Primary immunodeficiency syndrome in Japan. I. Overview of a nationwide survey on primary immunodeficiency syndrome

Abstract

The results of a nationwide survey on primary immunodeficiency syndrome (PIS) in Japan are presented. By the repeated questionnaire method, 497 PIS cases were collected prior to February 1979 with clinical information. Numbers of each type of PIS, age at the time of diagnosis, patient's status at the time of registration, familial incidence of PIS, and development of malignancy, autoimmune diseases, and allergic diseases among all reported patients are presented and discussed.

Clinical and Immunologic Characteristics of Healthy Children with Subnormal Serum Concentrations of IgG2

“…Also, despite apparent health in most IgA-deficient patients, there is an increased incidence of autoimmune disease among IgAdeficient individuals (28,29). Thus, we plan to follow the clinical status of these apparently healthy children with subnormal IgG2 levels over a longer period of time.…”

Population Prevalence of Diagnosed Primary Immunodeficiency Diseases in the United States

“…Whereas there have been estimates of these parameters from geographically limited studies [4,8,11,13,21,26]; those estimates were based on diagnosed cases. The number of undiagnosed cases is not known because there is no population-based screening process for PID anywhere in the world at any time during life.…”

Role of Antibodies in Controlling Viral Disease: Lessons from Experiments of Nature and Gene Knockouts

“…therapy may appear to be of little general importance. However, if the number of individuals affected by XLA (0.5 to 1 per million [4,29,41,65]) and CVID (about 0.5 to 1 per 100,000 [4,29,41,65]) is taken into account, it is safe to state that the incidence of severe viral manifestations, such as encephalitis, was considerably higher in these patients than in the general population, even when enterovirus infections are excluded. For instance, in the years of i.m.…”