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Kernicterus and G6PD deficiency--a case series from Oman
P Arun Kumar Nair1, Saleh Mohammad Al Khusaiby
1Department of Child Health, Royal Hospital, Sultanate of Oman. srinavin@omantel.net.om
Insights
Newborns with undiagnosed glucose-6 phosphate dehydrogenase (G6PD) deficiency face increased kernicterus risk. Early detection and management are crucial to prevent this severe form of jaundice.
Area of Science:
- Neonatal Medicine
- Pediatric Hematology
- Genetics
Background:
- Jaundice is common in newborns, but severe cases leading to kernicterus are concerning.
- Glucose-6 phosphate dehydrogenase (G6PD) deficiency is a known risk factor for neonatal jaundice.
- Increasing kernicterus incidence is linked to unrecognized G6PD deficiency and early hospital discharge.
Purpose of the Study:
- To investigate the prevalence of G6PD deficiency in neonatal kernicterus cases.
- To review current literature on G6PD deficiency and kernicterus.
- To suggest preventive strategies for kernicterus in high-prevalence G6PD deficiency settings.
Main Methods:
- Retrospective case series analysis.
- Review of recent scientific literature.
- Clinical data compilation and analysis.
Main Results:
- Fourteen cases of kernicterus were identified over six years.
- G6PD deficiency was present in 71% of the kernicterus cases.
- A high prevalence of G6PD deficiency in the study population was noted.
Conclusions:
- Undiagnosed G6PD deficiency is a significant contributor to neonatal kernicterus.
- Preventive measures are essential, especially in regions with high G6PD deficiency rates.
- Enhanced screening and monitoring protocols are recommended for at-risk newborns.
Abstract:
The relationship between glucose-6 phosphate dehydrogenase (G6PD) deficiency and jaundice in the newborn period is well recognized. However, there is concern about the increasing incidence of kernicterus being reported worldwide, especially due to unrecognized G6PD deficiency and early discharge from hospital after birth. We report a case series of kernicterus from a set-up where the high prevalence rate of G6PD deficiency is known. Fourteen cases of kernicterus were seen during a time period of 6 years, 71 per cent of them had G6PD deficiency. Recent literature is reviewed and possible preventive measures in the light of current information and practices are suggested.