PRIMARY HYPOGAMMAGLOBULINEMIA - IMPAIRED LUNG-FUNCTION AND BODY GROWTH WITH DELAYED DIAGNOSIS AND INADEQUATE TREATMENT

  • 1 January 1984
    • journal article
    • research article
    • Vol. 65  (7) , 529-536
Abstract
The prevalence of primary hypogammaglobulinemia in Sweden was at least 2.0/100,000, notwithstanding the average diagnostic delay of 12 yr in the 26 patients studied in detail. Height and weight were reduced significantly in patients affected before 15 yr of age, particularly in those inadequately treated. Lung function was affected in 19 patients, in 18 demonstrated by the single breath nitrogen test. According to dynamic spirometry and static lung volume measurements 8 were obstructive, 2 were restrictive and 2 had a combined impairement. Patients who had inadequate Ig prophylaxis (< 25 mg/kg wk) showed significantly worse lung function compared to those adequately treated. Evidently, efficient Ig prophylaxis supported by antibiotics when required, seems to promote normal growth and to inhibit development of disabling lung disease.

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