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. 2019 Feb 26;39(Suppl 1):1–151. doi: 10.1007/s10875-019-00597-5
viral infections bacterial infections end organ damage immune dysregulation immunology
mother mumps and varicella disease (despite immunization), recurrent HSV1 infections (face, index finger)

tracheobronchitis

(H. influenza)

P. jirovecii pneumonia

bronchiectases, COPD, loss of distal phalanx (Dig.II) following HSV infection, ulceration of vocal chords, hypopituitarism, vasculitis of lower legs IgG 3.44 g/l, IgA < 0.26 g/l, IgM 0.45 g/l, no protective tetanus and diphtheria antibodies following regular immunization, absence of autoantibodies, initally low switched memory B- cells, total loss of peripheral B-cells
daughter recurrent upper and lower respiratory infections, recurrent HSV1 infections periorally mild restrictive lung disease, alopecia totalis, nail dystrophy

IgG 2.33 g/l, IgA 0.23 g/l, IgM 0.34 g/l,

B-cells 1900/μl, no protective tetanus, measles or HiB antibodies (but positive to rubella)