PULMONARY-DISEASES IN CHILDREN WITH SEVERE COMBINED IMMUNE-DEFICIENCYAND DIGEORGE-SYNDROME

Pulmonary disease is a common presenting feature and complication of T -cell immunodeficiency. We retrospectively reviewed 15 children with s evere combined immune deficiency (SCID) and 19 children with DiGeorge syndrome at the time of their first presentation to the Royal Children 's Hospital in the 15-year period from 1981 to 1995. In children with SCID, pulmonary disease was a common (67%) presenting feature and the organisms identified were Pneumocystis carinii (PCP) (n = 7), bacteria (n = 4), viruses (n = 3), and a fungus (n = 1). Late pulmonary compli cations included lower respiratory tract infections, bronchiolitis obl iterans, and lymphointerstitial pneumonitis. Pulmonary infections were common (17 occasions) and the organisms identified were bacteria (n = 7), viruses (n = 6), fungi (n = 3), and Mycobacterium tuberculosis (n = 1). Pulmonary complications were responsible for 5 of 9 deaths. PCP was not identified as a late complication in any child, presumably as a result of effective prophylactic therapy. Although pulmonary diseas e was not a major presenting feature in children with DiGeorge syndrom e, pulmonary complications were common. These included recurrent bacte rial and viral infections and bronchomalacia, which complicated manage ment and predisposed to morbidity and mortality, even in those without a T-cell defect. We conclude that pulmonary disease is a common manif estation in children with SCID and DiGeorge syndrome. (C) 1997 Wiley-L iss, Inc.