Chronic Aichi Virus Infection in a Patient with X-Linked Agammaglobulinemia
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To the Editor,
X-linked agammaglobulinemia (XLA) is caused by mutations in BTK, the gene encoding Bruton’s tyrosine kinase. BTK is critical for human B cell development and maturation, and hemizygous loss-of-function (LOF) mutations result in peripheral B cell lymphopenia, rudimentary tonsils and lymph nodes, and severely reduced to absent levels of serum immunoglobulins (Ig). Affected boys present in infancy with recurrent and often life-threatening respiratory tract and skin infections with encapsulated bacteria. Gastrointestinal infections with pathogens such as Giardia lamblia are also common, and they can manifest autoimmunity and autoinflammation [1, 2]. XLA patients also have extreme susceptibility to viral infections, especially with Enteroviruses, which can cause severe central nervous system (CNS) disease . The risk of Enteroviral infection is particularly high in patients who experience a diagnostic delay. Chronic Norovirus infection of the gut is another problematic...
KeywordsX-linked agammaglobulinemia XLA Bruton Aichi virus AiV1
GB is supported by the Research Foundation—Flanders (project G0C8517N). LM is supported by the CSL Behring Chair in Primary Immunodeficiencies, by the KID-FONDS charity of KU Leuven and by the Jeffrey Modell Foundation. EL is supported by the Research Foundation—Flanders (Clinical Investigator grant 1801110N). JRB is supported by a pediatric research grant from the Great Ormond Street Hospital Children’s Charity (Diagnosis of encephalitis by deep sequencing, V4317). All research at Great Ormond Street Hospital NHS Foundation Trust and UCL Great Ormond Street Institute of Child Health is made possible by the NIHR Great Ormond Street Hospital Biomedical Research Centre. The views expressed are those of the authors and not necessarily those of the NHS, the NIHR or the Department of Health. IM is supported by the Jeffrey Modell Foundation and by the Research Foundation—Flanders (project G0C8517N).
Compliance with Ethical Standards
Author IM has received a CSL Behring grant paid to Institution. The authors have no further conflicts of interest to disclose. All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. Informed consent was obtained from all individual participants included in the study.
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