1772 PULMONARY FUNCTION IN HYPOGAMMAGLOBULINEMIA

Abstract

Pulmonary function tests (PFT) were performed on 18 patients with hypogammaglobulinemia in the period 1975-84 (7 with X-linked agammaglobulinemia, and 11 with common variable hypogammaglobulinemia, of which 4 were adult when diagnosed). Mean age at most recent study was 18.12 years (range: 9.5-30). 16 of 18 patients had small airway obstruction with diminished MEF 75%, and FEF 25-75% being the most frequent and most severe abnormalities (mean % predicted 42.8% and 57.9% respectively). Decreased VC occurred in 11 patients with airway obstruction, and an increased RV/TLC ratio was seen in 8 patients. One patient had restrictive changes. 13 of 16 patients tested improved after bronchodilators. Seven patients have been followed in our clinic since early childhood and treated with IM gammaglobulin replacement and frequent or prophylactic antibiotics. Serial PFTs done in this group were unchanged or improved with time. A comparison of the mean % predicted values for this subgroup with the other seven patients who have had their disease since early childhood but who were initially treated with IM gammaglobulin replacement alone indicated greater obstructive disease in the second group. VC was 92.0% vs. 66.2% (p < 0.005); RV/TLC ratio was 27.9 vs 41.2. (p<0.05); FEF 25-75% was 65.8% vs 38.0% (p<0.05); and MEF 75% was 51.5% vs 17.8% (p < 0.05). These findings suggest that early and vigorous intervention with both IM gammaglobulin and frequent antibiotic use limits the progression of obstructive lung disease in patients with hypogammaglobulinemia.

Log in or create a free account to read this content

Gain free access to this article, as well as selected content from this journal and more on nature.com

Access through your institution

or

Sign in or create an account

Continue with Google

Continue with ORCiD