Pulmonary complications in primary acquired hypogammaglobulinemia: surgical considerations

With the discovery and routine use of antibiotics, a virtually new disease--primary acquired hypogammaglobulinemia--was recognized. More precise clinical, genetic, and laboratory endeavor has proved, in fact, that it is really one of a whole host of individual disease entities, all with the common feature of inadequate production and marshalling of gamma globulin to combat infection. Although the condition has been recognized in children's medicine for two decades, the survival of these early victims into adolescence and adulthood is now bringing them to the attention of surgeons as candidates for drainage or resection of suppurative disease of the lung, air tubes, and pleura. In fact a triad has emerged, with some of these patients having infectious disease in the lungs and sinuses associated with enlargement of the spleen when first seen. Often it is the radiologist who first suspects the diagnosis when he recognizes one or more features of this diagnostic triad. Three personal cases are presented together with a technique of management that appears successful. As with the recognition of any new disease, occult and subclinical presentations become more common as suspicion progresses, and ease of confirmation is afforded.