Journal of Clinical Immunology

Similar Kinetics of Pulmonary SARS-CoV-2 Load in Intensive Care Unit Patients with COVID-19 Pneumonia with or Without Autoantibodies Neutralizing Type I Interferons

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J Clin Immunol. 2024 Nov 20;45(1):45. doi: 10.1007/s10875-024-01839-x. ABSTRACT PURPOSE: The pathogenesis of life-threatening coronavirus disease 2019 (COVID-19) pneumonia in ICU patients can involve pre-existing auto-antibodies (auto-Abs) neutralizing type I interferons (IFNs). The impact of these auto-Abs on SARS-CoV-2 clearance in the lower respiratory tract (LRT) is unclear. METHODS: We performed a retrospective study in … Read more

Dupilumab in a 9-week-old with Netherton Syndrome Leads to Deep Symptom Control

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J Clin Immunol. 2024 Nov 15;45(1):42. doi: 10.1007/s10875-024-01837-z. ABSTRACT PURPOSE: Netherton syndrome (NS) is a rare inborn error of immunity (IEI) with an incidence of approximately 1:200,000 and the phenotypic triad of trichorrhexis invaginate (bamboo hair), congenital ichthyosiform erythroderma, and multiple atopic manifestations. Treatment options especially in infants are scarce and generally not licensed. METHODS: … Read more

Outcomes of X-Linked Agammaglobulinaemia Patients

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J Clin Immunol. 2024 Nov 14;45(1):40. doi: 10.1007/s10875-024-01829-z. ABSTRACT BACKGROUND: X-linked agammaglobulinaemia (XLA), caused by mutations in BTK, is characterised by low or absent peripheral CD19 + B lymphocytes and agammaglobulinaemia. The mainstay of treatment consists of immunoglobulin replacement therapy (IgRT). As this cannot fully compensate for the immune defects in XLA, patients may therefore … Read more

A large cohort from an immunology reference center and an algorithm for the follow-up of chronic neutropenia

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J Clin Immunol. 2024 Nov 5;45(1):38. doi: 10.1007/s10875-024-01816-4. ABSTRACT Chronic neutropenia causes involve nutritional deficiencies and inborn errors of immunity(IEI), such as severe congenital neutropenia. To classify common chronic neutropenia causes in a pediatric immunology unit. We enrolled 109 chronic neutropenia patients admitted to a pediatric immunology department between 2002-2022. We recorded clinical/laboratory features and … Read more

Endophilin A2 Deficiency Impairs Antibody Production in Humans

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J Clin Immunol. 2024 Nov 5;45(1):37. doi: 10.1007/s10875-024-01827-1. ABSTRACT Endophilin A2, the sole endophilin A family member expressed in hematopoietic cells, regulates various aspects of membrane dynamics, including autophagy and endocytosis. Recent studies in rodents highlight the essential role of endophilin A2 in modulating immune responses. Here we report a homozygous frameshift variant in the … Read more

Polysaccharide, Conjugate, and mRNA-based Vaccines are Immunogenic in Patients with Netherton Syndrome

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J Clin Immunol. 2024 Oct 30;45(1):36. doi: 10.1007/s10875-024-01828-0. ABSTRACT BACKGROUND: Netherton syndrome (NS) is a rare, severe genetic skin disorder, currently classified as an inborn error of immunity (IEI) due to previously reported immune dysregulation. We recently reported the results of an immunological evaluation showing no evidence for a relevant B- and/or T-cell mediated immunodeficiency, … Read more

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