1987 Volume 10 Issue 2 Pages 171-179
The authors reported and discussed the clinical and immunological features of 80 children registered as sufferers of common variable immunodeficiency to the All Japan Immunodeficiency Registry up to April 16, 1986.
12.3% of the patients reported some cases suspected of common variable immunodeficiency in their family members.
Mean age of the diagnosis of the patients was 65.0±49.0 months old. Mean age of the 60 survivors at the time of registration was 112.1±66.4 months old, and mean age of death of the 19 expired patients was 76.3±84.4 months old.
Respiratory tract infection was the most common initial symptom among the patients, and 92.5% of the all patients reported susceptibility to infection. Pneumonia (55.0%), otitis media (37.4%), pyodermia (30.0%), gastroenteritis (17.5%) and sepsis (10.0%) were common infection among the reported patients.
Eczema (15.0%) and bronchial asthma (8.8%) were occasionally seen and there were two cases with rheumatoid arthritis, one with SLE and one with Hashimoto's thyroiditis. One patient was suffered from B cell type malignant lymphoma.
48 of all 80 patients were also reported the levels of serum immunoglobulins, and 87.5% of the patients showed significantly low levels of IgG for their age. Among these patients with hypo-IgG-emia, 43.8% also showed significantly low levels of both IgA and IgM and 29.2% also showed significantly low levels of IgA. 54.2% of 24 patients with hypo-IgG-emia were reported to have decreased numbers of B cells in the peripheral blood.
Replacement of IgG with human serum imunoglobulin preparations was reported to be the most common effective therapy applied to these patients with common variable immunodeficiency.
