Respiratory complications of primary hypogammaglobulinemia

The primary hypogammaglobulinemias, with onset of acute and recurrent bacterial infections in infancy and early childhood, consist of a heterogeneous group of largely genetically determined antibody deficiency states including that congenital sex-linked form, Bruton's agammaglobulinemia. Patients with panhypogammaglobulinemia require continuous gamma globulin therapy; in spite of this, they continue to develop infections of the upper and lower respiratory tract in the form of otitis media, mastoiditis, sinusitis, rhinitis, pharyngitis, tracheobranchitis, or pneumonia of a chronic and recurrent nature. The frequency and severity of these infections vary from patient to patient. These episodes all respond to antibiotic administration, often with a prolonged course. Many patients develop permanent pulmonary sequelae in the form of atelectasis, bronchiectasis, and pulmonary fibrosis. Most of these changes involve focal areas of the lower right middle and left lingular lobes. Occasionally, the patient may develop generalized bronchiectasis but without hilar lymphadenopathy. Management emphasizes early detection, early institution of gamma globulin treatment, and administration of appropriate antibiotic therapy at the earliest onset of infection. Good pulmonary toilet, nutritional care, emotional care, and a loving home environment are of utmost importance. These patients should be followed in a medical center with the joint effort of specialists in various disciplines.