Most hospitalized children with primary immunodeficiency survive in the United States

Published online: January 12, 2018

Primary immunodeficiency diseases (PIDDs) are a set of rare yet life-threatening diseases that make children more prone to infections. Although genetic testing can now identify children with PIDDs earlier, the prevalence and outcomes of these diseases in the United States has not been well understood.

In a recently published article in The Journal of Allergy and Clinical Immunology: In Practice, Rubin and colleagues studied the clinical features of children with a PIDD, and their outcomes, including morbidity, mortality, length of hospital stay, and costs. Using the Kids’ Inpatient Database, a large pediatric database from the Agency for Healthcare Research and Quality, they estimated the number of pediatric PIDD hospitalizations from 2003 to 2012.

Childhood PIDDs may have increased over time among all groups of children in the United States. Children five years of age and younger had the highest chance of death, longest hospital stays and greatest cost associated with hospitalization. Overall, however, although care was costly, the chance of death was low.

Additional studies would help us better understand and predict the chance of PIDD in the United States. More research is also needed to find cost-effective tests and treatments to improve the health of infants and young children with PIDD.

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