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This is one of the few reported XLA cases experiencing severe PCP.
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Methods: The LTD model for CVID and XLA was derived using decision analysis methodology.
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These findings strongly support pursuit of B lineage-targeted LV gene therapy in human XLA.
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Methods: We present a case report of a family with two affected patients with XLA.
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Further studies are needed to improve diagnosis and management of GI conditions in XLA patients.
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Although there is increased awareness of autoimmune and inflammatory complications in X-linkedagammaglobulinemia (XLA), the spectrum of gastrointestinal manifestations has not previously been fully explored.