title: [Common variable immunodeficiency--analysis of the clinical aspects and results of substitution treatment].
abstract: The clinical picture and the effects of treatment with intravenous immunoglobulins of 14 adult patients (4 women, 10 men) with common variable immunodeficiency are presented. In most patients the disease began in the second decade of life (17.4 years on average), and the diagnosis was delayed by 7.5 years. Mean observation time was 38.5 months. Recurrent sinopulmonary infections were the most significant clinical feature and were observed in all patients, although chronic diarrhea and viral hepatitis, predominant of B type, were also frequently seen (both in 57% of patients). The diagnosis was made according to following criteria: frequent bacterial infections of the respiratory tract, serum IgG levels reduced below 300 mg/dl, (mean, 60.7 mg/dl), normal numbers of circulating B lymphocytes and exclusion of the secondary type hypogammaglobulinaemia. After 6 months of treatment with fresh plasma 20 ml/kg or intravenous immunoglobulins 200-400 mg/kg, mean serum IgG levels rose to 654 mg/dl. Treatment was clinically efficacious and markedly reduced the number of infections of the respiratory tract. The delay of the diagnosis, resulted in high rate of irreversible damage of respiratory tract observed in our group of patients.
authors.affiliation: I Kliniki Gruźlicy, Warszawie.
authors.forename: J
authors.initials: J
authors.lastname: Kuś
authors.forename: D
authors.initials: D
authors.lastname: Maziarka
mesh_terms: D000293:Adolescent; D000328:Adult; D016516:CD4-CD8 Ratio; D017074:Common Variable Immunodeficiency / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*; D003967:Diarrhea / Q000453:epidemiology / Q000209:etiology; D005260:Female; D006525:Hepatitis, Viral, Human / Q000453:epidemiology / Q000209:etiology; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D015994:Incidence; D008297:Male; D008875:Middle Aged; D012141:Respiratory Tract Infections / Q000453:epidemiology / Q000209:etiology; D016896:Treatment Outcome
chemical_list: D007074:Immunoglobulin G
vernacular_title: Pospolity zmienny niedobór odporności--analiza obrazu klinicznego i wyników leczenia substytucyjnego.