Abstract
Ten boys with congenital hypogammaglobulinemia from 4 families have been followed for 7-18 years. Five were diagnosed in infancy. Serum IgG was below 1 mg/ml in all but one (2.1 mg/ml after therapy), IgA below 0.05 mg/ml in all and IgM below 0.1 mg/ml in all but one (0.2 mg/ml). All received gammaglobulin from diagnosis and 5 plasma for ½ to 4 years. All had multiple episodes of acute bronchitis and pneumonia beginning early. H.influenzae (non-typeable) and Staph.aureus were the major pathogens, viruses were not found. One child age 10, died of bronchopneumonia and sepsis complicating adrenocorticosteroid treated chronic myositis. He and 3 of the 7 surviving children aged 7, 9 and 13 years, have no definite chronic pulmonary disease, though acute infections were often followed by a persistent cough in spite of intensive antibiotic therapy. This phenomenon led to bronchiectasis in the other 6. Two died of pulmonary insufficiency at ages 15 and 20. Among those surviving with bronchiectasis, 2 have severe restrictive and obstructive patterns in pulmonary function and 3 aged 16-21 had lobectomy. Pathological examination revealed severe bronchiectasis and sclerosis of bronchial arteries. The severity of pulmonary disease could not be correlated with initial serum immunoglobulin levels, proportion of B lymphocytes in peripheral blood, age at onset of gammaglobulin therapy, serum immunoglobulin levels during therapy or antibiotic therapy.
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Huang, N., Laraya-Cuasay, L., Huff, D. et al. PULMONARY COMPLICATIONS OF HYPOGAMMAGLOBULTNEMIA. Pediatr Res 8, 413 (1974). https://doi.org/10.1203/00006450-197404000-00441
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DOI: https://doi.org/10.1203/00006450-197404000-00441