ORIGINAL ARTICLEIncidence and Temporal Trends of Primary Immunodeficiency: A Population-Based Cohort Study
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PATIENTS AND METHODS
Epidemiological research in Olmsted County, Minnesota (2000 US Census population, 124,277), is optimized by the county's relative isolation from other urban centers and the delivery of nearly all medical care to county residents by a small number of health care provider groups. With the exception of a higher proportion of the working population employed in the health care industry, the characteristics of the population of Olmsted County are similar to those of US whites (Figure 1).14
Since 1907,
RESULTS
Review of the 513 records with diagnostic codes related to PIDs revealed 158 incident (31%) and 14 prevalent (3%) cases of PIDs.
The study population was predominantly white (166; 97%), with 4 cases seen among African Americans (3%) and 1 case each from patients of Asian and Hispanic heritage. The median age of the population was 25 years (interquartile range, 4-51 years), and 93 (54%) of the patients were female.
Accurate information on the principal provider of longitudinal care for PIDs was
DISCUSSION
Although some data are available regarding the prevalence of PIDs, the current study is the first, to our knowledge, to study their incidence.
We found an overall incidence of 4.6 cases of PIDs per 100,000 person-years, and these incidence rates did not differ by sex. We also found an increasing temporal trend in incidence rates during the past 31 years, with a rate of 10.3 per 100,000 person-years rate in 2000-2006 compared with 2.4 per 100,000 person-years in 1976-1980. Incidence rates were
CONCLUSION
With the first-ever population-based study on the incidence of PIDs, we have found that the diagnosis of PID increased during the past 3 decades. We also found that a delay in diagnosis was associated with increased morbidity. Such epidemiological data are crucial if we are to raise the awareness of the medical community about PIDs. With the newborn screen for some types of PIDs (eg, severe combined immunodeficiency) on the horizon, we need epidemiological data to support the public health
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2021, Journal of Allergy and Clinical Immunology: In PracticeCitation Excerpt :Morbidity reviews and studies on PID usually do not mention AAA.49,50 With only 40 cases reported in more than 60 years and none reported in large case series, this work confirms the existence and rarity of AAA occurrence in the PID course.51-55 The typical clinical picture is a patient displaying recurrent infections, mostly bacterial pneumonia, since adolescence or beginning of adulthood, with a delayed diagnosis of PID and onset of treatment after 10 years of evolution.
This study was made possible by the Rochester Epidemiology Project (grant R01 AR30582).