosis in C3 Glomerulopathy.

J Am Soc Nephrol 2022 06 11;33(6):1137-1153. Epub 2022 May 11.

Department of Immunology, Complutense University and Research Institute Hospital 12 de Octubre (imas12), Madrid, Spain

Background: C3 glomerulopathy (C3G) is a heterogeneous group of chronic renal diseases characterized predominantly by glomerular C3 deposition and complement dysregulation. Mutations in factor H-related (FHR) proteins resulting in duplicated dimerization domains are prototypical of C3G, although the underlying pathogenic mechanism is unclear.

Methods: Using and assays, we performed extensive characterization of an FHR-1 mutant with a duplicated dimerization domain. Read More

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No child should suffer from vitamin K deficiency-induced bleeding disorders.

Clin Case Rep 2022 May 5;10(5):e05829. Epub 2022 May 5.

Department of Pediatrics Okanami General Hospital Iga Japan.

We encountered an 11-day-old male neonate with vitamin K deficiency-induced intracranial hemorrhage, despite receiving oral vitamin K (menaquinone-4) prophylaxis according to Japanese guidelines. This case suggests that the current vitamin K deficiency-bleeding prophylaxis programs cannot prevent bleeding completely. Better prophylaxis programs using both intramuscular and oral administration should be considered. Read More

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Posterior scleral perforation due to endogenous endophthalmitis in a pregnant with selective IgA deficiency.

Rom J Ophthalmol 2022 Jan-Mar;66(1):97-100

Department of Oculoplastic and Orbital Surgery, Hospital Universitario y Politécnico La Fe, Valencia, Spain.

We present the case of a 35-year-old female patient, pregnant in her third trimester, with no ophthalmologic history of interest and a medical history of IgA deficiency syndrome with bronchiectasis as the only symptomatology, who came to another center with clinical symptoms of ocular discomfort. She was initially diagnosed with anterior uveitis and treated with topical and periocular corticosteroids. Edema and palpebral erythema appeared a few days later and she was diagnosed with idiopathic orbital inflammation and was treated with intravenous (I. Read More

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Simultaneous quantification of plasma immunoglobulin subclasses for assessment of maternal and fetal immune response during pregnancy.

J Chromatogr A 2022 Jun 29;1673:463096. Epub 2022 Apr 29.

School of Environment, Guangdong Key Laboratory of Environmental Pollution and Health, Jinan University, Guangzhou 510632, China.

Measurement of immunoglobulin subclasses is a useful tool for exploring humoral immune deficiency in the presence of total immunoglobulins within reference intervals. Conventional methods for immunoglobulin measurement are mostly immunoassays, which are of low throughput and laborious to run multiple immunoglobulin subclass tests. Liquid chromatography-mass spectrometry (LC-MS) has emerged as a promising technology for the measurement of protein biomarkers in biological matrices, owing to its high specificity, selectivity, multiplexing, and wide dynamic range. Read More

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leaf polysaccharides regulation of immune response and gut microbiota composition in cyclophosphamide-treated mice.

Food Chem X 2022 Mar 29;13:100235. Epub 2022 Jan 29.

College of Food Science and Technology, Zhejiang University of Technology, Zhejiang, Hangzhou 310014, PR China.

In this study, the polysaccharides extracted from leaf (DOLP) was used in immune deficiency mice to evaluate the bioactivity. Thymus and spleen indices were calculated while the alleviation of the colon and liver histopathological progression was evaluated by H&E staining. The data indicated that DOLP improved immunity status by restoring the gut barrier and atrophy of immune organs. Read More

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