She was diagnosed with IEI at the age of 5 years as her respiratory evaluations showed bronchiectasis, and her immunological workup showed IgG of 3.64 g/L, IgA of 0.55 g/L, and IgM of 3.40 g/L, partly due to an exudative enteropathy caused by a chronic inflammatory colitis. The gene discussed is CD40LG; the disease is bronchiectasis.