Neonatal Screening for Chronic Granulomatous Disease before BCG Vaccination

Abstract

Chronic granulomatous disease is the most commonly encountered immunodeficiency involving the phagocyte. Between 6% and 57% of CGD patients will develop BCG complications if vaccinated. In this study, we screened 300 neonates for defective respiratory burst activity of neutrophils using dihydrorhodamine 123 (DHR) test. They were 200 healthy full-term neonates with family history suggestive of PID and 100 healthy full-term neonates without such history recruited from El-Galaa Teaching Hospital during the period from July 2013 to April 2014. Sibling death due to infections or history of affected family members with undue susceptibility to infections was considered to recruit neonates with family history suggestive of PID. Chronic maternal illness includes diabetes mellitus and hypertension as well as perinatal asphyxia, low birth weight were exclusion criteria to avoid possible affection of the results of the DHR test. Complete blood counts with differential count and dihydrorhodamine test were done to the whole studied sample. Results were expressed as percentage of neutrophils showing oxidative response to PMA stimulation. Eight neonates (0.04%) had family history of sibling death due to repeated infections. Pneumonia was the cause of death in 2 (25%) siblings during first year of life and in 3 (37.5%) of them during early childhood where they suffered of recurrent chest infections and skin abscesses. Neonatal sepsis was the cause of death in 3 (37.5%) of them the DHR test has a mean (SD) of 73.01% (17.76%) and ranged between 33.1%-98.9%. The 95% confidence interval (CI) is 70.98%-75.01%. The normal reference range of the DHR test, using PMA stimulation of the neutrophils, is ≥95% expressed as percentage of the stimulated neutrophils. There was no significant difference between male and female neonates as regard the results of the DHR test. Values of DHR test were significantly lower among neonates with family history suggestive of PID than those without such history (P = <0.001). There was no significant correlation between the values of the DHR test and the birth weight, gestational age or neutrophil counts among the whole studied sample. Multiple regression analysis showed that the most significant factors affecting the results of DHR test positively are apgar score at 5 min then WBC count then platelets count and the most significant factors affecting the results of DHR test negatively are Hemoglobin then Mean Corpuscular Volume.