Sinopulmonary Complications in Subjects With Primary Immunodeficiency

摘要: BACKGROUND: The aim of this work was to describe the frequency and spectrum sinopulmonary complications among subjects with primary immunodeficiency disorders. METHODS: included all patients who were registered prospectively between January 2004 December 2013 in Kuwait National Primary Immunodeficiency Disorders Registry. RESULTS: A total 202 during study period. Subjects combined immunodeficiencies most prevalent (65 subjects, 32.1%), followed by well-defined syndromes (45 22.2%) predominantly antibody deficiencies (35 17.3%). 295 manifestations observed 127 (63%); 157 (53.2%) presenting symptoms, 138 (46.8%) occurred after establishment disorder diagnosis. Sinopulmonary more common (2.3 manifestations/subject), (1.75 manifestations/subject). Pneumonia manifestation (108 episodes affecting 80 subjects), otitis media (81 59 bronchiectasis 28 (13.8%), asthma 22 (11%). Microbial organisms isolated 46 pneumonia (42.5%) (cytomegalovirus Pneumocystis jirovecii common). There 57 deaths (28%) Twenty-four (42%) due pulmonary as follows: (16 8%), hemorrhage (6 3%), aspiration (2 1%). CONCLUSIONS: are immunodeficiency. They can be serious continue occur even proper treatment is initiated. pulmonologist should play an important role management disorder.