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The phenotype included hypogammaglobulinemia (88·9per cent), reduced switched memory B cells (60·3%), and respiratory (83%) and intestinal (28·6%) attacks, hence characterizing the condition as primary immunodeficiency. But, the high-frequency of autoimmunity (57·4%), lymphoproliferation (52·4%), non-infectious enteropathy (23·1%), opportunistic infections (15·7%), autoinflammation (29·6%), and malignancy (16·8%) identified NF-κB1-related disease as an inborn error of immunity with