title: Primary immunodeficiency syndromes in Italy: a report of the national register in children and adults.
abstract: The Italian Register for Immune Deficiencies was organized in 1977. Seven hundred ninety-seven cases of primary immunodeficiencies, diagnosed according to the World Health Organization criteria, have been registered up to April 1982. In this paper we report the percentages of the different forms of primary immunodeficiencies and the incidence of neoplastic and autoimmune diseases. A prevalence of humoral defects and selective IgA deficiency was found, followed by T-cell disorders. Two and thirty-eight hundredths percent of the patients developed cancer, and 5.89% developed autoimmune diseases. A comparison among the Italian, Swedish, and Japanese Registers showed a higher incidence of IgA defects in the Italian Register while nonspecific phagocyte defects were more frequent in the Swedish Register. The incidence of cancer and autoimmune disorders is similar to that observed in other registers.
mesh_terms: D000328:Adult; D001327:Autoimmune Diseases / Q000150:complications / Q000453:epidemiology; D002648:Child; D004406:Dysgammaglobulinemia / Q000150:complications; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications; D017098:IgA Deficiency; D007153:Immunologic Deficiency Syndromes / Q000150:complications / Q000453:epidemiology*; D007558:Italy; D007564:Japan; D009369:Neoplasms / Q000209:etiology; D012042:Registries; D013548:Sweden; D014481:United States