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Pulmonary manifestations of immune dysregulation in CTLA‐4 haploinsufficiency and LRBA deficiency

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The primary immunodeficiency syndromes of cytotoxic T lymphocyte‐associated protein 4 (CTLA‐4) haploinsufficiency and lipopolysaccharide‐responsive and beige‐like anchor protein (LRBA) deficiency present with multisystem immune dysregulation. The aim of this study… Click to show full abstract

The primary immunodeficiency syndromes of cytotoxic T lymphocyte‐associated protein 4 (CTLA‐4) haploinsufficiency and lipopolysaccharide‐responsive and beige‐like anchor protein (LRBA) deficiency present with multisystem immune dysregulation. The aim of this study was to characterize and compare the pulmonary manifestations of these two diseases.

Keywords: ctla haploinsufficiency; pulmonary manifestations; immune dysregulation; lrba deficiency

Journal Title: Pediatric Pulmonology
Year Published: 2021

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