ArticleMayo Clinic proceedings2009
Incidence and temporal trends of primary immunodeficiency: a population-based cohort study.
Article in Mayo Clinic proceedings, 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 66 papers.
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Who cites it
66 citing papers in PubMed, 161 citations in OpenAlex.
- Diagnostic yield and clinical impact of genetic testing in inborn errors of immunity: lessons from a large Turkish cohort.European journal of pediatrics · 2026Article
- Case Report: A case series of using whole exome sequencing to detect novel variants in Vietnamese patients with inborn errors of immunity.Frontiers in genetics · 2026Article
- Diagnosing Hyper IgE Syndrome in a Resource-Limited Setting: A Case Report Highlighting the Utility of Clinical Scoring and Multidisciplinary Management.Case reports in immunology · 2026Article
- Sociodemographic drivers of care disparity among patients with inborn errors of immunity.Current opinion in pediatrics · 2025Review
- A Budget Impact Model of Treatment of Primary Immune Deficiency with Immune Globulin Subcutaneous (Human), 20% (IgPro20) (Hizentra).PharmacoEconomics - open · 2025Article
- Diagnostic disparities in inborn errors of immunity: From clinical suspicion to diagnosis.The journal of allergy and clinical immunology. Global · 2025Article
- Children with recurrent infections: perspective of immunoglobulin G subclasses deficiency and impaired specific antibody responses.Clinical and experimental immunology · 2025Review
- Immunological dysfunction of children with severe parapneumonic effusion.Frontiers in pediatrics · 2025Article
- Elevated Immunoglobulin E Serum Levels: Possible Underlying Factors That Can Cause an Inborn Error of Immunity in the Pediatric Population with Recurrent Infections.Antibodies (Basel, Switzerland) · 2024Article
- Current Issues in the Management of IgG Subclass Deficiencies in Adults With Chronic Respiratory Diseases.Allergy, asthma & immunology research · 2023Review
- The GAIN Registry - a New Prospective Study for Patients with Multi-organ Autoimmunity and Autoinflammation.Journal of clinical immunology · 2023Article
- Challenges of Diagnosing Mendelian Susceptibility to Mycobacterial Diseases in South Africa.International journal of molecular sciences · 2023Review
- Extended List of Warning Signs in Qualification to Diagnosis and Treatment of Inborn Errors of Immunity in Children and Young Adults.Journal of clinical medicine · 2023Article
- Missed Opportunities? A Retrospective Study Into Adults Hospitalized With Invasive Infection From Airway Pathogens.Open forum infectious diseases · 2022Article
- Immune Sexual Dimorphism: Connecting the Dots.Physiology (Bethesda, Md.) · 2022Review
- Immune Dysregulation in Monogenic Inborn Errors of Immunity in Oman: Over A Decade of Experience From a Single Tertiary Center.Frontiers in immunology · 2022Article
- Trends in Pediatric Primary Immunodeficiency: Incidence, Utilization, Transplantation, and Mortality.The journal of allergy and clinical immunology. In practice · 2022Article
- Treatment efficacy of dupilumab in a hyper-immunoglobulin E syndrome patient with severe atopic dermatitis.JAAD case reports · 2021Article
- Common and Rare Variant Prediction and Penetrance of IBD in a Large, Multi-ethnic, Health System-based Biobank Cohort.Gastroenterology · 2021Article
- A structured patient empowerment programme for primary immunodeficiency significantly improves general and health-related quality of life.Central-European journal of immunology · 2021Article
6 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
5 authors at 4 institutions in 1 country.
Funding
Abstract
objectiveTo determine the incidence and temporal trends of primary immunodeficiency diseases (PIDs) and examine whether an association exists between delayed diagnosis and increased morbidity. PATIENTS AND
methodsWe performed a historical cohort study to describe the epidemiology of PIDs in Olmsted County, Minnesota, during a 31-year period from January 1, 1976, through December 31, 2006, using the Rochester Epidemiology Project. Incidence and trends over time, presence of comorbid conditions, and trends in management were determined.
resultsDuring the 31-year study period, 158 new cases of PIDs were diagnosed, with an overall incidence rate of 4.6 per 100,000 person-years. The rate of PIDs from 2001 through 2006 (10.3 per 100,000 person-years) was nearly 5 times higher than that from 1976 through 1980 (2.4 per 100,000 person-years). The associations between continuous variable(s) and categorical outcome(s) were assessed by using the Wilcoxon rank sum test. Longer delay in diagnosis was significantly associated with recurrent sinusitis (P<.001), recurrent pneumonia (P=.03), and subsequent treatment with immunoglobulins (P<.001). On the basis of Kaplan-Meier survival estimates, the proportion of patients surviving at 10 years after diagnosis was 93.5% (95% confidence interval, 85.9%-97.1%). However, older age at diagnosis was significantly associated with mortality (P=.01).
conclusionThis is one of the first population-based studies to examine the temporal trends of PIDs. The incidence of PIDs increased markedly between 1976 and 2006. In this cohort, a delay in diagnosis was common and was associated with increased morbidity. Despite substantial morbidity, most patients with PIDs can expect a normal life span.
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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.