CD40LG and aortic valve insufficiency: Low %CD19 (6%; RV: 8-45*).Absent IgA with low IgG (36 mg/dL; RV: 345-1213**) and normal IgM.Moderately reduced proliferation assays.Poor growth and weight gain, facial dysmorphism, dysplastic mitral valve and aortic valve insufficiency, delayed teeth eruption, autoimmune hypothyroidism, atrophic gastritis, recurrent infections, hearing loss.