Severe Combined Immunodeficiency

WASHINGTON — Children with primary immunodeficiencies should receive routine inactive vaccinations and other prophylactic treatment, according to a presentation at the American Academy of Allergy, Asthma & Immunology Annual Meeting.

Whole-exome sequencing helped physicians identify undiagnosed primary immunodeficiency diseases in children with community-acquired sepsis, according to a study published in Pediatric Allergy and Immunology.

Between 40 and 80 infants are born with severe combined immunodeficiency disorder each year in the United States and Canada. The disease is fatal without bone marrow transplants as infections overwhelm compromised immune systems.

Five-year survival rates among children with severe combined immunodeficiency remained stable at 72% to 73% from 1982 to 2009 before improving to 87% from 2010 to 2018, according to a study published in The Lancet.

Infants with Artemis-deficient severe combined immunodeficiency improved after corrected genes in their stem cells enabled them to grow their own T cells and B cells, according to a study published in The New England Journal of Medicine.

Influenza remains an important threat to public health, and people with impaired immune systems are at particularly high risk for severe disease and complications. One comparative analysis showed that severely immunocompromised patients with influenza experienced significantly more hospitalizations, ICU admissions and mechanical ventilation than nonimmunocompromised patients. However, clinical signs and symptoms may be pronounced on initial presentation, underscoring the importance of seeking early medical attention. Additionally, antiviral resistance is more common and the mean viral shedding time was longer among immunocompromised patients (19 days compared with 6.4 days in healthy people).