Spectrum of Primary Immunodeficiency Disorders in Hospitalized Children: Multicentric Data From Kolkata

Area of Science:

• Pediatric Immunology
• Infectious Diseases
• Clinical Genetics

Background:

• Primary immunodeficiency diseases (PIDs) are a group of genetic disorders affecting the immune system.
• Hospitalized children with recurrent or severe infections are at higher risk for underlying PIDs.

Purpose of the Study:

• To determine the incidence and types of PIDs in hospitalized children presenting with infections.
• To establish the prevalence of PIDs in Eastern India.

Main Methods:

• A prospective study was conducted in five tertiary-care facilities in Kolkata.
• Children up to 12 years old hospitalized with infections were screened using the Jeffrey Modell Foundation (JMF) criteria.
• Children meeting at least 2 of 10 warning signs were further evaluated for PIDs.

Main Results:

• Out of 33,204 admissions, 50 children met JMF criteria, and 27 were diagnosed with PID, yielding a prevalence of 1 in 1000.
• Antibody deficiency (37.03%) and phagocytic defects (33.3%) were the most common PID types.
• Respiratory infections were the most frequent presentation (62.97%), with Acinetobacter baumannii being the most common pathogen.

Conclusions:

• This is the first cohort study of PIDs from Eastern India.
• A systematic clinical and diagnostic approach aids in the early identification and management of PIDs.
• Early diagnosis of PIDs is critical for timely therapeutic interventions and improved patient outcomes.