ArticleJournal of clinical immunology2007
Primary immunodeficiency diseases in Australia and New Zealand.
Article in Journal of clinical immunology, 2007. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 52 papers, 3 of them syntheses that pooled it.
What it found
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
52 citing papers in PubMed, 3 syntheses or guidelines pooled it.
- Pooled it
- A Systematic Review and Meta-regression Analysis on the Impact of Increasing IgG Trough Level on Infection Rates in Primary Immunodeficiency Patients on Intravenous IgG Therapy.Journal of clinical immunology · 2020Pooled it
- Global Distribution of Common Variable Immunodeficiency (CVID) in the Light of the UNDP Human Development Index (HDI): A Preliminary Perspective of a Rare Disease.Journal of immunology research · 2020Pooled it
- A single-center pilot study in Malaysia on the clinical utility of whole-exome sequencing for inborn errors of immunity.Clinical and experimental immunology · 2021Trial
- The Kuwait National Primary Immunodeficiency Registry 2004-2018.Frontiers in immunology · 2019Trial
- Efficacy, safety and pharmacokinetics of a novel subcutaneous immunoglobulin, Evogam®, in primary immunodeficiency.Journal of clinical immunology · 2012Trial
- Rheumatologic manifestations in children with underlying inborn errors of immunity.BMC rheumatology · 2025Article
- Time to diagnosis for a rare disease: managing medical uncertainty. A qualitative study.Orphanet journal of rare diseases · 2024Article
- Fecal Immunoglobulin Levels as a Modifier of the Gut Microbiome in Patients with Common Variable Immunodeficiency.Journal of clinical immunology · 2023Article
- The yin and the yang of early classical pathway complement disorders.Clinical and experimental immunology · 2022Review
- Clinical and Genetic Spectrum of Inborn Errors of Immunity in a Tertiary Care Center in Southern India.Indian journal of pediatrics · 2022Article
- Immune Sexual Dimorphism: Connecting the Dots.Physiology (Bethesda, Md.) · 2022Review
- Cytomegalovirus Infections in Children with Primary and Secondary Immune Deficiencies.Viruses · 2021Review
- Systematic review of literature and analysis of big data from the National Health Insurance System on primary immunodeficiencies in Korea.Clinical and experimental pediatrics · 2021Article
- The Primary Immunodeficiency Database in Japan.Frontiers in immunology · 2021Review
- The Italian Registry for Primary Immunodeficiencies (Italian Primary Immunodeficiency Network; IPINet): Twenty Years of Experience (1999-2019).Journal of clinical immunology · 2020Article
- Flow Cytometry for the Diagnosis of Primary Immunodeficiency Diseases: A Single Center Experience.Allergy, asthma & immunology research · 2020Article
- Frequency and Manifestations of Autoimmunity Among Children Registered in the Kuwait National Primary Immunodeficiency Registry.Frontiers in immunology · 2020Article
- Delayed Diagnosis and Complications of Predominantly Antibody Deficiencies in a Cohort of Australian Adults.Frontiers in immunology · 2018Article
- Primary Immunodeficiency Diseases in Highly Consanguineous Populations from Middle East and North Africa: Epidemiology, Diagnosis, and Care.Frontiers in immunology · 2017Review
Corrections and comments
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Authors and funding
2 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
introductionDespite rapid developments in the science of primary immunodeficiency diseases (PID), population characteristics and the burden of disease are poorly characterized. Aggregated data on PID via patient registries are a key component of the public health response. The web-enabled Australasian Society of Clinical Immunology and Allergy PID Register was designed and implemented to address gaps in knowledge of PID.
methodsThe register provided a cumulative, cross-sectional survey of PID patients in Australia and New Zealand via an online, single time point, center-based, voluntarily recalled, and patient-consented questionnaire.
resultEighty-eight centers reported 1,209 patients across 56 separate PID syndromes. The study prevalence (cases per 100,000 population) was 5.6 for Australia, 12.4 for the state of South Australia, and 4.9 for Australia and New Zealand combined. Predominately antibody deficiency syndromes accounted for 77% of patients. Common variable immunodeficiency was the most common diagnosis. Patients were geographically dispersed with 80% of centers reporting caseloads of less than 20 patients. Potentially preventable complications of disease were common. Immunoglobulin replacement therapy was used in 30 conditions with 26.5% of the total recipients having antibody deficiency disorders with normal serum IgG.
conclusionPID in Australia and New Zealand are prevalent, clinically diverse, geographically dispersed, and are characterized by high rates of potentially preventable morbidity and resource utilization. A public health focus on PID is required, including strategies to correct disparities in access to care, improve molecular diagnostics and reduce preventable complications of disease. Further studies in antibody deficiency syndromes with normal serum IgG are required.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.