LIFE-THREATENING LUNG INFECTION WITH PNEU MOCYSTIS-CARINII IN A 4 MONTHS OLD BOY SUFFERING FROM HYPER-IGM-SYNDROME

At the age of four months, a boy with a normal history and family hist ory, suddenly fell ill with a life-threatening pneumocystis carinii-pn eumonia. Surprisingly, this opportunistic infection was not brought ab out by a T-cell deficiency. However, the patient's diagnosis turned ou t to be the rare ''Hyper-IgM-syndrome'', confirmed by: serum levels of IgM always at least normal whereas IgG, IgA and IgE were markedly dec reased or absent; the development of neutropenia and occasional diarrh ea. Generally, infections with pneumocystis carinii are rare in isolat ed deficiencies of immunoglobulines, but relatively frequent in primar y ''Hyper-IgM-syndrome'' (approx. 12% of the cases described). The boy finally recovered after receiving Cotrimoxacol (20 mg/kg bw/d) in an intensive care unit. Now, at the age of nearly two his condition is al most good under regular substitution of IgG. Cotrimoxacol (4 mg/kg bw/ d) is recommended to prevent further pneumocystis carinii infections a nd most of the pathogenes which frequently appear in neutropenias.