Mauno Vihinen¹, Pekka T. Mattsson^2,3 and C. I. Edvard Smith²

¹Department of Biosciences, Division of Biochemistry, P. O. Box 56, FIN-00014 University of Helsinki, Finland

²Center for BioTechnology, Department of Bioscience at Novum, Karolinska Institute, S-141 57 Huddinge and Department of Immunology, Microbiology, Pathology and Infectious Diseases (IMPI), Karolinska Institute, Huddinge University Hospital, S-141 86 Huddinge, Sweden

³Department of Biochemistry and Food Chemistry, University of Turku, Vatselankatu 2, Arcanum, FIN-20014 Turku, Finland

Received 11/24/96; Accepted 12/16/96; On-line 01/01/97

3. SPECTRUM OF INFECTIONS AND TREATMENT

The onset of symptoms varies extensively; most patients will show an increased frequency of infections during their first year of life, whereas a few may be asymptomatic until adolescence. Pneumonia, otitis media, and diarrhea are frequent clinical presentations. Sinusitis, conjunctivitis, and pyoderma are also prevalent. Spread of the infection through the blood results in septicemia, meningitis, septic arthritis and sometimes osteomyelitis. Thus, a highly increased frequency of infections is seen essentially in all organs, with the possible exception of the urinary tract, in which only infections with mycoplasma species seem to be overrepresented (4, 9).

Typically, in patients with XLA the infections are bacterial and are caused by Haemophilus influenzae or Streptococcus pneumoniae. These infections affect all individuals with defective humoral immune responses. However, in contrast to most other primary humoral immunodeficiencies, enteroviral infections may cause an often fatal, slowly progressing disease affecting the central nervous system (5, 9). Thus, antibodies directed against these viruses play a pivotal role in the immune defense.

Bacterial infections are treated with a high dose of antibiotics for prolonged periods. The enteroviral infections may respond to gammaglobulins, but this is not always the case. However, it seems as if high dose gammaglobulin prophylaxis prevents enteroviral infections (9). High dose gammaglobulin given by intravenous or subcutaneous infusions also decrease the number of bacterial infections.