The Indian Journal of Pediatrics

, Volume 81, Issue 6, pp 599–607 | Cite as

Prevention of Kernicterus in South Asia: Role of Neonatal G6PD Deficiency and its Identification

  • Yassar H. Arain
  • Vinod K. BhutaniEmail author
Review Article


Extreme hyperbilirubinemia (EHB) caused by neonatal glucose-6-phosphate dehydrogenase (G6PD) deficiency is strongly associated with mortality and long-term neurodevelopmental impairment, yet there are limited national strategies to reduce this burden in South Asia. Current known and predicted prevalence of G6PD deficiency in Afghanistan, Bangladesh, Bhutan, India, Nepal, and Pakistan ranges from 3.8 to 15 %, with regional “hot spots” exceeding 22 %. Annually, 3.14 million infants are born at risk for this condition. In 2010, South Asian countries reported 37 million (27 %) of world-wide livebirths ≥ 32 wk gestational-age and G6PD deficiency accounted for > 33 % of the global EHB burden, in contrast to 2.2 % for those born in high-income nations. Traditional national approach includes universal newborn screening in malaria-endemic countries or those with prevalence >3.5 %. However, screening implementation should be best optimized using timely quantitative enzyme assay and identification of at-risk female newborns. Furthermore, economic and social constraints, in context of sub-regional variances, call for flexible problem-solving methods in anticipation of changing community demographics. Thus, incremental and need-based newborn screening programs could be the most optimal approach. A human-centered design (HCD) approach, as an alternate pathway, could build the evidence to translate the complex biology of G6PD deficiency and the biodesign of affordable technologies, allowing facilitation of access to knowledge and services, in order to deliver on a long-term public health mandate. Key steps would encompass the initiation of local inquiry of both quantitative and qualitative data to identify at-risk communities and to prospectively design for local innovative solutions.


Neonatal Jaundice Hyperbilirubinemia G6PD deficiency Impairment Disability Mortality Deafness 



The authors would like to thank Global Prevent Kernicterus Network (Stanford): Lois Johnson (USA); Ronald J. Wong (Stanford University); Shanmukha Srinivas for data collection and map design, Martin Castillo Cuadrado for statistical analysis, and Dr. Matthew B. Wallenstein for his editing.


YA: Conducted the research on the model design, its application, review of literature and initial draft of the manuscript; VKB: Conducted the research, assessed the burden, conceptualized the study design and completed the final version of the manuscript, and will act as guarantor of this paper.

Conflict of Interest


Role of Funding Source

No specific financial assistance was received in support of this study. This work was partially funded by SBIR; VKB has received grant support from the National Institutes of Health (NIHSBIR Sub-Award: HD062316).


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Copyright information

© Dr. K C Chaudhuri Foundation 2014

Authors and Affiliations

  1. 1.Division of Neonatal and Developmental Medicine, Department of PediatricsStanford University School of Medicine, Lucile Packard Children’s HospitalStanfordUSA
  2. 2.Department of Pediatrics, Division of Neonatal and Developmental MedicineStanford University School of MedicinePalo AltoUSA

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