Primary immunodeficiency disorders (PIDs) are uncommon conditions that require specialized immunologic services for diagnosis and management.
It is difficult to estimate the prevalence of these disorders from routinely collected health statistics.
We attempted to describe the prevalence of PID in Australia and the requirements for specific therapies, such as intravenous immunoglobulin, ascertained from a national register of PID.
A national longitudinal cross-sectional survey of patients with PID under the care of clinical immunologists was established by the Australasian Society of Allergy and Clinical Immunology in 1990.
Details of diagnosis and therapy were provided for patients with major PIDs including symptomatic IgA, IgG subclass, and complement deficiencies.
Subjects with asymptomatic IgA deficiency were not included.
The clinical features of the first 500 cases enrolled in the register were analyzed.
The most frequent type of PID was predominant antibody deficiency (71%). Common variable immunodeficiency, usually first seen as an antibody deficiency, was the single most common disorder with an estimated prevalence of 0.77/100,000 in the general population.
Other types of PID were infrequent, and of these, severe combined immunodeficiency accounted for 5.2% of cases.
The estimated prevalence of all forms of chronic granulomatous disease was 0.08/100,000. (...)
Mots-clés Pascal : Immunodéficit, Prévalence, Epidémiologie, Registre, Australie, Océanie, Immunoglobuline, Voie intraveineuse, Immunothérapie, Traitement, Homme, Immunopathologie
Mots-clés Pascal anglais : Immune deficiency, Prevalence, Epidemiology, Register, Australia, Oceania, Immunoglobulins, Intravenous administration, Immunotherapy, Treatment, Human, Immunopathology
Notice produite par :
Inist-CNRS - Institut de l'Information Scientifique et Technique
Cote : 97-0491384
Code Inist : 002B06D01. Création : 03/02/1998.