Competency of Jeffrey Modell Foundation warning signs and routine laboratory tests in suspecting primary immunodeficiencies: A cross-sectional multi-centric prospective study from eastern India

Aim

To test the efficaciousness of the 10 warning signs of Jeffrey Modell Foundation (JMF) and routine laboratory tests in predicting Primary Immunodeficiencies (PIDs).

Methods

Hospitalized children <12 years age satisfying at least two of 10 warning signs were subjected to routine and confirmatory tests.

Results

Of 35 204 admitted patients, 66 satisfied the JMF criteria and 34 had PID. Also, 59% were infants, with a female preponderance. The most common immunodeficiency disorder group were antibody deficiencies and phagocyte defects (35.3%). Chronic granulomatous disease (CGD) was the commonest overall (29.4%). The need for intravenous antibiotics was the most sensitive (91%) criterion for predicting PID. When combined with positive family history of PID, sensitivity (94%) increased further. The two most specific indicators were recurrent ear infections (88%), and family history of PID (88%). The best positive predictor was family history of PID (69%), and the best negative predictor was recurrent sinus infections (58%). Significant association was found between persistent oral thrush and PID (p .043), and insufficient weight gain and antibody deficiencies (p .037). Absolute neutrophil count, CRP, and elevated ESR were also significantly associated with PIDs (p-values being .036, .011, and .014 respectively).

Conclusion

Out of all 10 JMF criteria, the three most important ones to predict PID were need for IV antibiotics, family history of PID, and recurrent ear infections.