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The Interstitial Pneumonias
Published in Lourdes R. Laraya-Cuasay, Walter T. Hughes, Interstitial Lung Diseases in Children, 2019
The exact etiology remains unknown. Among possible factors indicated as contributory46,47 are rheumatoid factor, antinuclear antibody, lupus erythematosus phenomenon, viral infection, and foreign body reaction. In patients with DIP, UIP, and LIP, circulating immune complexes that have been identified may play a role on pathogenesis.48 Also, IgM antigen-antibody complexes have been found in two infants with immunoglobulin G deficiency who had congenital rubella.49 Both infants also had IgM deposits in the alveolar interstitium.
Psychological well-being of children and young people with coeliac disease
Published in Clarissa Martin, Terence Dovey, Angela Southall, Clarissa Martin, Paediatric Gastrointestinal Disorders, 2019
Ruth A Howard, Gary Urquhart-Law
In addition to the presence of various GI and non-GI symptoms, children and young people may also be at risk of CD if they have a first-degree relative with CD – the strong genetic component to the condition means that family members are encouraged to be tested after the diagnosis of a first-degree relative (Husby et al., 2012; NICE, 2009). In addition, increased risk for CD is associated with the following diagnoses: type 1 diabetes mellitusDown’s syndromeautoimmune thyroid diseaseTurner’s syndromeWilliams’ syndromeselective immunoglobulin A deficiencyautoimmune liver diseasefirst-degree relatives with CD.
Human Adenovirus
Published in Sunit K. Singh, Human Respiratory Viral Infections, 2014
Joseph P. Lynch III, Adriana E. Kajon, Marcela Echavarria
AdV infections may complicate congenital immunodeficiency disorders such as severe combined immunodeficiency syndrome (SCID), common variable immunodeficiency, agammaglobulinemia, immunoglobulin A deficiency, and others.83,198 Incidence data for HAdV in patients with congenital immunodeficiencies are limited.83 In one review of 201 patients with Bruton’s X-linked agam-maglobulinemia, only one died as a result of HAdV infection.198 Data from a registry of primary immunodeficiency disorders in Kuwait analyzed 176 patients observed from 2004 to 2011 (mean follow-up 3.5 years); two died as a result of HAdV-associated liver failure.199
Hematopoietic stem cell transplantation in systemic autoinflammatory diseases - the first one hundred transplanted patients
Published in Expert Review of Clinical Immunology, 2022
Sara Signa, Gianluca Dell’Orso, Marco Gattorno, Maura Faraci
Patients with SIFD (Sideroblastic anemia with B-cell Immunodeficiency, periodic fever, and Developmental delay) present loss of function (LOF) homozygous or compound heterozygous mutations in the TRNT1 gene, coding for tRNA nucleotidyltransferase cytidine-cytidine- adenosine-adding 1, implicated in tRNAs maturation. The clinical picture is characterized by an early onset systemic inflammation with musculoskeletal and mucocutaneous alterations, associated with developmental delay [26,27]. Activation of UPR causes an abnormal B cell maturation leading to immunoglobulin (Ig) deficiency [28]. Anti-tumor necrosis factor (TNF) treatments are more effective than IL-1 inhibitors in these patients [7]. HSCT can correct the immunological and hematological defect, with resolution of inflammation and independence from transfusions and IVIG (intravenous immunoglobulin) replacement [26,27,29]. However, the neurologic manifestations possibly associated with this condition do not benefit from HSCT [29]: in fact, development of pigmentary retinitis in one patient was reported some years after HSCT [26]. At present, only four transplanted patients are described and one of them died soon after transplant due to a pulmonary hemorrhage [27,29] (Table 2).
Primary Immunodeficiency and Thrombocytopenia
Published in International Reviews of Immunology, 2022
Maryam Mohtashami, Azadehsadat Razavi, Hassan Abolhassani, Asghar Aghamohammadi, Reza Yazdani
CVID and selective immunoglobulin A deficiency (SIgAD) are the most common cases among IEI with no monogenic defect. Thrombocytopenia is often seen in CVID patients due to chronic infection mainly EBV, autoimmune medicated, and hypersplenism as well as cancer-related (BM infiltration of lymphoma and cytotoxic drug treatment). In SIgAD, decreased selective IgA serum is due to defects in B-cells. A defect in maturation B- cells to produce IgA is commonly observed in these patients [123]. Interestingly, some autoimmune diseases like ITP may accompany by SIgAD similar to the CVID pattern [124]. On the other hand, about 15%-20% of SIgAD patients have manifested IgG subclass deficiency simultaneously, termed IgG subclass deficiency with IgA deficiency [125], which this association increases the probability of thrombocytopenia [62, 126].
Combined anti-SARS-CoV-2 IgA, IgG, and IgM Detection as a Better Strategy to Prevent Second Infection Spreading Waves
Published in Immunological Investigations, 2022
Marzia Nuccetelli, Massimo Pieri, Francesca Gisone, Sergio Bernardini
The lateral flow immunochromatographic test shows the same specificities in groups 1 and 2 (94% and 96%, for IgG and combined IgA/IgM respectively), whereas sensitivities are different: 80% for IgA/IgM in group 1 and 71% for IgA/IgM in group 2; 71% and 84% for IgG, in groups 1 and 2 respectively (Table 1). The ELISA assays show the same specificities in groups 1 and 2 for IgM (100%) and a little increase in group 2 compared to group 1 for both IgG and IgA (94% versus 93% respectively); on the other hand, sensitivities show better performances on group 2 compared to group 1 (90% versus 82% for IgA; 92% versus 80% for IgM; 98% versus 94% for IgG). Since selective immunoglobulin A deficiency (SIGAD) is the most common inherited immunodeficiency disorder worldwide (with a prevalence ranging from 1 in 100 to 1 in 1000 people, depending by population), we detected total IgA in all anti-SARS-CoV-2 IgA negative samples to avoid false negative results. Only two samples displayed moderate IgA deficiency, with a concentration of 57 and 77 mg/dl (normal range: 101–645 mg/dl) that presumably did not interfere with our results.