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Trends in diagnosis and outcomes for pediatric primary immunodeficiency patients

Published: October 27, 2021

The past 15 years has been an incredible time of discovery in the field of primary immunodeficiency, which is an umbrella term used to describe genetic disorders that effect how the immune system functions. In 2005, there were approximately 100 known genetic causes for primary immunodeficiency. Today, we know of 450 genetic defects that lead to changes in the immune system, a number that will continue to grow with scientific advancement.

In a recent The Journal of Allergy and Clinical Immunology: In Practice article, Eddens et al. examined how the frequency of diagnosis and how various healthcare outcomes changed for pediatric patients with primary immunodeficiency during the time in which the expansion of known primary immunodeficiencies was occurring (2004-2018). The team used the Pediatric Health Information System database, which collects data with each hospitalization from more than 50 children’s hospitals across the nation. Using this database, Eddens et al. evaluated how many new diagnoses of primary immunodeficiency occurred each year. Additionally, how often patients required care in an intensive care unit, rates of bone marrow transplantation, and mortality rates were examined yearly to evaluate for trends in care.

During the study period, approximately 17,000 patients with primary immunodeficiency were hospitalized. The rates of hospitalization and the rates of new diagnosis of primary immunodeficiency were unchanged during the study. For every 1,000 hospitalizations in the PHIS database, there were 3 first-time encounters for a primary immunodeficiency, which the team used as a surrogate for a new diagnosis. Specific immunodeficiencies, such as disorders affecting the cells that make antibodies, were diagnosed more frequently. More patients were diagnosed in teenage or early adulthood years. Patients with immunodeficiency due to T cell dysfunction had the highest rates of intensive care unit admission. In regards to bone marrow transplantation, the team found that primary immunodeficiency patients had fewer post-transplant complications over time. Finally, Eddens et al. demonstrated that the mortality rates for patients with pediatric immunodeficiency significantly decreased.

The Journal of Allergy and Clinical Immunology: In Practice is an official journal of the AAAAI, focusing on practical information for the practicing clinician.

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