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Competency of Jeffrey Modell Foundation warning signs and routine laboratory tests in suspecting primary
Asmita Ghosh1, Moumita Samanta2, Parasar Ghosh3
1Department of Pediatrics, IPGME&R and SSKM Hospital, Kolkata, India.
Insights
The 10 warning signs from the Jeffrey Modell Foundation (JMF) can help identify Primary Immunodeficiencies (PIDs) in children. Key indicators include IV antibiotic need, family history, and recurrent ear infections.
Area of Science:
- Pediatric Immunology
- Clinical Diagnostics
Background:
- Primary Immunodeficiencies (PIDs) are a group of rare genetic disorders affecting the immune system.
- Early diagnosis and intervention are crucial for managing PIDs and preventing complications.
Purpose of the Study:
- To evaluate the effectiveness of the Jeffrey Modell Foundation (JMF) 10 warning signs and routine laboratory tests in predicting PIDs in hospitalized children.
- To identify the most sensitive and specific indicators for PID diagnosis.
Main Methods:
- A study was conducted on hospitalized children under 12 years old who met at least two of the 10 JMF warning signs.
- Children underwent routine and confirmatory laboratory testing for PID diagnosis.
Main Results:
- Out of 35,204 admitted patients, 66 met JMF criteria, and 34 were diagnosed with PID.
- The most sensitive criterion was the need for intravenous antibiotics (91%), increasing to 94% with a positive family history.
- Recurrent ear infections and family history of PID were the most specific indicators (88%).
Conclusions:
- The need for IV antibiotics, a family history of PID, and recurrent ear infections are the most significant JMF criteria for predicting PID.
- Routine laboratory tests like absolute neutrophil count, CRP, and ESR showed significant associations with PIDs.
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.
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