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[Streptococcus group B tardive meningitis revealing chronic septic granulomatosis]
Abstract:
A first-born boy operated on D20 for an abscess of the anal margin (E. Coli, Klebsiella) developed purulent meningitis due to a group B serotype III streptococcus on D35. The outcome was rapidly fatal. Results of immunologic investigations done on the day before death were suggestive of chronic granulomatous disease (CGD) as complete absence of reduction of nitroblue tetrazolium (NBT) was evidenced. Studies of polymorphonuclear cells from the mother showed normal production of oxygen, chemiluminescence and NBT reduction. During the subsequent pregnancy, fetal blood was sampled 19 weeks after the last menstrual period; results showed the fetus was male and the polymorphonuclear cells were incapable of reducing NBT and exhibited decreased chemiluminescence and oxygen production. The pregnancy was terminated. This case shows that delayed group B streptococcus infection can occur as the first manifestation of CGD, although this condition is usually responsible for infections due to staphylococci, enterobacteriaceae and yeasts.
Insights
A rare group B streptococcus infection in a newborn was the first sign of chronic granulomatous disease (CGD). This immunodeficiency disorder, diagnosed via NBT testing, led to a fatal outcome, highlighting delayed infection as a key indicator.
Area of Science:
- Immunology
- Pediatrics
- Infectious Diseases
Background:
- Chronic granulomatous disease (CGD) is a primary immunodeficiency primarily affecting phagocytes' ability to kill ingested microbes.
- Typical infections in CGD patients involve bacteria like Staphylococcus aureus, Serratia marcescens, and fungi such as Aspergillus species.
Observation:
- A male infant presented with an anal margin abscess followed by fatal purulent meningitis caused by group B Streptococcus (GBS).
- Immunological investigations revealed a complete absence of nitroblue tetrazolium (NBT) reduction in neutrophils, strongly suggesting CGD.
- Prenatal diagnosis in a subsequent pregnancy confirmed male sex and impaired neutrophil function (decreased NBT reduction, chemiluminescence, and oxygen production), leading to pregnancy termination.
Findings:
- This case highlights a delayed GBS infection as the initial clinical manifestation of CGD.
- Complete absence of NBT reduction confirmed the diagnosis of CGD in the affected infant.
- Normal maternal neutrophil function ruled out carrier status and indicated a de novo or inherited genetic defect.
Implications:
- CGD should be considered in neonates with unusual or severe bacterial, fungal, or even certain streptococcal infections.
- Early diagnosis of CGD through neutrophil function tests like NBT assay is crucial for timely management and genetic counseling.
- This case underscores the importance of considering CGD in the differential diagnosis of severe neonatal infections, even those not typically associated with the condition.