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A Multi-Center Retrospective Database Evaluation of Pediatric Subjects Diagnosed With Methemoglobinemia
Neha Sinha1, Brooke Lichak2, Neal J Thomas1,3
1Pediatric Critical Care Medicine, Department of Pediatrics, Penn State Hershey Children's Hospital, Hershey, PA, USA.
Insights
Pediatric methemoglobinemia is rare, with low functional hemoglobin levels not significantly impacting treatment approaches. Glucose-6-phosphate dehydrogenase deficiency testing is infrequent, posing a risk with methylene blue treatment.
Area of Science:
- Pediatric Hematology
- Clinical Toxicology
- Rare Diseases
Background:
- Methemoglobinemia requires prompt recognition and management, yet clinical practices and outcomes in children remain under-documented.
- Limited understanding exists regarding current therapeutic strategies and their effectiveness in pediatric cases.
Purpose of the Study:
- To ascertain the prevalence of methemoglobinemia in the pediatric population.
- To evaluate the influence of methemoglobin and functional hemoglobin levels on clinical management.
- To assess current clinician approaches to treating pediatric methemoglobinemia.
Main Methods:
- A retrospective observational cohort study was conducted using deidentified electronic health record (EHR) data from the TriNetX® network.
- Multicenter EHR data were analyzed, including patient demographics, diagnostic codes, laboratory results, and medication records.
Main Results:
- The study included 98 children, revealing a methemoglobinemia prevalence of 0.0015% with a 30-day mortality rate of 6.1%.
- Methylene blue administration was more frequent in patients with methemoglobin levels >20% (70.6% vs. 24.7%).
- Treatment and critical care needs were similar for patients with functional hemoglobin <7 g/dL and ≥7 g/dL. Glucose-6-phosphate dehydrogenase deficiency (G6PD) testing was performed in only 13.2% of subjects.
Conclusions:
- Pediatric methemoglobinemia is rare, and G6PD testing is infrequently performed despite the risk of hemolysis with methylene blue.
- Treatment approaches appear consistent across varying functional hemoglobin levels, suggesting potential areas for educational improvement.
- The findings underscore the critical nature of methemoglobinemia and highlight the need for enhanced awareness, particularly regarding G6PD testing in pediatric patients.
Background:
Methemoglobinemia requires early identification and treatment, but limited knowledge exists regarding the current therapeutic approach taken by clinicians as well as the outcomes that occur in children.
Objectives:
To determine the current prevalence of this rare disease in the pediatric population, evaluate the impact of methemoglobin and functional hemoglobin levels, and assess how this disease is approached by clinicians. We hypothesize that methemoglobinemia prevalence is low and more methylene blue use would be observed in subjects with functional hemoglobin levels less than 7 g/dL.
Design:
This was a retrospective observational cohort study utilizing deidentified TriNetX® electronic health record (EHR) data.
Methods:
Using a multicenter EHR database, we evaluated subjective characteristics, diagnostic, laboratory results, medication, and procedural codes.
Results:
Ninety-eight children (mean age 5.3 ± 5.3 years) from 53 healthcare organizations were included. Methemoglobinemia prevalence was 0.0015% with an overall 30-day mortality of 6.1%. Subjects with methemoglobin percentages greater than 20% had a higher frequency of methylene blue administration (70.6% versus 24.7%, P = .0005). Critical care service requirements and methylene blue administration were similar in the subjects with functional hemoglobin less than 7 g/dL and more than 7 g/dL groups. Overall, 13 (13.2%) subjects underwent glucose-6-phosphate dehydrogenase deficiency (G6PD) testing.
Conclusion:
In our study, we found methemoglobinemia prevalence in children is low, there is a low frequency of G6PD testing despite methylene blue hemolysis risk, and subjects appeared to be treated similarly despite a low functional hemoglobin. These findings highlight the continued critical nature of this disease and may highlight opportunities for education aimed at improving care in children diagnosed with methemoglobinemia, particularly related to G6PD testing.
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