Allogeneic hematopoietic stem cell transplantation outcome in oldest known surviving patients with Wiskott-Aldrich syndrome.

Regardless of their age, adult patients with Wiskott-Aldrich syndrome should be considered for hematopoietic stem cell transplantation if clinically indicated.

Transition of care in inborn errors of immunity.

Purpose Of Review: This review outlines the principles of transition, summarizes current information about transition practices in inborn errors of immunity (IEI) and highlights general and specific considerations for transition of patients with these conditions.

Recent Findings: Recent surveys demonstrate the variability in access to and transition practices in IEI. Key challenges of transition in IEI from the perspective of healthcare professionals include lack of adult subspecialists, lack of access to holistic care and fragmentation of adult services.

Clinical Features, Immunological Characteristics, and Treatment Outcomes of Campylobacter spp. Infections in Patients With Common Variable Immunodeficiency.

Background: Campylobacter infection usually causes a self-limited clinical illness lasting 5 to 7 days, resolving without antimicrobial treatment in immunocompetent subjects. However, an inadequate immune response can lead to a prolonged and severe disease requiring antibiotics and more aggressive therapeutic approaches.

Objective: To comprehensively describe Campylobacter spp.

Commercial Immunoglobulin Products Contain Neutralizing Antibodies Against Severe Acute Respiratory Syndrome Coronavirus 2 Spike Protein.

Background: Patients with antibody deficiency respond poorly to coronavirus disease 2019 (COVID-19) vaccination and are at risk of severe or prolonged infection. They are given long-term immunoglobulin replacement therapy (IRT) prepared from healthy donor plasma to confer passive immunity against infection. Following widespread COVID-19 vaccination alongside natural exposure, we hypothesized that immunoglobulin preparations will now contain neutralizing severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) spike antibodies, which confer protection against COVID-19 disease and may help to treat chronic infection.

Impact of vaccination on hospitalization and mortality from COVID-19 in patients with primary and secondary immunodeficiency: The United Kingdom experience.

Background: Individuals with primary and secondary immunodeficiency (PID/SID) were shown to be at risk of poor outcomes during the early stages of the SARS-CoV-2 pandemic. SARS-CoV-2 vaccines demonstrate reduced immunogenicity in these patients.

Objectives: To understand whether the risk of severe COVID-19 in individuals with PID or SID has changed following the deployment of vaccination and therapeutics in the context of the emergence of novel viral variants of concern.

The presence of overlapping quality of life symptoms in primary antibody deficiency (PAD) and chronic fatigue syndrome (CFS).

Background: Fatigue, sleep disturbance and altered mood are frequently reported in patients with primary antibody deficiency syndrome (PADS) on adequate immunoglobulin replacement therapy. This study aimed to determine the frequency of symptoms compatible with chronic fatigue syndrome (CFS) in patients with PADS.

Methods: The study involved the distribution of 682 self-completed postal questionnaires to ascertain the presence and frequency of symptoms compatible with CFS in patients with PADS.

Case Report: Resolution of chronic urticaria following treatment of odontogenic infection.

Chronic spontaneous urticaria (CSU) is a condition characterised by the presence of hives with/without angioedema, that affects individuals on more days than not for 6 weeks or more. The role of infection as a potential trigger for CSU is well described, but the current clinical guidelines do not recommend routine screening for underlying infections. We report a case of severe prolonged chronic spontaneous urticaria in a 19-year-old, that went into rapid remission following the treatment of dental infection.

Beer, Cider, and Wine Allergy.

. Allergy to beer is often due to specific proteins in barley and sometimes to lipid transfer protein. Allergy to wine is frequently due to a sensitivity to grape proteins.

Pneumocystis jirovecii pneumonia in systemic autoimmune rheumatic disease: A case-control study.

Introduction And Objectives: Pneumocystis jirovecii pneumonia (PJP) is an opportunistic fungal infection that affects the immunocompromised. Patients with systemic autoimmune rheumatic disease are increasingly recognised as an at-risk clinical population with a high mortality. This case-control study examined differences in the characteristics and peripheral blood parameters between patients with systemic autoimmune rheumatic disease who developed PJP and gender, age and disease-matched controls.

Pneumocystis jirovecci pneumonia in connective tissue diseases: Comparison with other immunocompromised patients.

Introduction: Pneumocystis jirovecci pneumonia (PJP) is an opportunistic fungal infection occurring in immunocompromised patients, such as those with human immunodeficiency virus (HIV), organ transplantation, malignancies and connective tissue diseases (CTDs). Risk factors for PJP are not well characterised, leading to uncertainty regarding the indications for antimicrobial prophylaxis and monitoring. This study compared differences between patients with and without CTDs who developed PJP.

Genetic susceptibility to experimental autoimmune glomerulonephritis in the Wistar Kyoto rat.

In experimental autoimmune glomerulonephritis (EAG), a model of Goodpasture's disease, Wistar Kyoto (WKY) rats immunized with collagenase-solubilized glomerular basement membrane (GBM) or the recombinant NC1 domain of the α3 chain of type IV collagen [α3(IV)NC1] develop anti-GBM antibodies and focal necrotizing glomerulonephritis with crescent formation. However, Lewis (LEW) rats, which share the same major histocompatibility complex (MHC) haplotype, are resistant to EAG development. A genome-wide linkage analysis of backcrossed animals with EAG revealed a major quantitative trait locus (QTL) on rat chromosome 13 (LOD = 3.