A 6-year-old boy with recurrent otitis media, sinopulmonary infections, wheezing, velopharyngeal insufficiency, and speech delay. IgM ↓, IgA, and IgG N. Protective antibody titers to protein and carbohydrate antigens.A 14-year-old girl with neonatal seizures, atrial and ventricular septal defects, recurrent otitis media, intellectual disability, and asthma. IgM ↓, IgA, and IgG N. Protective antibody titers to protein and carbohydrate antigens. Here, CD40LG is linked to ventricular septal defect 1.