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Life-Threatening Infections Due to Live-Attenuated Vaccines: Early Manifestations of Inborn Errors of Immunity

Published in Journal of Clinical Immunology

Live-attenuated vaccines (LAVs) can protect humans against 12 viral and three bacterial diseases. By definition, any clinical infection caused by a LAV that is sufficiently severe to require medical intervention attests to an inherited or acquired immunodeficiency that must be diagnosed or identified. Self-healing infections can also result from mi...

Role of Allogeneic Hematopoietic Stem Cell Transplant for Chronic Granulomatous Disease (CGD): a Report of the United St...

Published in Journal of Clinical Immunology

PurposeChronic granulomatous disease (CGD) is a primary immunodeficiency for which allogeneic hematopoietic stem cell transplant (HSCT) offers potential cure. Direct comparison of HSCT to non-HSCT management in the North American population was performed to identify clinical factors associated with overall survival (OS) and transplant-related survi...

Variable Responses to Tocilizumab in Four Patients with Schnitzler Syndrome

Published in Journal of Clinical Immunology

Enterovirus-Associated HLH: Addition of Anakinra to IVIG and Corticosteroids

Published in Journal of Clinical Immunology

Recurrent Salmonella typhi Infection and Autoimmunity in a Young Boy with Complete IL-12 Receptor β1 Deficiency

Published in Journal of Clinical Immunology

Fifteen Years of the J Project

Published in Journal of Clinical Immunology

IKZF1 Loss-of-Function Variant Causes Autoimmunity and Severe Familial Antiphospholipid Syndrome

Published in Journal of Clinical Immunology

Factors Beyond Lack of Antibody Govern Pulmonary Complications in Primary Antibody Deficiency

Published in Journal of Clinical Immunology

PurposePulmonary complications occur frequently in primary antibody deficiency (PAD). While the impact of antibody deficiency may appear implicit for certain respiratory infections, immunoglobulin replacement therapy does not completely ameliorate pulmonary complications in PAD. Thus, there may be antibody-independent factors influencing susceptibi...

A Unique Presentation of Infantile-Onset Colitis and Eosinophilic Disease without Recurrent Infections Resulting from a ...

Published in Journal of Clinical Immunology

PurposeThis study aimed to characterize the clinical phenotype, genetic basis, and consequent immunological phenotype of a boy with severe infantile-onset colitis and eosinophilic gastrointestinal disease, and no evidence of recurrent or severe infections.MethodsTrio whole-exome sequencing (WES) was utilized for pathogenic variant discovery. Wester...

Defining Primary Selective IgM Deficiency

Published in Journal of Clinical Immunology