Subject(s)
Allergens , Food Hypersensitivity/immunology , Skin Tests/methods , Adolescent , Adult , Child , Child, Preschool , Female , Humans , Male , Middle Aged , Predictive Value of TestsABSTRACT
To determine the best predictors of chronic pulmonary disease in patients with hypogammaglobulinemia, we evaluated the clinical records, chest x-ray films, and pulmonary function tests of 10 patients with X-linked agammaglobulinemia (XLA) followed for a mean of 12.5 years, and 12 patients with common variable immunodeficiency (CVID) followed for a mean of 10.5 years. These patients, most of whom were treated with intramuscular gamma globulin and long-term oral antibiotics, had very few pneumonias after diagnosis. The patients with XLA had 0.10 pneumonias per treatment year, and the patients with CVID had 0.18 pneumonias per treatment year. Seven of the 10 patients with XLA had normal chest x-ray films 8 to 15 years after diagnosis, and none had bronchiectasis. Pulmonary disease was more common and more severe in the group with CVID, but five patients in this group also had normal chest x-ray films after long follow-up. In the entire group of 22 patients, nine of the 10 patients with abnormal chest x-ray films on most recent evaluation already had pulmonary disease at the initial visit (p = 0.00002). These studies indicate that the best predictors of good pulmonary function in patients with hypogammaglobulinemia are early diagnosis and good compliance with gamma globulin replacement therapy and oral antibiotics.
Subject(s)
Agammaglobulinemia/complications , Lung Diseases/epidemiology , Agammaglobulinemia/epidemiology , Child , Chronic Disease , Female , Genetic Linkage , Humans , Immunologic Deficiency Syndromes/complications , Immunologic Deficiency Syndromes/epidemiology , Incidence , Lung/diagnostic imaging , Lung Diseases/diagnosis , Lung Diseases/etiology , Male , Philadelphia/epidemiology , Pneumonia/diagnosis , Pneumonia/epidemiology , Pneumonia/etiology , Prognosis , Radiography , Respiratory Function Tests , Retrospective Studies , X ChromosomeABSTRACT
Four infants with hyperimmunoglobulinemia E presented with a persistent papulovesicular rash and eosinophilia. Serum IgE levels and specific anti-Staphylococcus aureus IgE antibodies were studied during the first year of life. Increased anti-S aureus IgE antibodies were an early indicator of the disease; they appeared as soon as 7 weeks of age in patient 1 and before 1 year of age for the other patients. These antibodies were detected before the development of deeper staphylococcal infections. Prompt diagnosis and treatment are important, as they may prevent long-term infectious complications.
